anti-Sm are specific to SLE,

Another question about SLE antibodies. Clinical case of SLE with renal involvement, in which positive double-stranded anti-DNA increases the risk of nephritis. As for the rest, anti-Sm are specific to SLE, ANA are detected in more than 90% of cases, and anti-centromere are typical of scleroderma.

A 27-year-old black woman consults for the appearance of edema in the lower limbs, decreased diuresis, fever and a malar rash in butterfly wings of 20 days of evolution. Laboratory tests showed creatinine 3 mg/dl, leukopenia, hemoglobin 10.5 g/dl (normochromic normocytic), CRP 9 mg/dl and ESR 60 mm. Regarding the pathology presented by this patient, point out the correct answer:

The anti-Sm antibodies are not specific for this pathology.

Anti-centromere antibodies are detected in 90% of cases.

ANA (antinuclear antibodies) are detected in 50% of cases.

Anti-two-stranded or native anti-DNA correlates with the risk of nephritis.

Clinical and laboratory features of systemic lupus erythematosus (SLE) in Pakistani patients.

To study the clinico-pathological features of systemic lupus erythematosus (SLE) in Pakistani patients at the time of presentation in the four teaching hospitals of Rawalpindi-Islamabad area. Department of Immunology, Armed Forces Institute of Pathology (AFIP), Rawalpindi, Military Hospital (MH), Rawalpindi, Armed Forces Institute of Urology (AFIU), Rawalpindi, Combined Military Hospital (CMH), Rawalpindi, Department of Rheumatology, Pakistan Institute of Medical Sciences (PIMS), Islamabad. Patients suffering from SLE, diagnosed on the basis of the ARA criteria. Clinico-pathological features at the time of presentation. Twelve male patients in age range 5-30 years (mean 21.6 years) and 38 female patients in age range 8-37 years (mean 22.2 years) were included in the study, showing a female to male ratio of 3:1. Fever was the most common presenting feature (100%), followed by arthralgias/arthritis (98%), malar rash (64%) and oral ulcers (58%). Laboratory results showed antinuclear antibody to be positive in all patients followed by anti double stranded DNA antibodies (anti DNA antibodies: 64%), anti extractable nuclear antigen antibodies (anti ENA antibodies: 64%) and evidence of complement consumption in 64% of the patients. Erythrocyte Sedimentation Rate (ESR) was found to be increased with a mean of 64 mm first hour fall in males and a mean of 87 mm first hour fall in the female patients. The C reactive protein was detected to be normal or marginally increased inspite of the increased ESR with mean of 7 mg/l for male patients and 5 mg/1 for the females. The subject patient population presented almost universally with fever and arthralgias or arthritis in combination with malar rash or oral ulcers and in some patients a combination of all of the above was observed. A combination of positive anti nuclear antibody test, evidence for consumption of the complement in form of low C4 and C3, increased ESR and low C reactive protein were found to be a sensitive and cost effective set of laboratory findings for the diagnosis of patients suffering from SLE. The above mentioned set of clinical and laboratory features would help in the correct and early diagnosis of patients suffering from SLE, a relatively rare disease, in the busy medical out patient departments in our set up.

Identical twins:one with anti-glomerular basement membrane glomerulonephritis,the other with systemic lupus erythematosus.

Anti-glomerular basement membrane (GBM) glomerulonephritis and systemic lupus erythematosus (SLE) are both disorders of the immune system; however, they are known as distinct diseases. Till now no clinical evidence suggests the genetic relationship between these two diseases. Herein, we present two identical twins; one was diagnosed as anti-GBM glomerulonephritis, the other SLE. This is the first clinical report on the genetic relationship between these two diseases. A 25-year-old female was admitted complaining of intermittent gross hematuria for 6 months and elevated serum creatinine for 1 month. She denied hemoptysis. Laboratory examinations showed hemoglobin 7.4 g/dL, serum creatinine 7.15 mg/dL and albumin 2.8 g/dL. Urinalysis showed hematuria (484 RBCs per high-power field) and proteinuria 4+. Antinuclear antibody, complement levels and ANCAs were all normal. Renal ultrasound showed normal-sized kidneys without obstruction or masses. Serum anti-GBM antibody assay showed 119.70 RU/mL (normal range, <20 RU/mL). Chest X-ray was normal. She was diagnosed as anti-GBM glomerulonephritis and received plasma exchange (2000-3000 ml plasma/exchange, 5 turns), methylprednisolone 0.5 g for three days, plus cyclophosphamide. Although serum anti-GBM antibodies decreased gradually to a normal range, her renal function did not improve. One month later, her identical twin sister was diagnosed as SLE based on malar erythema, arthralgia, antinuclear antibody positive with liter 1:1000, and Anti-Smith (Sm) antibody ++. Anti-GBM antibody and complements were within normal ranges. Further study showed these twins were HLA-DRB1*1501 homozygotes. The presence of identical twins having anti-GBM nephritis and SLE respectively provides clinical evidence to support that anti-GBM nephritis and lupus may share a common genetic background to some extent, while environment may contribute to disease evolution in part.

Rowell Syndrome in Nigeria: Systemic Lupus Erythematosus Presenting as Recurrent Erythema Multiforme in a Young Woman.

Dear Editor, Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by diverse patterns of auto-antibody production with multi-organ affectation. Cutaneous involvement, either alone or in association with other systemic illnesses, is one of its most common manifestations (1). Dermatologic disorders like malar and discoid rashes are quite suggestive of SLE. However, the occurrence of non-specific skin lesions like erythema multiforme (EM) in patients with SLE (Rowell syndrome) can rarely occur (1). In such patients, a diagnosis of SLE may be missed or delayed in the absence of other overt clinical features of lupus. Herein we report a case of recurring EM-like eruptions as the cardinal cutaneous manifestation of previously undiagnosed, active SLE in a young Nigerian woman. A 26-year-old Nigerian woman presented with a three-day history of non-pruritic, generalized, and target-like, erythematous annular patches and plaques which mostly affected the trunk. A few lesions had presented with crusting and erosions at the time of examination (Figure 1). Associated symptoms included oral painful ulcers, low grade fever, and malaise. The patient had no other systemic symptoms and her prior drug history was not remarkable. Her erythrocyte sedimentation rate (ESR) was 66 mm/hour using the Westergren method. Screening for HIV and hepatitis B and C was negative. Herpes simplex, cytomegalovirus, and Epstein Barr viruses could not be screened for. Other baseline investigations (complete blood count, electrolytes, urea and creatinine as well as urinalysis) were within normal limits. The patient was managed as a case of EM of an unidentified inciting agent and her symptoms resolved with supportive care and antibiotics. However, she developed a recurrence about 5 weeks later, with more extensive and coalescent skin lesions (Figure 2). Additionally, there was a new onset of alopecia and pain in the small joints of the hands as well as both knees and ankles. At this time, the patient's ESR had gone up to 112 mm/h and she had developed significant proteinuria, with a protein creatinine ratio of 1.3 g/g (reference <0.5 g/g). Her antinuclear antibody (ANA) titer was high (1:320) with a speckled pattern. Anti-Smith antibody was also positive. A renal biopsy was declined. A tentative diagnosis of Rowell syndrome was made. The patient was started on high-dose steroids and hydroxychloroquine 200 mg twice daily. Subsequent care included the use of mycophenolate mofetil 1 g twice daily for 6 months. This was then changed to azathioprine at 50 mg twice daily. Follow-up after 6 months showed sustained clearance of skin lesions, resolution of fever and joint pains, as well as improvement in the renal profile, with a urine protein-creatinine ratio of 0.77 g/g. The presence of systemic lupus erythematosus, EM-like lesions, and a speckled pattern of antinuclear antibody in our patient fulfils the revised diagnostic criteria for RS put forward by Zeitouni et al. at the turn of the twenty-first century (2). Considering the rarity of EM-like lesions in SLE and the possibility of constitutional symptoms in EM, a diagnosis of RS may be readily overlooked in patients like the one described, whose major cutaneous manifestation of severe active SLE was EM-like lesions. In contrast to classic EM, where skin lesions are concentrated in the extremities, a predominant truncal distribution of EM-like lesions as found in our patient may favor a clinical consideration of RS (3). However, some authors have challenged the existence of Rowell syndrome as a distinct clinical laboratory entity. Arguments put forward in this regard include the fact that none of the immunological markers that have been described in RS are specific to any disorder. Additionally, the annular polycyclic dermatosis seen in sub-acute cutaneous lupus erythematosus (SCLE) can be difficult to clinically and histologically differentiate from EM (4,5). Patients with SLE also have a higher likelihood of developing adverse drug reactions (6). The inherent complexity of SLE may make for delayed and oftentimes difficult diagnosis, especially in a country where immunologic tests are expensive and rheumatologists are scarce. When patients do occasionally present with recurrences of skin lesions in the spectrum of EM, Steven-Johnson syndrome, and toxic epidermal necrolysis in the absence of a definite inciting agent, undiagnosed lupus may indeed be present in some of these individuals and should be considered in the differential diagnosis. In conclusion, while it is very rare, SLE may present first with recurrent episodes of EM-like rash. Despite the various possibilities which underlie their association, prompt identification and treatment of SLE in patients presenting with EM is important to prevent death or irreversible organ damage.

[Interstitial lupus nephritis].

A 17-year old-male presented with a 6-week history of weight loss, lassitude and calf pains. On examination he was very pale. Laboratory tests showed a very high erythrocyte sedimentation rate (155 mm in the first hour), anaemia (haemoglobin 10.1 g/dl), and a raised serum creatinine of 1.54 mg/dl. Microhaematuria (5-10 erythrocytes/microliter) and pronounced pyuria (500 leucocytes/microliter) were present, but the urine was sterile and there was no increase in albumin excretion. The serum IgG was raised to 75.7 g/l, suggesting an autoimmune disorder. Anti-nuclear antibodies (titre 1 : 1920) and anti-double-stranded DNA antibodies (31 U/ml) were present, while the serum complement C4 was decreased to 0.11 g/l. Renal histology showed an interstitial nephritis without glomerular involvement, while the bone marrow showed vasculitis accompanied by a prominent plasma-cell infiltrate. A diagnosis of interstitial nephritis associated with systemic lupus erythematosus was made, with asymptomatic cardiac and hepatic involvement. Renal function recovered rapidly with prednisolone therapy (initial dose 2 mg/kg.d). While glomerulonephritis is the most common lupus-associated renal disorder, isolated interstitial nephritis may occur in some cases, often with an absence of proteinuria.

