Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Epileptic activity is an underdiagnosed cause that can determine a disruption of memory and cognitive performance, leading an incorrect diagnosis of dementia. We report a 68-year-old man, referred with a 5-year history of subtle behavioral changes and a subjective memory impairment, who was admitted to our department because of recurrent episodes of confusional state lasting from 1 week. Neuropsychological evaluation demonstrated a marked impairment of all cognitive domains examined. Electroencephalogram (EEG) recording showed frequent almost continuous sharp waves localized on the bilateral posterior temporal regions with mild right side predominance. Treatment with phenytoin reversed his cognitive dysfunction and behavioral disturbances. We presume that ictal temporal lobe epileptiform activity is the cause of his confusional episodes and cognitive dysfunction, showing an electroclinical picture of complex partial status epilepticus. However, we hypothesize that the interictal discharges and ictal and postictal effects of subclinical seizures could be involved in the behavioral changes and memory impairment complained by our patient in the last years.
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PMID:Temporal lobe epileptic activity mimicking dementia: a case report. 1619 Sep 19

Hashimoto's encephalopathy (HE) is a severe but treatable condition that rarely complicates Hashimoto's thyroiditis. Clinically it is characterized by progressive or relapsing symptoms, including tremor, myoclonus, stroke-like episodes, seizures, impairment of consciousness, and dementia. We describe a patient presenting with recurrent generalized convulsive status epilepticus (GCSE), despite antiepileptic medications, who was successfully treated with methylprednisolone. Our observation confirms that the clinical spectrum of HE at presentation is heterogeneous and diagnosis is often difficult. This case highlights the crucial importance of antithyroid antibody measurement in patients presenting with otherwise unexplained episodes of GCSE with or without adjunctive signs of encephalopathy or thyroiditis.
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PMID:Recurrent status epilepticus as the main feature of Hashimoto's encephalopathy. 1634 98

Cockayne syndrome and xeroderma pigmentosum-Cockayne syndrome complex are rare autosomal recessive disorders with poorly understood biology. They are characterized by profound postnatal brain and somatic growth failure and by degeneration of multiple tissues resulting in cachexia, dementia, and premature aging. They result in premature death, usually in childhood, exceptionally in adults. This study compares the clinical course and pathology of a man with Cockayne syndrome group A who died at age 31(1/2) years with 15 adequately documented other adults with Cockayne syndrome and 5 with xeroderma pigmentosum-Cockayne syndrome complex. Slowing of head and somatic growth was apparent before age 2 years, mental retardation and slowly progressive spasticity at 4 years, ataxia and hearing loss at 9 years, visual impairment at 14 years, typical Cockayne facies at 17 years, and cachexia and dementia in his twenties, with a retained outgoing personality. He experienced several transient right and left hemipareses and two episodes of status epilepticus following falls. Neuropathology disclosed profound microencephaly, bilateral old subdural hematomas, white-matter atrophy, tigroid leukodystrophy with string vessels, oligodendrocyte proliferation, bizarre reactive astrocytes, multifocal dystrophic calcification that was most marked in the basal ganglia, advanced atherosclerosis, mixed demyelinating and axonal neuropathy, and neurogenic muscular atrophy. Cellular degeneration of the organ of Corti, spiral and vestibular ganglia, and all chambers of the eye was severe. Rarely, and for unexplained reasons, in some patients with Cockayne syndrome the course is slower than usual, resulting in survival into adulthood. The profound dwarfing, failure of brain growth, cachexia, selectivity of tissue degeneration, and poor correlation between genotypes and phenotypes are not understood. Deficient repair of DNA can increase vulnerability to oxidative stress and play a role in the premature aging, but why patients with mutations in xeroderma pigmentosum genes present with the Cockayne syndrome phenotype is still not known.
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PMID:Cockayne syndrome in adults: review with clinical and pathologic study of a new case. 1709 72

Creutzfeldt-Jakob disease (CJD) is a rare disorder caused by prions that can affect any part of the central nervous system. It is characterized by a long incubation period, but once symptoms start there is a progressive neurological decline. Clinical features include dementia, ataxia and myoclonus (startle), among others. We report a biopsy-proven case of familial CJD (fCJD) presenting with continuous focal seizures, epilepsia partialis continua (EPC), as the initial presentation. CJD is an unusual neurological disorder with an incidence of approximately one case per million population (Prusiner 2001). The disorder is due to neuronal degeneration resulting from the accumulation of a pathological isoform (PrP) of the prion protein (PrPc). Patients with fCJD have mutations in the gene encoding PrPc (PRNP) (Vercueil 2006, Collins et al. 2004). This fCJD represents 10-15% of CJD cases making the sporadic form more common 85-95% (Parry et al. 2001). During the course of the disease myoclonus has been reported in 88% of cases, and epileptic seizures (partial seizures, generalized status epilepticus) in 8% (Vercueil 2006). Periodic sharp wave complexes (PSWC) are uncommon in fCJD and occur in about 10% of patients (Wieser et al. 2006).
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PMID:Familial Creutzfeldt-Jakob disease presenting as epilepsia partialis continua. 1901 68

Creutzfeldt-Jakob disease (CJD) is a rare human transmissible spongiform subacute encephalopathy. The most common clinical manifestations of CJD include rapidly progressive dementia, behavioural changes, cerebellar dysfunction and myoclonus. Other seizure types are rare and nonconvulsive status epilepticus (SE) is exceptional. We report a case of a 44-year-old man who presented a psychotic episode followed by akinetic mutism and refractory nonconvulsive SE. The final diagnosis was CJD. Continuous video-EEG monitoring revealed the ictal pattern of nonconvulsive SE to be periodic sharp wave complexes characteristic of CJD. [Published with video sequences].
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PMID:Refractory nonconvulsive status epilepticus in Creutzfeldt-Jakob disease. 2064 12

