the most commonly reported types of seizures are complex partial seizures and tonic - clonic seizures [ 4 , 5 ] , even though eeg documentation of such seizures have been sparse . status epilepticus has also been rarely reported . here , we report two patients with hereditary coproporphyria ( hcp ) who presented with status epilepticus and an eeg documentation . a 49-year - old right - handed man with a past medical history of berger 's disease presented to the emergency room on december 13 , 2009 , for a 1-min - long tonic - clonic seizure and increasing confusion over the past week . , he developed continuous right hand myoclonic jerks compatible with epilepsia partialis continua ( epc ) . repeat brain mri performed 3 days after admission disclosed non - enhancing high t2 and flair signal changes over the bilateral temporal lobes ( fig . 1 ) . brain pet revealed focal hypermetabolism over the bilateral frontal and temporal lobes , predominantly over the left side . bacteriological , viral serological ( including herpes simplex virus pcr ) workup , immunologic parameters as well as toxicology screen revealed no abnormal findings . while the confusion progressively waned , epc persisted despite several antiepileptic drug trials ( phenytoin , valproic acid , carbamazepine , clobazam , lamotrigine ) . a probable diagnosis of hpc was eventually made when urine coproporphyrins and fecal total porphyrins returned to twice the normal range ( even when testing was made 9 days after admission while the patient was improving ) . these abnormal results could not be explained by other conditions which have previously been associated with secondary coproporphyrinurias such as abuse of toxic substances , liver diseases , malignancies , diverse hematological diseases , etc . . over the last 3 years , the patient has continued to present non - disabling epc ( manifesting only as very low - amplitude right hand myoclonic jerks ) and has had a total of four tonic - clonic seizures in the context of alcohol consumption . he remains with mild memory problems and is currently treated with levetiracetam . on the last brain mri ( 2 years after initial setting ) , t2 signal changes had decreased significantly ( fig . a 30-year - old man , known only for a generalized anxiety disorder , was brought to the emergency room on november 16 , 2011 , in convulsive status epilepticus preceded over the last 3 days by nausea , vomiting and headaches . initial management included intravenous benzodiazepines and phenytoin followed by intubation and a perfusion of midazolam and propofol . the patient then evolved to epc as he presented continuous left hand and mouth myoclonic jerks , very rarely associated with altered consciousness . 4 ) showed diffuse slowing , sometime associated with right frontotemporal periodic lateralized epileptiform discharges . mri disclosed right hemispheric gyriform increased signal on t2-weighted images , restricted diffusion on dwi / adc images and mild midline shift to the left . brain pet revealed focal hypermetabolism over the right frontal and temporal lobes , and to a lesser extent in the right occipital region . as in the first patient , bacteriological , viral serological workup as well as immunologic parameters failed to disclose a plausible cause . his condition eventually worsened , with decreased level of consciousness leading to intubation , despite large spectrum antibiotics , acyclovir , several antiepileptic drug trials ( phenobarbital , levetiracetam , lacosamide , topiramate , vigabatrin , gabapentin ) and immunotherapy ( intravenous immunoglobulins , plasma exchanges ) . diagnosis of porphyria was made when results from urine , fecal and serum porphyrins returned elevated at 3 weeks after admission . one month and a half after admission and despite hematin therapy and withdrawal of porphyrinogenic drugs , the patient eventually died from brain edema secondary to the status epilepticus , after he had suffered a porphyric attack from an isoflurane trial . later , genetic testing confirmed hcp with a rare homozygous missense g189s mutation in the coproporphyrinogen iii oxidase gene . a 49-year - old right - handed man with a past medical history of berger 's disease presented to the emergency room on december 13 , 2009 , for a 1-min - long tonic - clonic seizure and increasing confusion over the past week . , he developed continuous right hand myoclonic jerks compatible with epilepsia partialis continua ( epc ) . repeat brain mri performed 3 days after admission disclosed non - enhancing high t2 and flair signal changes over the bilateral temporal lobes ( fig . 1 ) . brain pet revealed focal hypermetabolism over the bilateral frontal and temporal lobes , predominantly over the left side . bacteriological , viral serological ( including herpes simplex virus pcr ) workup , immunologic parameters as well as toxicology screen revealed no abnormal findings . while the confusion progressively waned , epc persisted despite several antiepileptic drug trials ( phenytoin , valproic acid , carbamazepine , clobazam , lamotrigine ) . a probable diagnosis of hpc was eventually made when urine coproporphyrins and fecal total porphyrins returned to twice the normal range ( even when testing was made 9 days after admission while the patient was improving ) . these abnormal results could not be explained by other conditions which have previously been associated with secondary coproporphyrinurias such as abuse of toxic substances , liver diseases , malignancies , diverse hematological diseases , etc . . over the last 3 years , the patient has continued to present non - disabling epc ( manifesting only as very low - amplitude right hand myoclonic jerks ) and has had a total of four tonic - clonic seizures in the context of alcohol consumption . he remains with mild memory problems and is currently treated with levetiracetam . on the last brain mri ( 2 years after initial setting ) , t2 signal changes had decreased significantly ( fig . a 30-year - old man , known only for a generalized anxiety disorder , was brought to the emergency room on november 16 , 2011 , in convulsive status epilepticus preceded over the last 3 days by nausea , vomiting and headaches . initial management included intravenous benzodiazepines and phenytoin followed by intubation and a perfusion of midazolam and propofol . the patient then evolved to epc as he presented continuous left hand and mouth myoclonic jerks , very rarely associated with altered consciousness . 