InternalMed_Harrison

Diagnostically, the most important autoantibodies to detect are ANA because the test is positive in >95% of patients, usually at the onset of symptoms. A few patients develop ANA within 1 year of symptom onset; repeated testing may thus be useful. ANA tests using immunofluorescent methods are more reliable than enzyme-linked immunosorbent assays (ELISAs) and/or bead assays, which have less specificity. ANA-negative lupus exists but is rare in adults and is usually associated with other autoantibodies (anti-Ro or anti-DNA). High-titer IgG antibodies to double-stranded DNA (dsDNA) (but not to single-stranded DNA) are specific for SLE. ELISA and immunofluorescent reactions of sera with the dsDNA in the flagellate Crithidia luciliae have ~60% sensitivity for SLE; identification of high-avidity anti-dsDNA in the Farr assay is not as sensitive but may correlate better with risk for nephritis. Titers of anti-dsDNA vary over time. In some patients, increases in quantities of anti-dsDNA herald

Anti-dsDNA antibodies

Anti-double stranded DNA (Anti-dsDNA) antibodies are a group of anti-nuclear antibodies (ANA) the target antigen of which is double stranded DNA. Blood tests such as enzyme-linked immunosorbent assay (ELISA) and immunofluorescence are routinely performed to detect anti-dsDNA antibodies in diagnostic laboratories. They are highly diagnostic of systemic lupus erythematosus (SLE) and are implicated in the pathogenesis of lupus nephritis. Discovery The first evidence for antinuclear antibodies arose in 1948 when Hargraves, Richmond and Morton discovered the LE cell. These abnormal cells, which are found in the bone marrow of persons who have SLE are categorised as polymorphonuclear leukocytes with phagocytosed whole nuclei. Subsequently, in 1957, antibodies to dsDNA were the first autoantibodies to be identified in patients with SLE. Antibody production

Shrinking Lung Syndrome -- Evaluation

Routine blood testing is typically normal. A raised white cell count may be suggestive of infection. Raised levels of CRP may indicate serositis. The ESR may be raised in the context of active lupus. Renal function is expected to be normal unless there is co-existent lupus nephritis. Creatine kinase levels are typically normal unless active myositis elsewhere. The serological profile of the lupus patient should be clarified. A positive ANA is present in all cases. Positive antiphospholipid serology is noted in approximately 2/3 of patients. There may be evidence of hypocomplementemia, increased DNA binding (3/4 cases), and a positive ENA (including SS-A, SS-B, anti-RNP, and anti-Sm). However, these tests may also be normal and they neither confirm nor refute the diagnosis. [15]

Obstentrics_Williams

TABLE 59-1. Some Autoantibodies Produced in Patients with Systemic Lupus Erythematosus (SLE) Best screening test, multiple antibodies; a second negative test makes SLE unlikely High titers SLE-specific; may correlate with disease activity, nephritis, and vasculitis Specific for SLE Not SLE-specific, high titers associated with rheumatic syndromes Not SLE-specific; associated with sicca syndrome, predisposes to cutaneous lupus, neonatal lupus with heart block, reduced risk of nephritis Associated with anti-Ro Common in drug-induced lupus Lupus anticoagulant and anticardiolipin antibodies associated with thrombosis, fetal loss, thrombocytopenia, valvular heart disease; false-positive test for syphilis Direct Coombs test, may develop hemolysis Thrombocytopenia in 15%; poor clinical test dsDNA = double-stranded DNA; RNP = ribonucleoprotein. Data from Arbuckle, 2003; Hahn, 2015. TABLE 59-2. Some Clinical Manifestations of Systemic Lupus Erythematosus Modified from Kasper, 201r5.

[Membranous nephropathy in Japanese children].

Since 1973, 15 patients, consisting of 8 boys and 7 girls, were diagnosed as having membranous nephropathy (MN). The average age at detection was 8.2 years (2-14 years). The presenting symptom was edema in 1, pyrexia in 1 and upper respiratory infection in 1 case, in the all other cases, abnormal urinalysis was detected by the school or chance urinalysis. Surface antigen of hepatitis B virus (HBs) was positive in 6 patients and negative in 9. Anti-nuclear antibody (ANA) was positive in 3 and negative in 11. In one patient, ANA was not tested. One patient who was negative for ANA was diagnosed as having SLE 4 years later. At the last follow-up, 10 patients continued to have urinary abnormalities. Among these was one case positive for HBs antigen who went into end-stage renal failure. In the other 14 patients, the serum creatinine level was below 1.4 mg/dl. All patients showed a normal mesangium or mild mesangial proliferation. The patient diagnosed as having SLE. 4 years later showed mesangial deposits at the first renal biopsy. In our experience, most patients with MN were detected by the school or chance urinalysis and six of the these had positive HBs antigen. Lupus nephritis must be ruled out in making a diagnosis of idiopathic MN.

Clinical features and evolution of antinuclear antibody positive individuals in a rheumatology outpatient clinic.

To identify individuals with antinuclear antibodies (ANA) not fulfilling criteria for systemic lupus erythematosus (SLE) or other connective tissue diseases (CTD); to describe their clinical and serological features, to identify factors indicating evolution to SLE. A case-control study, based on retrospective evaluation of clinical files. The cases were ANA positive individuals (n = 50) examined in a medical outpatient setting, for symptoms compatible with SLE, but not fulfilling SLE classification criteria. Two patients with SLE were matched to each case in terms of age at initial symptom onset and sex. Thyroid autoimmunity was assessed by detecting antithyroid antibodies. Fisher's exact test and conditional logistic regression were used to evaluate the predictive ability of initial findings for SLE development. ANA positive individuals suspected of having a CTD present a wide variety of symptoms and findings, usually at the 4th to 5th decade of life. Antibodies to Sm and U1RNP were absent; anti-Ro(SSA) and anti-La(SSB) occurred in 6%, while anti-dsDNA occurred in less than 10% of the cases. Arthritis, butterfly and discoid rash, Raynaud's phenomenon, and anti-Ro/SSA antibodies are the initial findings indicating evolution to SLE. Hematological abnormalities such as leukopenia and thrombocytopenia as well as constitutional symptoms such as easy fatigue and arthralgias are not associated with evolution to SLE. Antithyroid antibodies were detected in 16% of the cases and 2.3% of controls. ANA may connote a form of systemic autoimmunity that is expressed as a wide variety of complaints, even in the absence of a definite diagnosis of CTD. Arthritis, rash, Raynaud's phenomenon, and anti-Ro/SSA antibodies indicate evolution to SLE. Autoimmune thyroid disease occurs in ANA positive individuals not fulfilling SLE classification criteria rather than in patients with SLE.

[Systemic lupus erythematosus and silicosis].

A 60-year-old stonemason, suffering for many years from joint pains and exertional dyspnoea, developed a high fever with weight loss. Physical examination revealed reddening of light-exposed skin areas, fine rales and overly warm and reddened hand and knee joints. Abnormal laboratory findings were increased erythrocyte sedimentation rate of 66 mm/h, C-reactive protein concentration of 1 mg/dl, haemoglobin of 9.4 g/dl and white cell count of 3300/microliters. Urine contained albumin (100 mg/dl) and cylinders. Titres of both antinuclear and anti-ds-DNA antibodies were elevated (1:2560 and > 97 U/ml, respectively). The chest radiography showed enlarged hili, as well as reticular and nodular shadows which histologically showed silicosis. Systemic lupus erythematodes was diagnosed and the patient was treated with prednisone (2 mg/kg daily), the dosage being reduced to 12 mg daily within 3 months. When the joint pains recurred, azathioprine (50 mg daily) was added for 24 months. At present he is receiving prednisone (12 mg daily) and there has been no recurrence for 4 years.

An Unusual and Rare Case of a Seronegative Male Patient With Lupus Nephritis.

Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect nearly every organ system. The 2019 European League Against Rheumatism and American College of Rheumatology (EULAR/ACR) SLE have proposed an additive, weighted multicriteria system for classifying SLE while using antinuclear antibody (ANA) as an entry criteria. Most patients with SLE will test positive for ANA. We report a 28-year-old half Caucasian and half Asian male patient who initially presented with malar rash, severe bilateral lower extremity edema, and proteinuria. Typical serological tests associated with SLE were negative. The diagnosis of seronegative lupus nephritis was made with kidney biopsy that revealed class IV and V lupus nephritis. Creatinine improved from 2.06 to 0.87 mg/dL with oral mycophenolate mofetil (MMF) and a tapered dose of glucocorticoids. This case highlights the difficulty of diagnosis and treatment of seronegative lupus nephritis.

Lupus

ANA screening yields positive results in many connective tissue disorders and other autoimmune diseases, and may occur in normal individuals. Subtypes of antinuclear antibodies include anti-Smith and anti-double stranded DNA (dsDNA) antibodies (which are linked to SLE) and anti-histone antibodies (which are linked to drug-induced lupus). Anti-dsDNA antibodies are highly specific for SLE; they are present in 70% of cases, whereas they appear in only 0.5% of people without SLE. The anti-dsDNA antibody titers also tend to reflect disease activity, although not in all cases. Other ANA that may occur in people with SLE are anti-U1 RNP (which also appears in systemic sclerosis and mixed connective tissue disease), SS-A (or anti-Ro) and SS-B (or anti-La; both of which are more common in Sjögren's syndrome). SS-A and SS-B confer a specific risk for heart conduction block in neonatal lupus.

InternalMed_Harrison

Fever ˜38.3° C (101° F) and illness lasting ˜3 weeks and no known immunocompromised state History and physical examination Stop antibiotic treatment and glucocorticoids Obligatory investigations: ESR and CRP, hemoglobin, platelet count, leukocyte count and differential, electrolytes, creatinine, total protein, protein electrophoresis, alkaline phosphatase, AST, ALT, LDH, creatine kinase, antinuclear antibodies, rheumatoid factor, urinalysis, blood cultures (n=3), urine culture, chest x-ray, abdominal ultrasonography, and tuberculin skin test

InternalMed_Harrison

Fever >38.3°C (101°F) on at least two occasions 2. Illness duration of ≥3 weeks 3. 4. Diagnosis that remains uncertain after a thorough history-taking, physical examination, and the following obligatory investigations: determination of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level; platelet count; leukocyte count and differential; measurement of levels of hemoglobin, electrolytes, creatinine, total protein, alkaline phosphatase, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, creatine kinase, ferritin, antinuclear antibodies, and rheumatoid factor; protein electrophoresis; urinalysis; blood cultures (n = 3); urine culture; chest x-ray; abdominal ultrasonography; and tuberculin skin test (TST). The range of FUO etiologies has evolved over time as a result of changes in the spectrum of diseases causing FUO, the widespread Percentage of Cases Due to Indicated Cause

[Systemic lupus erythematosus: a clinical and immunological study of 300 patients].

