Spectroscopic correlation and role of Azathioprine in long-term remission in patients of Hashimoto encephalopathy
Dev Raj, a 45-year-old healthy male presented with abnormal brief shock-like movements of the body for 20 days with confused behavior for 1 week. The abnormal movements developed insidiously over 2 days without any stimulating factors and did not remit with sleep or anti-epileptics (valproate 1.5 gm and clobazam 40 mg divided doses), which he had been prescribed for approximately 2 weeks before seeking an admission at our institute. They occurred without any periodicity with a frequency of about 8-10 per minute and incapacitated the patient. There was no loss of consciousness or bowel and bladder tone. He gave no history of addictions of any kind.\nPatient was obese with a BMI of 34 kg/m2 and was seen to have loss of lateral third of eyebrows with dry and eczematous skin. Blood pressure was 170/100 mmHg. He had severe cognitive impairment on admission, scoring about 4/30 in MMSE. Speech was fluent and articulate but lacked insight and consistency. Cerebellar signs, motor and sensory examination could not be performed consistently, but reflexes were slow with a delayed relaxation phase observed in the ankle jerk. Plantar response was extensor in both limbs. There were no signs of meningeal or cranial nerve involvement.\nThere were no similar episodes noted in the patient in the past or any history of trauma. He did not suffer from any chronic cardiovascular, pulmonary, or neurologic conditions.\nSerum electrolytes, blood sugar, liver and renal function tests, and hematology profile were seen to be within normal range. However, his thyroid hormone levels were abnormal with an initial TSH of 46.04 IU/ml. MRI of brain was normal, but on spectroscopy, there was an evidence of a lactate peak corresponding to 1.3 μm in the right occipital area, suggestive of a focus of anaerobic metabolism in the brain. [Figures , ] There was a corresponding dip of N-acetyl aspartate in the corresponding area. There were no areas of hypoperfusion in the brain. EEG and brain mapping showed a background α activity with a 6-7 Hz slowing in the delta region with frontal predominance β-fast activity. [] The Anti-TPO (Thyroid peroxidase) antibody levels were 3195.70 units (normal values below 60.00 units). A lumbar puncture revealed mildly raised protein levels in cerebrospinal fluid at 95 mg%, with a sugar level of 84 mg%, and a total leukocyte count of 5/cumm. CSF sent for serology against common viruses did not reveal any viral titers and involvement, and Gram stain and oligo-clonal bands were negative. He was sero-negative for HIV, HBSAg, and anti-HCV. An ultrasonogram for thyroid showed bilateral lobes of thyroid to be small and atrophic with altered echotexture. The biopsy for thyroid tissue did not reveal any functional thyroid follicles.\nWith the provisional diagnosis of Hashimoto Encephalopathy in mind, he was started on levothyroxine 150 μgm/day along with I.V. methylprednisolone. With this treatment, the seizures were controlled completely on the second day itself. Repeat assessments of thyroid hormone levels were done weekly. T3, T4, and TSH levels normalized, with second TSH at 7.52 IU/ml and anti-TPO antibodies were 1457 units after 2 weeks and 40 units at 2 months of treatment. A repeat EEG was normal, and a follow-up MRI showed no abnormality. He was started on oral prednisolone 60 mg/day but he developed one episode of hematemesis, after which oral steroids were stopped and he was started on azathioprine 100 mg in divided doses. He was also prescribed telmisartan for hypertension at a dose of 40 mg per day.\nHe was in remission for up to 4 months after the initial episode and discontinued his medications. His symptoms recurred, and this time they consisted of vague tingling sensations on the face with slurring of speech and loss of attention. Anti-thyroperoxidase levels were elevated at about 400 U/ml. An MR angiogram was repeated with spectroscopy, which revealed a lactate peak corresponding to 1.3 μm in the left temporal area. However, a probe placed on the right occipital are showed no activity, suggesting new onset activity. [] He was not given steroids this time and merely started on azathioprine at 100 mg, and symptoms remitted as before. He was discharged on oral azathioprine with telmisartan, atorvastatin, and oral calcium supplements. Patient continues to stay in remission till today after about one and half years of therapy.