4 ) showed diffuse slowing , sometime associated with right frontotemporal periodic lateralized epileptiform discharges . mri disclosed right hemispheric gyriform increased signal on t2-weighted images , restricted diffusion on dwi / adc images and mild midline shift to the left . brain pet revealed focal hypermetabolism over the right frontal and temporal lobes , and to a lesser extent in the right occipital region . as in the first patient , bacteriological , viral serological workup as well as immunologic parameters failed to disclose a plausible cause . his condition eventually worsened , with decreased level of consciousness leading to intubation , despite large spectrum antibiotics , acyclovir , several antiepileptic drug trials ( phenobarbital , levetiracetam , lacosamide , topiramate , vigabatrin , gabapentin ) and immunotherapy ( intravenous immunoglobulins , plasma exchanges ) . diagnosis of porphyria was made when results from urine , fecal and serum porphyrins returned elevated at 3 weeks after admission . one month and a half after admission and despite hematin therapy and withdrawal of porphyrinogenic drugs , the patient eventually died from brain edema secondary to the status epilepticus , after he had suffered a porphyric attack from an isoflurane trial . later , genetic testing confirmed hcp with a rare homozygous missense g189s mutation in the coproporphyrinogen iii oxidase gene . porphyrias are uncommon , complex metabolic disorders caused by deficiencies in the activities of the seven out of eight enzymes of the heme biosynthetic pathway . they are generally subdivided into acute and non - acute porphyrias on the basis of their major clinical manifestations . the acute porphyrias are well - defined genetic disorders of heme biosynthesis characterized by acute life - threatening attacks of nonspecific neurologic symptoms . these acute porphyrias are comprised of acute intermittent porphyria ( aip ) , hcp , variegate porphyria ( vp ) and 5-aminolevulinic acid dehydratase porphyria ( adp ) in which aip is the most frequent type . the combined prevalence of the acute porphyrias is approximately 5 cases per 100,000 persons , they are more common in women than in men , and often generally develop during adult life . acute porphyric attacks can be exacerbated by a variety of factors , including porphyrinogenic drugs , alcohol , endogenous hormones particularly progesterone , pregnancy , cigarette smoking , metabolic stress induced by infections or surgery and reduced caloric intake . misdiagnoses of porphyrias are common because the signs and symptoms of acute attacks are variable and may mimic many other diseases . they can present with acute neurovisceral attacks , which may be accompanied by skin lesions in hcp and vp . abdominal pain , reported approximately in 8595% of patients , is often accompanied by nausea , vomiting , constipation and diarrhea . other symptoms include acute psychiatric symptoms , seizures , tachycardia and hypertension , and peripheral neuropathy . the most common types of seizures reported in the literature consist of complex partial or tonic - clonic seizures . a summary of these previously reported cases is presented in table 1 [ 6 , 7 , 8 , 9 , 10 ] along with our cases . status epilepticus was part of the initial presentation for all subjects with the presence of visceral symptoms in two . the type of status varied greatly from convulsive status epilepticus to non - convulsive status epilepticus , complex partial status and epc . non - surprisingly , confirmation of diagnosis led to modification of the treatment regimen with good outcome for all patients except one ( case 2 ) . out of the previously five reported cases , 3 patients had aip , 1 patient had vp and 1 had an unknown type of acute porphyria , while both of our patients had hcp . current understanding of the pathogenesis of seizures suggests the hepatic production of a neurotoxic substance , presumably ala [ a -aminobutyric acid ( gaba ) analogue ] and/or pbg which may interact with gaba or glutamate receptors . a combination of endothelial dysfunction , hypoperfusion , and vasoconstriction in this setting of neurotoxicity can lead to a compromise of the blood - brain barrier and brain edema . mri may help detect changes occurring in the brain at the time of the acute attack though lesions are non - specific ( cortically and/or subcortically , anterior or posterior , without or with mild enhancement , generally but not always reversible ) [ 11 , 12 ] . seizures , when they occur , are a therapeutic challenge because most aeds ( phenobarbital , carbamazepine , clonazepam , phenytoin , primidone , ethosuximide , valproic acid , lamotrigine , felbamate , tiagabine , topiramate ) may exacerbate attack of acute porphyria . drugs reported to be relatively safe include gabapentin [ 6 , 8 , 14 ] , levetiracetam , and possibly oxcarbazepine . although rare , their recognition is important as it modifies significantly patient management and probably their outcome .