The aims of the present study were to analyze the clinical and immunologic characteristics of a wide group of patients with systemic lupus erythematosus (SLE) and define homogeneous subgroups with their own characteristics. A prospective study of 300 patients diagnosed of SLE were studied. These patients were subdivided according to sex, age at the onset of the disease and immunologic profile. The statistical study was carried out by the chi (2), Fisher, Student's t and Mann-Whitney U tests. The series was made up of 266 (89%) females and 34 (11%) males. The mean age at onset of the disease was 31.8 +/- 14.6 years. In 48 (16%) patients the first manifestations appeared after the age of 50. Males were shown to present a lower prevalence of arthritis (59% vs 82% in woman, p < 0.005) and malar rash (29% vs 50%, p < 0.05), but had more cutaneous discoid lesions (18% vs 3% p < 0.001). In patients in whom the disease appeared after the age of 50 a lower prevalence of arthritis was presented (67% vs 82% in patients of less than 50 years of age, p < 0.005), malar rash (23% vs 53%, p < 0.001) and nephropathy (21% vs 41%, p < 0.05), but had greater myositis (17% vs 6%, p < 0.01). The absence of antinuclear antibodies (ANA) and the presence of anti-ds DNA and anti-ENA antibodies were associated with differences in the prevalence of different clinical manifestations. Sex, age and immunologic pattern in systemic lupus erythematosus permit the definition of homogeneous subgroups with their own characteristics: a) males present a lower prevalence of arthritis and malar rash, but a greater prevalence of cutaneous discoid lesions; b) patients over the age of 50 develop arthritis, malar rash and nephropathy with a lower prevalence but have a greater prevalence of myositis; c) patients without antinuclear antibodies and those with anti-ds DNA and anti-ENA antibodies present differences in the prevalences of different clinical manifestations.

Pediatrics_Nelson

Cellular casts (RBC, granular, or tubular) Hemolytic anemia with reticulocytosis or Leukopenia (<4000 on two occasions) or Lymphopenia (<1500 on two occasions) or Thrombocytopenia (<100,000/mm3) Evidence of presence of antiphospholipid antibodies IgG or IgM anticardiolipin antibodies or Lupus anticoagulant or False-positive VDRL for >6 mo Positive ANA in absence of drugs known to induce lupus ANA, Antinuclear antibody; RBC, red blood cell; VDRL, Venereal Disease Research Laboratory. *These are the 1997 revised criteria for diagnosing systemic lupus erythematosus (SLE). A patient must have 4 of the 11 criteria to establish the diagnosis of SLE. These criteria may be present at the same or at different times during the patient’s illness. Additional, less specific diagnostic manifestations are noted in Table 90-2. proteinuria or hematuria to gross hematuria, nephrotic syndrome, and renal failure. Hypertension or the presence of edema suggests lupus renal disease.

Autoimmune manifestations of visceral leishmaniasis in Chinese patients.

Visceral leishmaniasis (VL) is a rare, parasitic infection with distinctive features that may mimic autoimmune diseases. In this study, we report on the laboratory autoimmune manifestations of VL in Chinese patients. Twenty-seven patients who were hospitalized with VL were included in this retrospective study. Routine blood and biochemical tests were conducted, and a variety of autoimmune antibodies and complement fractions were detected. Continuous variables are expressed as means ± standard deviations, and categorical data are expressed as a number (percentage). Missing data were not included for statistical analysis. Data were analyzed with SPSS v. 13.0 statistical software. All patients had cytopenia (82% with pancytopenia) and hepatosplenomegaly, and 25 (93%) patients also suffered from fever. The prevalence of autoantibodies (number of patients with antibody detected/total number of patients tested for the antibody) for each antibody tested was as follows: antinuclear antibodies (ANA; 18/22, 82%), anti-neutrophil cytoplasmic antibodies (ANCA; 4/5, 80%), anti-mitochondrial M2 antibodies (AMA-M2; 1/6, 17%), anti-liver cytosol specific type 1 antibodies (anti-LC1; 1/6, 17%), anti-liver/kidney microsomal type 1 antibodies (anti-LKM1; 1/6, 17%), anti-centromere protein-B antibodies (anti-CENP-B; 4/21, 19%), anti-Sjögren's syndrome type A antibodies (anti-SSA; 2/21, 10%), anti-Sjögren's syndrome type B antibodies (anti-SSB; 1/21, 5%), anti-Jo-1 antibodies (1/21, 5%), anti-double-stranded DNA antibodies (anti-dsDNA; 1/25, 4%), direct antiglobulin test (direct Coombs; 6/6, 100%), and rheumatoid factor (RF; 3/11, 27%). Increased serum C-reactive protein (CRP) was found in 14 (100%) patients. Of the 19 patients tested for serum IgG, 17 patients (89%) were found with increased IgG levels, while complement 3 protein (C3) and complement 4 protein (C4) levels were not decreased in any of the 19 patients. Of note, in one patient followed up 1 month after therapy, only ANA was still present, and all the other laboratory autoimmune manifestations had disappeared. VL infection associated with laboratory autoimmune manifestations is common. This may lead to patients with VL being misdiagnosed as having an autoimmune disorder. An obligatory differential diagnosis that considers VL should be undertaken for patients diagnosed with systemic lupus erythematosus (SLE), especially in endemic areas, is necessary.

[Clinical and serological characteristics of systemic lupus erythematosus: 128 cases].

To analyse the clinical and serological characteristics of systemic lupus erythematosus (SLE) in the center of Tunisia. We studied 128 patients with SLE aged one to 73 years. Antinuclear antibodies (ANA) were detected by an immunofluorescence method. Anti-double-stranded DNA (anti-dsDNA) antibodies, anti-extractable nuclear antigen antibodies (anti-Sm, anti-SS-A, anti-SS-B and anti-RNP) and anti-cardiolipin (aCL of IgG, IgA and IgM isotypes) antibodies were detected by ELISA. Malar rash (71%) and anemia (71%) were the most common clinical manifestations. Arthritis was seen in 62.5%. Severe kidney damage was observed in 39%. Pericarditis and pleuritis were observed in only 23%. Neurological manifestations (16%) were uncommon. Clinical manifestations of anti-phospholipid syndrome (SAPL) were observed in 15%. ANA were detected in 100%, anti-dsDNA in 76%, anti-Sm in 55.5%, anti-SS-A in 64%, anti-SS-B in 33.6%, anti-RNP in 49%. aCL of IgG, IgA and IgM isotypes were detected in 63.5%, 49% and 40.6% of the patients respectively. The only significant positive clinical associations were those of arthritis with anti-dsDNA antibodies (p = 0.022) and malar rash with anti-SS-A antibodies (p = 0.002). This study suggests that tunisians with SLE present, in general, a mild form of disease predominantly manifested by cutaneous, musculoskeletal and hematologic involvement but low prevalence of major organ damage.

[A case of systemic lupus erythematosus presenting with myelofibrosis as a cause of pancytopenia].

A 54 year-old woman who had a 6 month history of polyarthralgias, oral ulcers, weight loss and fatigue was admitted to the Urawa Municipal Hospital. She developed high fever, dyspnea and thrombocytopenia. Chest radiograph revealed massive right pleural effusion. At this time, laboratory investigations gave the following results: hemoglobin 12.7 g/dl, WBC 7700/microliters and platelet count 9.2 x 10(4)/microliters. Antibody to DNA was negative. Antinuclear antibody was positive at a titer of 320x in a centromere pattern; Anti-RNP and anti-Sm antibodies were negative. CH50 was 18.6 u/ml. C3 was 42.9 mg/dl. C4 was 11.5 mg/dl. Circulating immune complex (Clq) was 30.5 micrograms/ml. Circulating lupus anti-coagulant and anticardiolipine antibodies were positive. Thoracocentesis was performed; the material was a straw-colored exudate with over two thousands white cell per ul and showed marked reduction of complement titiers and elevated immune complex levels. She was then diagnosis as having SLE. Two weeks after admission, progressive leukopenia and anemia succeedingly occurred and resulted in severe pancytopenia. Bone marrow biopsy demonstrated marked marrow fibrosis and increased reticulin content with no evidence of malignancy. Steroid pulse therapy for 3 days started, and subsequently she was treated with 60 mg/day of prednisolone. Three weeks after starting on steroids, the massive pleural effusion was completely disappeared and complement titiers were normalized. Circulating immune complex has not been detected any more. After 8 weeks, the peripheral blood count was normalized. The dose of prednisolone was reduced progressively. On this occasion, the biopsy showed normocullular marrow with a marked reduction in the amount of reticulin.(ABSTRACT TRUNCATED AT 250 WORDS)

Nephritis as an initial diagnosis of lupus in Nigerian patients.

Systemic Lupus Erythematosus (SLE) is a multi-systemic autoimmune disease. Renal involvement is a common complication, causing considerable mortality and morbidity. SLE is rarely reported among black Africans, though recent reports from Nigeria indicate otherwise. Nephritis, though a common complication of SLE has rarely been reported as the initial diagnosis of lupus among black Africans. The aim of our study is to highlight the clinical, laboratory and histological features in Nigerian patients presenting with features of nephritis and subsequent diagnosis of SLE. This is a three year prospective study of patients with renal diseases, who were admitted to the rheumatology and nephrology units of the Lagos State University Teaching Hospital (LASUTH) Ikeja. Serology, biochemical, haematologic tests, kidney biopsy were done. Twelve patients were studied (F11; M1); mean age 30.4 years (SD ± 9.8); mean illness 8 weeks (SD ± 6.6). Six patients had a nephritic condition. Nine of the patients had baseline hypertension while 3 had a rapidly progressive nephritis. Two patients had baseline End Stage Renal Disease (ESRD). All had dipstick proteinuria 2+/3+, mean protein creatinine ratio 2.2 (SD ± 0.6), mean 24hr protein 2.8 gm (SD ± 2.7); more than 10 red blood cells/hpf haematuria (n-6), hyaline casts (n-5), granular casts (n-2), mean GFR 31.4 ml/ min (SD ± 21.3.), mean serum creatinine 6.9 mg/dl (SD ± 5.3); mean urea 138.8 mg/dl (SD ± 56.2). For the serology, Anti Nuclear Antibody (ANA) was positive in all the 12 subjects; positive anti dsDNA -10 patients; ENA - 10. Renal biopsy showed mostly WHO/ISSN classes III, IV and V. Treatment was with Euro Lupus regimen and rituximab/ cyclophosphamide. Four patients had dialysis. In terms of the outcome, there were 3 deaths while 9 patients were discharged. A high index of suspicion is needed to diagnose Lupus Nephritis in black Africans especially when their presentations do not fulfil the America College of Rheumatology (ACR) diagnostic criteria for SLE.

InternalMed_Harrison

sedimentation rate (ESR) as part of the evaluation for mimics, but unless the value is very high, such nonspecific testing in the absence of other features is unlikely to clarify the situation. Routine screening with an antinuclear antibody (ANA) test is also unlikely to be informative in isolation and is frequently positive at low titers in otherwise healthy adults. Additional unfocused studies, such as whole-body imaging scans, are usually not indicated; in addition to their inconvenience, potential risk, and cost, they often reveal unrelated incidental findings that can prolong the workup unnecessarily.

Pauci-immune lupus nephritis: a case report.

A 26-year-old female with systemic lupus erythematosus was admitted because of dyspnea and progressive lower extremity edema. Laboratory testing showed blood urea nitrogen levels of 147 mg/dL, creatinine of 6.7 mg/dL, serum albumin of 1.7 g/dL and the daily protein loss was 12.7 g. Her C3 level was 60.4mg/dL and C4 level was 10.2 mg/dL. The antinuclear antibody titer was 1:320, with a homogeneous pattern, but she was negative for anti-dsDNA. ELISA testing for anti-PR3 antibodies and anti-MPO antibodies were all negative. She was also negative for circulating lupus anticoagulant. Renal biopsy revealed diffuse proliferation of glomerular cells, but immunofluorescent microscopy showed no immune deposits and electron microscopy revealed only scanty electron-dense deposits. She received 1 g/day of methylprednisolone intravenously for 3 days, followed by 60 mg/day of prednisolone. She was discharged with serum creatinine decreased to 4.7 mg/dL, and a great improvement in dyspnea. Diffuse proliferative lupus nephritis that contains little or no subendothelial deposits is rare. The differential diagnosis, possible mechanisms and treatment are discussed.

Diagnostic and prognostic significance of different antinuclear antibodies in more than 1000 consecutive Albanian patients with rheumatic diseases.

In an unselected population of 1390 consecutive Albanian patients with rheumatic diseases (RD) and other miscellaneous non-rheumatic diseases (MNRD), for whom antinuclear antibody (ANA) testing was requested, we calculated the diagnostic sensitivity, specificity and positive predictive value (PPV) of ANA positive results, ANA titres over 1:100, anti-native DNA (nDNA), anti-Sm, anti-U1 RNP, anti-SSA (Ro) anti-SSB (La) and anti-non-identified extractable nuclear antigen (NIENA) antibodies. The PPVs of these ANA types were found to be appreciable only for systemic lupus erythematosus (SLE); only the positive predictive value of ANA for SLE (26.4%) was lower than that for RA (34.3%). The anti-snRNP (Sm/U1RNP) positive SLE patients were more likely to have over 4 of the ARA criteria for SLE, ANA titres over 1:100, and anti-nDNA antibodies, in contrast with the anti-snRNP negative subgroup. On the other hand, the anti-ENA positive and anti-nDNA positive SLE patients generally showed higher frequencies of renal disease, over 4 of the criteria for SLE and ANA titres over 1:100, compared to anti-ENA positive and anti-nDNA negative patients. Our data suggest that the association of anti-snRNP antibodies with a more severe form of SLE is not to be attributed to these antibodies themselves, but rather to their close association with the concomitant presence of anti-nDNA antibodies.

The initial manifestations and final diagnosis of patients with high and low titers of antinuclear antibodies after 6 months of follow-up.

The antinuclear antibody (ANA) test is the most commonly used test to screen for autoimmune diseases. However, only a limited numbers of studies have addressed the characteristics of patients positive for ANA. In this study, we aimed to clarify the relationship between initial presentations, ANA titer, and final diagnoses. Patients who visited National Taiwan University Hospital and received a first ANA test were enrolled and then followed for a further 6 months. The symptoms and signs at the time of ANA testing, ANA titers, and the final diagnoses were recorded and analyzed. A total of 355 patients were positive for ANA. Joint pain was the most common initial presentation at the time of ANA testing. Compared with the patients with low ANA titers (<1:640), those with high ANA titers (≥ 1:640) were more susceptible to autoimmune diseases. More importantly, of the patients with initial presentations of joint pain, fever, abnormal urinalysis, or skin rash/skin tightness, autoimmune diseases were more frequently diagnosed in those with high ANA titers than with low ANA titers (p<0.05). In addition, both anti-double strand DNA antibodies and anti-extractable nuclear antibodies were more commonly detected in patients with high ANA titers. A high ANA titer seems to be a useful biomarker for the diagnosis of autoimmune diseases, especially for patients presenting with joint pain, fever, abnormal urinalysis, or skin rash/skin tightness.

Lupus

Other than the ACR criteria, people with lupus may also have: fever (over 100 °F/ 37.7 °C) extreme fatigue hair loss fingers turning white or blue when cold (Raynaud's phenomenon) Criteria for individual diagnosis Some people, especially those with antiphospholipid syndrome, may have SLE without four of the above criteria, and also SLE may present with features other than those listed in the criteria. Recursive partitioning has been used to identify more parsimonious criteria. This analysis presented two diagnostic classification trees: Simplest classification tree: SLE is diagnosed if a person has an immunologic disorder (anti-DNA antibody, anti-Smith antibody, false positive syphilis test, or LE cells) or malar rash. It has sensitivity = 92% and specificity = 92%. Full classification tree: Uses 6 criteria. It has sensitivity = 97% and specificity = 95%. Other alternative criteria have been suggested, e.g. the St. Thomas' Hospital "alternative" criteria in 1998.

A case of juvenile Sjögren's syndrome with interstitial nephritis.

Primary Sjögren's syndrome (SS) is a rare autoimmune disease, especially in children. Juvenile primary SS with interstitial nephritis is rare in Japan. We report on a 12-year-old girl in whom salivary gland swelling had recurred from the age of 5 years, SS was diagnosed at the age of 10 years, and interstitial nephritis developed at the age of 12 years. The patient presented with a chief complaint of swelling of both parotid glands. The patient had a history of recurrent parotitis from 5 years of age, with episodes recurring 5 to 6 times a year and resolving within 3 days each time. However, at the age of 11 years, the patient had continuous mild swelling of the parotid glands. Examination on admission showed bilateral nontender parotid gland swelling; mild swelling of the lower extremities, xerostomia, and xerophthalmia but no exanthem. Laboratory findings were as follows: serum protein, 10.1 g/dL; immunoglobulin (Ig) G, 3,828 mg/dL; antinuclear antibodies, 1,280-fold; anti-Ro/SS-A antibody, 512-fold; anti-Ro/SS-B antibody, 4-fold; creatinine, 0.45 mg/dL; blood β2-microglobulin, 2.2 mg/L (slightly elevated); and cystatin C, 0.86 mg/L. Urinalysis showed proteinuria and a β2-microglobulin concentration of 11,265 mg/L. Thus, this patient had low molecular weight proteinuria. Schirmer's test showed decreased tear secretion (5 mm), and fluorescein staining showed marked bilateral superficial punctate keratitis. A lip biopsy showed infiltration by small round cells (mild to moderate), interstitial fibrosis, loss of salivary gland parenchyma, and atrophy, with no obvious epimyoepithelial islands, leading to a diagnosis of SS. Light microscopic examination of the renal biopsy specimens showed expansion of mononuclear cell infiltration in the renal interstitium, inflammatory cell infiltration of interstitial areas with edema and mild fibrosis, and tubulitis and mononuclear cell infiltration that included many lymphocytes and plasma cells. There were no pathological findings of glomerulonephritis. Small arteries showed no obvious abnormalities. Immunofluorescent staining showed slight, nonspecific deposition of IgM, but no deposition of IgG, complement 1q, 3, or 4. On the basis of the renal biopsy showing nonspecific chronic interstitial nephritis, renal tubular atrophy, and interstitial enlargement, tubulointerstitial nephritis associated with SS was diagnosed.

Autoantibodies in lupus nephritis useful in distinguishing proliferative nephritis from membranous nephritis.

This study was conducted to determine autoantibodies associated with lupus nephritis, especially those useful in diagnosing proliferative and membranous nephritis. 106 patients with lupus nephritis and 63 patients with SLE but no nephritis were enrolled. 55 patients were selected from the lupus nephritis group and were divided into two groups: proliferative nephritis patients (n=36) and membranous nephritis patients (n=19). The autoantibody profiles of patients' sera were evaluated using the EUROLINE ANA profile 3 (IgG) kit. A higher positivity rate of anti-dsDNA antibody and anti-histone antibody were seen in lupus nephritis patients compared to non-renal SLE patients. In comparing between proliferative and membranous nephritis, positivity of anti-nucleosome antibody was higher in proliferative nephritis compared to membranous nephritis, although it was not statistically significant. However, anti-nucleosome antibody-positive patients with lupus nephritis had higher prevalence of hematuria and pyuria, which are strong indications of proliferative nephritis. Also, a significantly higher positivity rate of anti-RNP70 antibody was seen in membranous nephritis compared to proliferative nephritis. Our results showed anti-nucleosome antibody and anti-RNP70 antibodies may be predictive non-histological factors for discriminating between proliferative and membranous lupus nephritis.

When Coke Is Not Hydrating: Cocaine-Induced Acute Interstitial Nephritis.

A 47-year-old African American man was admitted with 4 days of back pain, nausea and vomiting, and low urine output. There was no history of fever, dysuria, frequency, hesitancy, viral symptoms, trauma, rash, or constipation. Despite his past medical history of hypertension, diabetes mellitus, and hyperlipidemia he denied taking any medications for 18 months, including nonsteroidal anti-inflammatory drugs, acetaminophen, or antacids. He denied smoking and alcohol but admitted to cocaine use. No significant FH. Physical examination results were as follows: BP 235/125 mm Hg, heart rate 90 beats/min, temperature 98°F, O2 saturation normal; lungs and heart normal, abdomen soft but bilateral costovertebral angle tenderness. Neurological examination was normal. Laboratory tests yielded the following results: creatinine (Cr) 10.5 mg/dL (1.2 mg/dL in 2010), blood urea nitrogen 63 mg/dL, glucose 151 mg/dL, Ca 9.4 mg/dL, PO4 6.1 mg/dL, Hgb 15 g/dL, white blood cells (WBC) 9100, platelets 167 000, amylase/lipase normal, aspartate aminotransferase/alanine aminotransferase (AST/ALT) normal, bilirubin 1.4 mg/dL, alkaline phosphatase 39 IU/L, creatine phosphokinase 127 µg/L. Hepatic panel, C- and P-ANCA (cytoplasmic- and perinuclear-antineutrophil cytoplasm antibodies, respectively), anti-GBM (anti-glomerular basement membrane), antimyeloperoxidase, antinuclear antibody, and Helicobacter pylori were all negative. C3, C4 normal, urinalysis: 2+ blood, no white blood cells or eosinophils, no casts, no albumin, negative for nitrate/leukocyte esterase and bacteria. Imaging: chest radiograph, abdominal radiograph, computed tomography of the abdomen, electrocardiography, and transthoracic echocardiography were all normal. Course. The patient's urine output declined from 700 to 400 cm(3)/d and the on third day he required hemodialysis with Cr 14 mg/dL. Renal biopsy showed typical findings of interstitial nephritis. The patient was dialyzed for 10 days and responded to steroids and went home with an improving Cr of 3.5 mg/dL, back to baseline of 1.5 in 8 weeks. Discussion. Internists encounter patients with acute kidney injury (AKI) on a daily basis, most of which can be explained by prerenal azotemia, acute tubular necrosis (ATN), obstruction, or rhabdomyolysis among other etiologies. Cocaine is only rarely implicated as an etiology of AKI and if it is, usually the injury is due to ATN or pigment effects. Acute interstitial nephritis (AIN) caused by cocaine, on the other hand, has only been described in a handful of cases. AIN is a renal lesion that causes a decline in creatinine clearance and is characterized by an inflammatory infiltrate in the kidney interstitium and is most often associated with drug therapy. AIN can also be seen in autoimmune disorders like systemic lupus erythematosus, Sjögren's syndrome, or sarcoidosis; or with infections remote to the kidney like Legionella, leptospirosis, and streptococcal disease. Our case was very similar to the other reported cases of AIN due to cocaine in that all have occurred in middle-aged African American males and all have responded to steroids. This case reminds clinicians to consider AIN in patients with AKI and a history of cocaine abuse.

New-onset lupus nephritis after male-to-female sex reassignment surgery.

We report an original case of a 27-year-old transgender woman who developed lupus nephritis after male-to-female sex reassignment surgery. The patient had been taking hormones to induce feminization since the age of 18. She was admitted with malar "butterfly" rash, anasarca and hypertension, associated with an increase in serum creatinine (1.7 mg/dl). Renal involvement was characterized by nephritic and nephrotic syndrome. Autoantibody tests were positive for antinuclear antibodies and anti-double-stranded DNA, and complement levels were markedly reduced. Renal biopsy demonstrated diffuse proliferative glomerulonephritis and granular immune complexes deposits with a "full-house" pattern at the immunofluorescence level. The induction treatment was realized with corticosteroid and cyclophosphamide and maintenance immunosuppression phase with mycophenolate, obtaining complete remission. We speculated that lupus nephritis was induced by estrogens and antiandrogen therapy and gonadectomy. In the present case, we discuss the role of sex hormones in systemic lupus erythematosus onset and review the cases linked to transgender patients.

[Curative effect of integrative medical therapy for systematic lupus erythematosus].

To observe the curative effect of integrative medicine for treatment of systematic lupu erythematosus (SLE). Totally 110 cases of SLE were randomized into two groups, 50 in the control group and 60 in the treated group, both were treated with prednisone, but to the treated group, integrative medica treatment was given additionally according to syndrome differentiation. The course for both groups was 6 months. Clinical symptoms, immunological indexes, peripheral blood figure, erythrocyte sedimentation rate (ESR), levels of C-reactive protein (CRP) and creatinine (Cr) in blood, and 24 h urinary total protein (u-TP/24 h) were observed before and after treatment. Of the 60 patients in the treated group, the treatment on 29 was evaluated as clinical remission, 18 as remarkably effective, 9 as effective, and 4 as ineffective, the total effective rate being 93.33% (56/60), while that in the control group was clinical remission in 11, remarkably effective in 10, effective in 19, ineffective in 10 respectively, and the total effective rate 80% (40/50), the difference on total ef fective rate between the two groups was significant (chi2 = 4.36, P <0.05). Besides, the improvement in the treated group was superior to that in the control group in terms of clinical symptoms such as fever, arthralgia and baldness (P <0.05, P <0.01); the negative reversion rate of anti-nuclear antibody (ANA, 51.3% vs 28.1%), antidouble-stranded DNA (ds-DNA, 53.6% vs 26.1%), anti- ribonucleoprotein (RNP, 63.2% vs 29.4%) and circulating immnue complexes (CIC, 63.2% vs 29.4%, all P <0.05); lowering of immunoglobulin (for IgG, P < 0.05; IgA, P <0.01; IgM, P <0.01); as well as bettering in peripheral blood figure, ESR, CRP, Cr and u-TP/24 h (P <0.05 or P <0.01). Integrative medical treatment is obviously effective for SLE patients.

Polyarteritis nodosa

A patient is said to have polyarteritis nodosa if he or she has three of the 10 signs known as the 1990 American College of Rheumatology (ACR) criteria, when a radiographic or pathological diagnosis of vasculitis is made: Weight loss greater than/equal to 4.5 kg Livedo reticularis (a mottled purplish skin discoloration over the extremities or torso) Testicular pain or tenderness (occasionally, a site biopsied for diagnosis) Muscle pain, weakness, or leg tenderness Nerve disease (either single or multiple) Diastolic blood pressure greater than 90 mmHg (high blood pressure) Elevated kidney blood tests (BUN greater than 40 mg/dL or creatinine greater than 1.5 mg/dL) Hepatitis B (not C) virus tests positive (for surface antigen or antibody) Arteriogram (angiogram) showing the arteries that are dilated (aneurysms) or constricted by the blood vessel inflammation


Clearly the answer is 4, with a very characteristic clinic of temporary seizures.

Clearly the answer is [HIDDEN], with a very characteristic clinic of temporary seizures.

In a woman with an epileptic seizure presenting with the following clinical features: epigastric aura, unpleasant odor, disconnection from the environment, motor automatisms (sucking, swallowing, opening and closing of one hand) and postcritical amnesia, what is your diagnostic suspicion?

Generalized non-convulsive seizure or typical absence.

[Ictal semiology of temporal partial complex seizures: usefulness for localizing and lateralizing the origin of the attacks].

To review signs and symptoms associated with temporal complex partial seizures (CPS) and their utility in the localization and lateralization of seizure onset. CPS are particularly resistant to the standard antiepileptic drugs. Since surgical treatment is a therapeutical alternative in patients with intractable seizures, localization and lateralization of seizure origin are the principal aims in the preoperative assessment. Video-EEG monitorization has made possible characterization of ictal behaviour and correlation with cerebral regions generating the epileptic discharge. Therefore, ictal semiology has increased its importance in the localization and lateralization of seizures. The utility of auras, automatisms, motor manifestations, speech disturbances and autonomic features have been reviewed in relation to this approach. Viscerosensorial and experiential auras have been associated with temporal lobe epilepsy. The automatisms are not exclusive of temporal seizures and may be observed in frontal epilepsy and parietal and occipital seizures with spreading to temporal structures. There is not agreement in relation to head turning and version, therefore, this clinical sign should be used in correlation to other clinical manifestations. Distonic posturing, comprehensible ictal speech and postictal dysphasia appear to be the most reliable clinical signs in the lateralisation of temporal lobe seizures.

Semiology of temporal lobe epilepsies.

Temporal lobe epilepsies (TLE) represent the majority of the partial symptomatic/cryptogenic epilepsies. Excellent results of epilepsy surgery in well-selected patients have encouraged a search for localizing and lateralizing signs that could assist in the identification of the best surgical candidates. Seizure types in TLE include simple partial, complex partial and secondarily generalized seizures. Temporal lobe seizures most often arise in the amygdalo-hippocampal region. More than 90% of patients with mesial TLE report an aura, most commonly an epigastric sensation that often has a rising character. Other autonomic symptoms, psychic symptoms, and certain sensory phenomena (such as olfactory) also occur. The complex partial seizures of mesial TLE often involve motor arrest, oroalimentary automatisms or non-specific extremity automatisms at onset. Ictal manifestations that have lateralizing value include dystonic posturing (contralateral), early head turning (usually ipsilateral), and adversive head turning in transition to generalization (contralateral). Well-formed ictal language favors right temporal localization. Ictal vomiting, spitting, and drinking tend to be right sided. The duration of TLE complex partial seizures is generally greater than one minute and postictal confusion usually occurs. When postictal aphasia is noted a left-sided lateralization is favored. A lateral temporal onset is less common in TLE, and is most often suggested by an auditory aura. Somatosensory and visual auras are highly unlikely with TLE, and suggest neocortical extratemporal localization. A cephalic aura is non-specific, but is more common in frontal lobe epilepsy.

Psychomotor seizures of temporal lobe onset: analysis of symptom clusters and sequences.

We analyzed 91 psychomotor seizures from 31 patients seizure free at least one year after temporal lobectomy (implying temporal lobe onset). Fifty symptoms were looked for in every seizure and their time of onset and ending noted. Statistical analysis was used to define symptom clusters and the order of appearance of symptoms. Of the eighteen most common symptoms examined, all of these symptoms form a tight cluster showing a high degree of correlation. Within this cluster, there was a tendency towards the following subclusters: (a) epigastric aura, ictal vomiting, alimentary and hand automatisms; (b) behavioral arrest, complete loss of consciousness, staring and bilateral facial contraction; (c) unilateral dystonic posturing of an arm, mimetic automatisms, complex gestures, ictal speech and partial loss of consciousness; (d) looking around, agitation, vocalizations and whole body movements. We also found a strong correlation between epigastric sensation and ictal vomiting in psychomotor seizures arising from the right but not the left temporal lobe. The commonest sequence of symptoms was: behavioral arrest followed by alimentary and hand automatisms, looking around and whole body movements, in that order.

Temporal lobe epilepsy: clinical semiology and age at onset.

The objective of this study was to define the clinical semiology of seizures in temporal lobe epilepsy according to the age at onset. We analyzed 180 seizures from 50 patients with medial or neocortical temporal lobe epilepsy who underwent epilepsy surgery between 1997-2002, and achieved an Engel class I or II outcome. We classified the patients into two groups according to the age at the first seizure: at or before 17 years of age and 18 years of age or older. All patients underwent intensive video-EEG monitoring. We reviewed at least three seizures from each patient and analyzed the following clinical data: presence of aura, duration of aura, ictal and post-ictal period, clinical semiology of aura, ictal and post-ictal period. We also analyzed the following data from the clinical history prior to surgery: presence of isolated auras, frequency of secondary generalized seizures, and frequency of complex partial seizures. Non-parametric, chi-square tests and odds ratios were used for the statistical analysis. There were 41 patients in the "early onset" group and 9 patients in the "later onset" group. A relationship was found between early onset and mesial temporal lobe epilepsy and between later onset and neocortical temporal lobe epilepsy (p = 0.04). The later onset group presented a higher incidence of blinking during seizures (p = 0.03), a longer duration of the post-ictal period (p = 0.07) and a lower number of presurgical complex partial seizures (p = 0.03). The other parameters analyzed showed no significant differences between the two groups. We conclude that clinical and semiological differences exist between patients with temporal lobe epilepsy according to the age at onset. [Published with video sequences].

Focal seizure

Complex partial seizures A complex partial seizure is a seizure that is associated with unilateral cerebral hemisphere involvement and causes impairment of awareness or responsiveness, i.e. alteration of consciousness. Presentation Complex partial seizures are often preceded by an aura. The seizure aura is a focal aware seizure. The aura may manifest itself as a feeling of déjà vu, jamais vu, fear, euphoria or depersonalization. The aura might also occur as a visual disturbance, such as tunnel vision or a change in the perceived size of objects. Once consciousness is impaired, the person may display automatisms, such as lip smacking, chewing or swallowing. There may also be loss of memory (amnesia) surrounding the seizural event. The person may still be able to perform routine tasks such as walking, although such movements are not purposeful or planned. Witnesses may not recognize that anything is wrong. The person may or may not even realize that they experienced a seizure.

Clinical characteristics of complex partial seizures: a temporal versus a frontal lobe onset.

The site of origin of complex partial seizures (CPS) is predominantly in the temporal lobe, but frontal, parietal, and occipital lobes may also be involved. In order to correlate clinical behaviours with either a temporal or frontal site of origin, we analysed 211 seizures occurring during invasive monitoring in 26 patients with temporal lobe epilepsy (TLE) and in eight patients with frontal lobe epilepsy (FLE). Although leg movements, defined as thrashing, pedalling and kicking, hand posturing, facial twitching, sitting up, and tonic-clonic movements occurred more frequently in FLE, and hand automatisms were more frequent in TLE, no statistically significant difference was found between the two groups. When analysing only electrographic seizures that did not spread or propagated only to homologous controlateral lobes, leg movements and hand posturing were seen only with FLE, and oral automatisms only in TLE. Seizures of temporal lobe origin were longer in duration and had a higher frequency of auras. We therefore conclude that the reliability of clinical behaviour alone to predict the site of origin of an epileptic discharge is limited when the surface EEG is equivocal or the neuroradiologic evidence of a focus is not clear.

Mesial Temporal Lobe Epilepsy -- History and Physical -- Seizure Semiology

When there is preserved awareness, the patient can describe the occurrence of unusual sensations known as auras. Auras could be visceral, autonomic, gustatory, or affective symptoms. Patients commonly experience a rising epigastric sensation, nausea, and olfactory and/or gustatory hallucination. Psychic sensations can occur, such as depersonalization (out-of-body feeling), déjà vu (a feeling of familiarity), jamais vu (feeling of unfamiliarity), déjà entendu (hearing familiar sounds), or panoramic visions (a rapid recollection of episodes from the past). Dysphoric or euphoric feelings, fear, terror, anger, and other sensations can also occur. Often, the patients find the aura hard to describe. Autonomic features include piloerection, pallor/flushing, tachycardia, or pupillary dilatation. [24] With the loss of awareness, patients have a behavioral arrest and portray a blank staring facial appearance, which is followed by the development of oro/facial/alimentary automatisms such as lip-smacking, chewing, sucking, or swallowing, which is usually accompanied by ipsilateral gestural automatisms such as repetitive hand movements, picking and/or fidgeting behavior, disrobing and contralateral dystonic posturing of limbs. The presence of speech suggests non-dominant hemispheric seizure onset, but its absence is not a reliable lateralizing finding. Patients commonly have a period of postictal confusion following the episode. Less commonly, episodes may progress to generalized tonic-clonic seizures.

EEG Localization Related Epilepsies -- Clinical Significance -- Temporal Lobe Epilepsy (TLE)

Seizure semiology: Auras: Visceral sensations and fear are associated with mesial temporal lobe epilepsy, while auditory and vertiginous auras have been associated with the neocortical temporal lobe epilepsy. Neocortical temporal lobe epilepsy: At seizure onset, patients with neocortical TLE are more likely to describe any type of hallucination or illusion. Automatisms and dystonic posturing are not found in neocortical TLE. [32] [35] Mesial temporal epilepsy: Patients present with a behavioral arrest that is observed as a blank facial expression along with loss of awareness. This change is followed by oral, facial, or alimentary automatisms such as lip-smacking, chewing, sucking, or swallowing or is accompanied by ipsilateral automatisms such as repetitive hand movements, picking, or fidgeting behavior, and contralateral abnormal posturing of limbs. Patients commonly have a period of postictal confusion. Rarely, this progresses to secondary generalization. [36]

Temporal lobe epilepsy

These may be: Sensations such as déjà vu (a feeling of familiarity), jamais vu (a feeling of unfamiliarity) Amnesia of a single memory or set of memories A sudden sense of unprovoked fear and anxiety Nausea Auditory, visual, olfactory, gustatory, or tactile hallucinations; olfactory hallucinations often seem indescribable to patients beyond "pleasant" or "unpleasant" Visual distortions such as macropsia and micropsia Dissociation or derealisation Synesthesia (stimulation of one sense experienced in a second sense) Dysphoric or euphoric feelings, fear, anger, and other emotions Focal aware seizures are often called "auras" when they serve as a warning sign of a subsequent seizure. Regardless, an aura is actually a seizure itself, and such a focal seizure may or may not progress to a focal impaired awareness seizure. People who experience only focal aware seizures may not recognize what they are, nor seek medical care.

Complex partial status epilepticus

Complex partial status epilepticus (CPSE) is one of the non-convulsive forms of status epilepticus, a rare form of epilepsy defined by its recurrent nature. CPSE is characterized by seizures involving long-lasting stupor, staring and unresponsiveness. Sometimes this is accompanied by motor automatisms, such as eye twitching. Diagnosis As is the case with other non-convulsive status epilepticus forms, CPSE is dangerously underdiagnosed. This is due to the potentially fatal yet veiled nature of the symptoms. Usually, an electroencephalogram, or EEG, is needed to confirm a neurologist's suspicions. The EEG is also needed to differentiate between absence status epilepticus (which affects the entire brain), and CPSE, which only affects one region. Treatment Treatment is in the form of anti-epileptic drugs, such as barbiturates, benzodiazepines and topiramate. References External links Medical emergencies Epilepsy

Complete seizure freedom is possible in patients with MTLE-HS after surgery in spite of extratemporal electro-clinical features.

The aim was to identify the common typical and atypical ictal semiologic and EEG features of patients with mesial temporal lobe epilepsy and hippocampal sclerosis who became seizure-free postoperatively. The semiologic and EEG findings of 126 ictal video-EEG recordings of 50 patients who remained seizure-free for at least 2 years (mean=9.46±3.20; range=3-14.5 years) after surgery were reviewed. Statistical analysis was used to determine the frequencies of the most common auras, semiologic features and EEG patterns and to define the symptom clusters. Aura was reported in 82% (n:41) of patients and the most common type was epigastric sensation. Early symptoms were contralateral upper extremity dystonic posturing (66%), oro-alimentary automatisms (50%) and ipsilateral hand automatisms (40%). Ictal EEG findings revealed localized and lateralized activity in 62% of patients. Of the 17 most common early symptoms, there was a tendency toward two clusters. Cluster 2 consisted of findings that were typical for patients with mesial temporal lobe epilepsy whereas Cluster 1 mostly consisted of atypical findings. This study demonstrated two clusters of the semiologic repertoire, and the most frequent EEG finding was lateralized and localized ictal activity, while other types of EEG patterns were also recorded. Although the presence of atypical findings may suggest a more extended or even extratemporal zone, the seizure-free outcome demonstrated that if the critical amount of tissue can be resected within the epileptogenic network, this may be sufficient for termination of seizures.

[A case with frontal lobe epilepsy presenting with absence seizures as cardinal manifestation: ictal EEG findings].

We report here a 9-year-old boy presenting with absence and complex partial seizures. Absence seizures occurred several times a day, with sudden arrest of speech and gesture, alteration of consciousness, myoclonus of unilateral or bilateral angles of the mouth, occasional simple automatism and brisk recovery of consciousness. Complex partial seizures occurred once to three times a month with loss of consciousness, salivation, deviation of the head and eyes toward the left, elevation of upper limbs and tonic convulsion of the left upper and lower limbs. Interictal EEG showed right frontal pole-dominant high-voltage slow waves or spike-and-waves. Ictal simultaneous video-EEG recordings of absence seizures revealed a frontal dominant 3-3.5 Hz spike-wave burst lasting several seconds. A partial seizure never preceded the absence seizure. Transverse topographical analysis revealed that the first spike component of the spike-wave burst of absence seizure always showed phase reversal on the right anterior temporal electrode. The following ones, however, showed phase reversal on the left anterior temporal electrode. Ictal EEG of the complex partial seizure could not be detected because it rarely occurred. There was no abnormal finding on brain MRI. Interictal single photon emission tomography (SPECT) indicated hypoperfusion of the dorsal and medial cortex of the right middle frontal lobe. Interictal positron emission tomography (PET) also indicated hypometabolic areas in the dorsal and medial cortex of the right frontal lobe, together with those in the right temporal and parietal cortex. EEG evolution and neuroimaging studies suggested that the epileptic focus of the absence seizure might have originated at the dorsal cortex of the right middle frontal lobe and immediately spread to the medial cortex. Both the seizures were well controlled by the combination of phenytoin and high dose sodium valproate.

Focal Impaired Awareness Seizure -- Introduction -- Seizures of Temporal Lobe Origin

These are the most common type of focal impaired awareness seizures. Stereotyped automatisms occur in about 40% to 80% of patients with temporal lobe epilepsies. Seizures with predominantly oral and manual automatism and some other motor manifestations are highly suggestive of temporal lobe origin. About 60% of temporal lobe seizures have a secondary generalization. Gradual recovery after several minutes of confusion occurs postictally in most patients; however, in some patients, automatic behavior like running or walking about, the non-directed violent behavior may occur. Temporal lobe focal impaired seizures can have features similar to frontal seizures, but temporal lobe focal impaired seizures typically have slower onset and progression and more pronounced confusion. Certain features can help in localizing the seizure onset to one hemisphere. Ictal vomiting, ictal speech, urinary urge, and automatisms with intact consciousness suggest seizure onset in the non-dominant hemisphere, and speech disturbance postictally is suggestive of seizure onset in the dominant hemisphere. Upper limb dystonia lateralizes seizure to the opposite hemisphere.

Seizure types

Focal seizures (Older term: partial seizures) Simple partial seizures – consciousness is not impaired With motor signs With sensory symptoms With autonomic symptoms or signs With psychic symptoms Complex partial seizures – consciousness is impaired (*Note: impaired does not necessarily mean [fully] lost) (Older terms: temporal lobe or psychomotor seizures) Simple partial onset, followed by impairment of consciousness With impairment of consciousness at onset Partial seizures evolving to secondarily generalized seizures Simple partial seizures evolving to generalized seizures Complex partial seizures evolving to generalized seizures Simple partial seizures evolving to complex partial seizures evolving to generalized seizures Generalized seizures Absence seizures (Older term: petit mal, meaning 'small, or smaller, bad') Myoclonic seizures Clonic seizures Generalized tonic–clonic seizures (Older term: grand mal, meaning 'great, or greater, bad') Atonic seizures

Clinical and EEG features of complex partial seizures of extratemporal origin.

Complex partial seizures (CPSs) of extratemporal origin are frequently misdiagnosed. Common reasons for diagnostic errors are failure to recognize the epileptic cause of the attacks or to appreciate localizing clinical seizure characteristics and nonspecific or misleading scalp EEG findings. This report describes clinical characteristics of two types of extratemporal CPSs, frontal and occipital. Frontal lobe CPSs are brief, frequent attacks that begin and end suddenly. They are dominated by complex, semi-purposeful motor automatisms, and vocalization and sexual automatisms are common. Their often bizarre appearance frequently leads to a misdiagnosis of hysteria. Occipital lobe CPSs are characterized primarily by their spread pattern, which can follow various paths. They may mimic temporal lobe CPSs, sensorimotor partial seizures, or supplementary motor partial seizures. Scalp EEGs are frequently misleading. The initial clinical symptoms are the most important clue to correct diagnosis and include elemental visual symptoms, visual loss, eye pulling or movement sensations in the absence of detectable movement, rapid forced blinking or eye flutter, and contralateral eye deviation.

Neurology_Adams

The motor components of a focal temporal lobe or limbic seizure, if they occur, arise during the later phase of the seizure and take the form of automatisms such as lip-smacking, chewing or swallowing movements, salivation, fumbling of the hands, or shuffling of the feet. Patients may walk around in a daze or act inappropriately. Complex acts that were initiated before the loss of consciousness—such as walking, chewing food, turning the pages of a book, or even driving—may continue. However, when asked a specific question or given a command, the patients are obviously out of contact with their surroundings. There may be no response at all, or the patient may look toward the examiner in a perplexed way or utter a few stereotyped phrases. The patient may walk repetitively in small circles (volvular epilepsy), run (epilepsia procursiva), or simply wander aimlessly, either as an ictal or postictal phenomenon (poriomania). These forms of seizure, according to some epileptologists, are

Temporal lobe epilepsy

These seizures tend to have a warning or aura before they occur, and when they occur they generally tend to last only 1–2 minutes. It is not uncommon for an individual to be tired or confused for up to 15 minutes after a seizure has occurred, although postictal confusion can last for hours or even days. Though they may not seem harmful, due to the fact that the individual does not normally seize, they can be extremely harmful if the individual is left alone around dangerous objects. For example, if a person with complex partial seizures is driving alone, this can cause them to run into the ditch, or worse, cause an accident involving multiple people. With this type, some people do not even realize they are having a seizure and most of the time their memory from right before or after the seizure is wiped. First-aid is only required if there has been an injury or if this is the first time a person has had a seizure.

[Ictal semiology in patients with medial temporal lobe epilepsy: value in lateralizing the seizure focus].

We have evaluated useful lateralizing signs in 28 patients with medial temporal lobe epilepsy who were seizure-free after anterior temporal lobectomy by reviewing videotapes during video-EEG monitoring. The most frequent types of aura were epigastric sensation and psychic symptom in 8, respectively, both of which did not predict lateralization of the focus. Of the motor signs, early head deviation and unilateral upper extremity automatism predicted an ipsilateral focus in 72 and 80%, respectively. On the other hand, late head deviation(< 15 seconds before secondarily generalized seizure) and unilateral upper extremity dystonic posturing predicted a contralateral focus in 80 and 100%, respectively. Twelve of the patients displayed oroalimentary automatism which did not predict focus lateralization. Three patients with ictal speech demonstrated a seizure focus contralateral to their language-dominant hemisphere. In medial temporal lobe epilepsy, several clinical seizure manifestations such as: early and late head deviation, unilateral upper extremity automatism and dystonic posturing were not a little noted and provided additional information as to the side of seizure origin.

Mesial Temporal Lobe Epilepsy -- Differential Diagnosis

The seizures in patients with MTLE share semiological characteristics with other types of epilepsies, such as absence seizures, insular seizures, and occipital lobe seizures. Patients with absence seizures also manifest with a blank stare and may have subtle automatisms which can mimic seizures of MTLE. [53] Patients with seizures arising from the insula can sometimes present in a very similar manner to TLE in terms of epigastric aura and oro-alimentary automatisms. [54] Some patients with occipital lobe epilepsy have seizures that rapidly spread anteriorly to the temporal lobes and can produce semiology very similar to TLE. [55]

Seizure types

Self limiting seizure types Generalized seizures Tonic-clonic seizures (includes variations beginning with a clonic or myoclonic phase) Clonic seizures (with and without tonic features) Typical absence seizures Atypical absence seizures Myoclonic absence seizures Tonic seizures Spasms Myoclonic seizures Massive bilateral myoclonus Eyelid myoclonia (with and without absences) Myoclonic atonic seizures Negative myoclonus Atonic seizures Reflex seizures in generalized epilepsy syndromes Seizures of the posterior neocortex Neocortical temporal lobe seizures Focal seizures Focal sensory seizures Focal motor seizures Gelastic seizures Hemiclonic seizures Secondarily generalized seizures Reflex seizures in focal epilepsy syndromes Continuous seizure types Generalized status epilepticus Generalized tonic-clonic status epilepticus Clonic status epilepticus Absence status epilepticus Tonic status epilepticus Myoclonic status epilepticus Focal status epilepticus

Complex partial seizures of temporal lobe origin in children of different age groups.

The semiology of complex partial seizures (CPS) of temporal lobe origin in adults is well known and is important in establishing seizure localization in patients considered for epilepsy surgery. In contrast, the behavioral features of temporal lobe seizures (TLS) in children described in the literature have not been consistent. In the present study, we investigated children with TLS to compare their attacks to TLS occurring in adults. The study was based on video recordings of 29 children with TLS aged 18 months to 16 years. Children were included, if they became seizure-free after temporal lobectomy (except 4 children with a marked reduction in seizure frequency and 1 with isolated auras), and if clear unitemporal seizure onset in ictal EEG-recordings, unilateral radiological lesions, and corresponding histopathological findings were detected. Children aged > 6 years had TLS with features similar to those of adults. In younger children, typical semiology included symmetric motor phenomena of the limbs, postures similar to frontal lobe seizures in adults, and head nodding as in infantile spasms. We concluded that the clinical features of TLS in younger children can be misleading and should therefore be considered with caution in selecting patients for surgical procedures on the temporal lobe.

Epilepsy EEG -- Definition/Introduction

Focal Seizures [2] In focal seizures, the onset of the seizure arises from one area of the brain. It also was previously known as a partial seizure. Focal seizure with intact awareness (previously called simple partial seizure): During the attack, the patient is fully alert and conscious. Electroencephalography performed during this condition rarely shows an ictal EEG discharge, but most of the time, scalp EEG cannot pick up the ictal activity. Focal seizure with impaired awareness (previously called complex partial seizure): During the attack, the patient develops a variable degree of impaired awareness, or consciousness gets altered. The clinical behavior of this condition will depend on region or lobe of onset. Combined Generalized and Focal Epilepsies [5]

Absence Seizure -- Differential Diagnosis

Focal epilepsy with alteration of awareness (previously called complex partial epilepsy) can also present with behavioral arrest and automatisms. However, these seizures are usually less frequent than absence seizures. Patients can have generalized seizures with focal epilepsy. The semiology of automatisms can vary depending on the area of the cerebral cortex where the seizures originate.

[Ictal automatisms during partial complex seizures in 36 children].

Partial complex seizures are relatively frequent type of epileptic seizures. These attacks are having aura in 60% of cases, that can be vegetative, motor, sensor or psychological one. Important is aura in the form of uncinatus seizures (unpleasant odor), because we must exclude tumor of temporal region in these patients. Altered consciousness comes after aura, patient has opened eyes, face can be pale or red, and automatism appear, in the form of different movements. These automatisms are often considered insignificant by parents or eyewitnesses. There is often misdiagnosis of this type of seizures, because only about 20% of standard EEG recordings find specific epileptic grapho-elements. Therapy of these seizures is difficult, with the success in only 50% of cases. Ictal automatisms were tested in the group of 36 children with partial complex seizures aged 4 to 17 years. Duration of epilepsy was 1 to 5 years. All patients had proved diagnosis of partial complex seizures, clinically and on electroencephalography, CT scan and MRI in medically intractable seizures. Eleven patients were videotaped during the attack. All patients had questionnaire filled by parents, about automatisms. Thirty-four patients (94.4%) had ictal automatisms, and 2 with epileptic focus in frontal region did not. Some patients had different types of automatisms. Most frequent ones were mimicking (15.19%), sitting-standing up (10.76%), swinging (8.23%), swallowing (7.59%) etc. It can be concluded that automatisms are almost obligatory part of partial complex seizure, and their registration is essential for correct diagnosis and treatment of these seizures.

Ictal semiology in hippocampal versus extrahippocampal temporal lobe epilepsy.

We have analysed retrospectively the clinical features and electroencephalograms in 35 patients with complex partial seizures of temporal lobe origin who were seizure-free after epilepsy surgery. Two groups were differentiated for statistical analysis: 16 patients had hippocampal temporal lobe seizures (HTS) and 19 patients had extrahippocampal temporal lobe seizures (ETS) associated with a small tumour of the lateral or inferior temporal cortex. All patients in the HTS group had ictal onset verified with intracranial recordings (depth or subdural electrodes). In the ETS group, extrahippocampal onset was verified with intracranial recordings in eight patients and assumed, because of failure of a previous amygdalohippocampectomy, in one patient. Historical information, ictal semiology and ictal EEG of typical seizures were analysed in each patient. The occurrence of early and late oral automatisms and dystonic posturing of an upper extremity was analysed separately. A prior history of febrile convulsions was obtained in 13 HTS patients (81.3%) but in none with ETS (P < 0.0001, Fisher's exact test). An epigastric aura preceded seizures in five patients with HTS (31.3%) and none with ETS (P = 0.0135, Fisher's exact test), while an aura with experiential content was recalled by nine patients with ETS (47.4%) and none with HTS (P = 0.0015), Fisher's exact test). Early oral automatisms occurred in 11 patients with HTS (68.8%) and in two with ETS (10.5%) (P = 0.0005, Fisher's exact test). Early motor involvement of the contralateral upper extremity without oral automatisms occurred in three patients with HTS (18.8%) and in 10 with ETS (52.6%) (P = 0.0298, Fisher's exact test). Arrest reaction, vocalization, speech, facial grimace, postictal cough, late oral automatisms and late motor involvement of the contralateral arm and hand occurred with similar frequency in both groups. These observations show that the early clinical features of HTS and ETS are different.

Analysis of the initial ictal phenomenon in patients with temporal lobe epilepsy.

We aimed to assess the localizing value of the initial semiological element in temporal lobe epilepsy (TLE). Video-EEG-documented seizures of 97 adult TLE patients were studied in relation to seizure origin (left versus right; mesial versus extra-mesial). Strikingly, seizures with mesial onset started with very few ictal phenomena, while seizures of extra-mesial origin began with a larger variety of ictal elements. Furthermore, following noticeable distributions were observed for the mesial group: (i) aura was the most common initial ictal phenomenon in the total patient collective, occurring significantly more frequently in mesial than in extra-mesial seizure onset. Aura appeared most often in seizures of left mesial origin. (ii) Vocalization presented a trend towards mesial left seizure origin. (iii) Oral automatisms showed a trend towards mesial seizure origin. Following noticeable distribution was observed for the extra-mesial group: In patients without aura, restlessness as initial ictal phenomenon appeared exclusively in seizures of extra-mesial right origin. Finally, behavioral arrest showed a trend towards left-sided seizure origin. In conclusion, the initial ictal element may add useful information concerning differentiation of seizure onset in TLE.

Ictal oroalimentary automatisms with preserved consciousness: implications for the pathophysiology of automatisms and relevance to the international classification of seizures.

A patient showing seizures presenting ictal automatisms with preserved consciousness is reported. A 30-year-old, right-handed man with normal development and without family history of epilepsy was referred for surgical treatment of epilepsy. At 15 he began to have seizures, starting with an epigastric aura, occasionally developing automatisms (lip-smacking, chewing), sometimes followed by tonic-clonic convulsions. At the time of referral, he averaged six convulsive seizures per year and one nonconvulsive per week. His sleep EEG showed sharpened slow activity over the right anterior quadrant magnetic resonance imaging (MRI) showed a benign lesion in the mesial aspect of the right occipital lobe. Simultaneous video monitoring and intracranial EEG with subdural strips recording from the right temporal and occipital lobes was undertaken. During one seizure, he had pronounced oroalimentary automatisms while holding a conversation with a technician, answering her questions, and explaining details of his seizures. Memory of this event was preserved. At seizure onset, spike activity was seen at the mesial occipital strips. At midseizure, high-voltage sharpened delta was seen throughout the right hemisphere. Left-sided scalp electrodes remained relatively uninvolved. The lesion, a dysembryoplastic neuroepithelial tumour was removed. Surgery was followed by abolition of seizures described. Because it is agreed that complex partial seizures require impaired consciousness, a history of automatisms with retained consciousness usually suggests nonepileptic attacks. This case suggests that automatisms in epileptic seizures can take place with minimal loss of consciousness, particularly if there is widespread but unilateral involvement. The need for a revision of the International Classification is suggested.

Value and limitations of seizure semiology in localizing seizure onset.

Seizure semiology has been the foundation of clinical diagnosis of seizure disorders. This article discusses the value and the limitations of behavioral features of seizure episodes in localizing seizure onset. Studies have shown that some semiologic features of seizures are highly accurate in the hemispheric lateralization and lobar localization of seizures. There is good agreement between blinded reviewers in lateralizing video-recorded seizures in temporal lobe and extratemporal lobe epilepsies. However, seizure semiology alone should not be used to determine the site of seizure onset. Each semiologic feature may falsely localize seizure onset. Seizure semiology in some patients may signify the site of seizure propagation rather than origination. Moreover, seizure semiology may not be as reliable in multifocal epilepsies as it is in unifocal epilepsies. Many semiologic features of seizures of adults are often missing in seizures of children. Seizure semiology should be analyzed and integrated with EEG and neuroimaging data to localize the seizure focus. A sample of the recorded seizures should be shown to the patient's relatives or friends to verify that it is representative of habitual seizures.

[Psychomotor epilepsy: phenomenology, localization, pathogenesis and therapy].

Next to tonic-clonic seizures psychomotor (complex focal) seizures are the most common form of all epileptic seizures, except in infancy where they are seen rarely. Differently from generalised non convulsive seizures (like petit mal absences), their first appearance has no typical age limit, however, their proportion to other forms of seizures increases in adolescence and adults especially between the third and fifth decade of life. The main symptom is the disorder of consciousness which lasts at least more than half a minute, normally several minutes in completely distinct seizures, which doesn't begin abruptly and which often ends ill defined. This twilight attack is proceeded by an aura of sensory, psychic or vegetative character. The aura is followed either by a transitory state of immobility and later by motor phenomena or at once by motor phenomena in the form of diverse automatisms of variable intensity, reaching from mild movements in the oral region over verbal expressions to highly dramatic scenes, often accompanied by vegetative symptoms. Tonic versive and tonic symmetrical or tonic asymmetrical seizure symptoms are quite often motor variants which also can lead to sudden drops. Psychomotor attacks can be reduced to "pseudo-absences", however, they also can develop into tonic-clonic seizures (Grand mal). Generally, the succession of seizure symptoms is constant in the same patient, the expression can differ from seizure to seizure. Psychomotor attacks can be spread over the whole day or can show a strict connection to sleep, in the course they can likely occur in clusters and can accumulate to a continuous or discontinuous form of psychomotor status epilepticus. Predominantly, but not exclusively psychomotor attacks start from the temporal lobe, whereas neocortical temporal attacks (especially of lateral posterior origin) can be distinguished from those coming from the limbic system, especially from hippocampal or mesio-basal temporal structures and from the nucleus amygdalae. About 20% of the psychomotor attacks are of frontal origin coming from the mesial frontal region or from the gyrus cinguli anterior. Also seizures of occipital or parietal origin can spread so quickly that the seizure itself is impressing as a "temporal lobe attack". On account of that, epilepsies with psychomotor attacks cannot be compared to temporal lobe epilepsies. The etiology of psychomotor epilepsies is closely connected to the topographic site of the temporal lobe, who is especially vulnerable for traumatic lesions, cerebral edema and hypoxemia. Also small dysgeneses, heterotopies or small abnormalities of vessels are relatively often found in surgical specimens.(ABSTRACT TRUNCATED AT 400 WORDS)

Epilepsy

Signs and symptoms Epilepsy is characterized by a long-term risk of recurrent epileptic seizures. These seizures may present in several ways depending on the parts of the brain involved and the person's age. Seizures The most common type (60%) of seizures are convulsive which involve involuntary muscle contractions. Of these, one-third begin as generalized seizures from the start, affecting both hemispheres of the brain and impairing consciousness. Two-thirds begin as focal seizures (which affect one hemisphere of the brain) which may progress to generalized seizures. The remaining 40% of seizures are non-convulsive. An example of this type is the absence seizure, which presents as a decreased level of consciousness and usually lasts about 10 seconds.

[Quantitative clinical and EEG measures of epileptogenesis in temporal lobe epilepsy].

In this controlled, prospective and partially blind study two groups of patients with temporal lobe epilepsy were evaluated, respectively with good and bad prognosis. Measurements of epileptogenesis were based on frequency of seizures, and on epiletogenic electroencephalographic abnormalities obtained from scalp electrodes over the temporal lobes. The results were analysed by non-parametric analysis of variance, comparison of groups and analysis of correlation. The results indicated the temporal lobe groups were similar to at least one of the control groups in age, sex, educational and social level, therapeutic regime, age at onset and length of history of epilepsy. The quantitative measurements showed a global difference between the group of temporal lobe with bad and good prognosis, reaching statistical significance in clinical epileptogenesis, and a trend towards greater epileptogenesis on the electroencephalogram, in the same group of patients. The results indicate the experimental usefulness of some of the original measurements used in the study, but also their problems. A review of the literature is carried out.

The effect of age on seizure semiology in childhood temporal lobe epilepsy.

Complex partial seizure is the characteristic seizure type observed in epilepsy arising from temporal lobe structures. The seizure evolution in adult patients is quite stereotyped and well characterized, manifesting initially with an aura, behavioral arrest, and oroalimentary and gestural automatism. A greater variability of semiology including motor features with tonic or myoclonic components, as well as a paucity of automatism, has been reported in young children with temporal lobe epilepsy. The aim of our study was to examine in more detail the effects of age on individual ictal features to be able to determine the critical age when lesional temporal lobe seizure semiology undergoes transition from the pediatric to the more adult-type clinical pattern. We performed a video analysis of 83 seizures from 15 children (aged 11-70 months) selected by post-temporal lobectomy seizure-free outcome, looking specifically at the motor and behavioral (nonmotor) manifestations in relation to age of the children. All of the children younger than 42 months had seizures with early and marked motor features, which included tonic and myoclonic components and epileptic spasms. Parallel with age, the frequency of these motor components decreased, and in five of 11 children older than 3 years, motor features were totally absent. Analyzed quantitatively, we saw a linear and inverse correlation of the ratio of motor components with age at monitoring. These findings support the hypothesis that events in brain maturation significantly affect clinical seizure semiology and may override the more typical localizing features seen in adult-type temporal lobe epilepsy. These findings are important to consider in the early diagnosis of childhood temporal lobe epilepsy.