Diligence in the interpretation of results is essential as information gained from the psychiatric patient's history might often be restricted. Nonobservance of established guidelines may lead to a wrong diagnosis, induce a false therapy and result in life-threatening situations. Communication errors between hospitals and doctors and uncritical acceptance of prior diagnoses add substantially to this problem. We present a patient with alcohol-related dementia who received anti-retroviral therapy that promoted a non-convulsive status epilepticus. HIV serodeconversion was considered after our laboratory result yielded a HIV-negative status. Critical review of previous diagnostic investigations revealed several errors in the diagnosis of HIV infection leading to a "pseudo-serodeconversion." Finally, anti-retroviral therapy could be discontinued.
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PMID:Anti-retroviral therapy-induced status epilepticus in "pseudo-HIV serodeconversion". 2085 Dec 80

We report about a patient (66 years) who was referred to our psychiatric hospital because of a progressive confusional state with acute onset. The colleagues of the referring psychiatric hospital considered a first manic episode as the cause of the symptoms and under therapy with haloperidol the confusional state had shown a progression.The clinical examination's findings were a mild central facial paresis on the right side and a mild hemiparesis on the right side with elevated reflex levels.The patient was disoriented, he had cognitive and mnestic deficits. His reasoning was slowed, incoherent and perseverating. The patient had a slight euphoria.An EEG recording showed a continuous regional EEG-seizure pattern. In combination with the clinical symptoms we diagnosed a nonconvulsive status epilepticus. Under anticonvulsive treatment with Lorazepam and Valproic acid the status epilepticus sustended but a control EEG recording showed signs of a Valproate-encephalopathy. Under treatment with Topiramate symptoms ameliorated but due to a vascular dementia the patient still showed fluctuating symptoms of cognitive and mnestic disturbances.
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PMID:[Delirium caused by nonconvulsive status epilepticus]. 2247 64

We report on a 72-year-old-Japanese man with dementia with Lewy bodies (DLB) who presented with hemispheric cerebral cortical atrophy with selective neuronal necrosis after status epilepticus. His disease manifested with psychiatric symptoms, such as a "hot feeling" in the abdomen, at the age of 68 years. He was found to have hypochondriasis and anxiety disorder and was treated in the Department of Psychiatry. Parkinsonism gradually developed approximately 1 year later. Approximately 3 years after the onset of psychiatric symptoms, he suddenly experienced left hemiclonic seizures with consciousness disturbance, and he was admitted to our hospital. Status epilepticus occurred for several hours. His convulsive seizures were controlled by the administration of antiepileptic drugs (diazepam, phenytoin); however, he later showed prolonged disturbance of consciousness and left spastic hemiparesis. Gastrostomy was performed, but the patient died of aspiration pneumonia approximately 9 months after the onset of status epilepticus. Pathological examination showed widespread neuron loss with gliosis involving the entire right cerebral cortex, particularly in the frontotemporal lobe and hippocampus, and with patchy involvement of the contralateral cerebellum, especially in the cortex and dentate nucleus. These findings seemed to be consistent with a chronic stage of selective neuronal necrosis and secondary cerebellar degeneration due to diaschisis of the cerebral cortical involvement. Evidence of limbic-type DLB was also observed. We believe that selective neuronal necrosis associated with status epilepticus is an interesting neuro-pathological finding.
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PMID:[An autopsied case of dementia with lewy bodies presenting with hemispheric cerebral cortical atrophy with selective neuronal necrosis after status epilepticus]. 2294 44

Progranulin (PGRN) haploinsufficiency accounts for up to 10% of frontotemporal lobe dementia. PGRN has also been implicated in neuroinflammation in acute and chronic neurological disorders. Here we report that both protein and mRNA levels of cortical and hippocampal PGRN are significantly enhanced following pilocarpine-induced status epilepticus. We also identify intense PGRN immunoreactivity that colocalizes with CD11b in seizure-induced animals, suggesting that PGRN elevation occurs primarily in activated microglia and macrophages. To test the role of PGRN in activation of microglia/macrophages, we apply recombinant PGRN protein directly into the hippocampal formation, and observe no change in the number of CD11b(+) microglia/macrophages in the dentate gyrus. However, with pilocarpine-induced status epilepticus, PGRN application significantly increases the number of CD11b(+) microglia/macrophages in the dentate gyrus, without affecting the extent of hilar cell death. In addition, the number of CD11b(+) microglia/macrophages induced by status epilepticus is not significantly different between PGRN knockout mice and wildtype. Our findings suggest that status epilepticus induces PGRN expression, and that PGRN potentiates but is not required for seizure-induced microglia/macrophage activation.
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PMID:Progranulin promotes activation of microglia/macrophage after pilocarpine-induced status epilepticus. 2388 54

We report a 93-year-old woman with dementia who developed generalized convulsion and involuntary movement of her tongue. She could independently walk and eat meals until 8 months ago, however she turned into bedridden. When she was admitted to our emergency room due to status epilepticus, her tongue intermittently moved from the midline to the left. She could not eat or speak during this episodic tongue movement. MR imaging study revealed brain atrophy in the bilateral mesial temporal lobe, consistent with senile dementia of Alzheimer type. Despite her tongue movements seemingly developing to the generalized convulsion, EEG study did not indicate epileptiform discharges corresponding to this movement. Although antiepileptic drug therapy was effective, we needed polytherapy to control this movement. Paroxysmal tongue movements were previously reported in cases of epilepsy, brain tumor, and stroke, observed bilaterally in most cases. This episodic tongue movement would be rare in terms of the clear laterality. The etiology of this movement was presumed as focal seizure, palatal tremor, dyskinesia or others, but was undetermined. Episodic movements involving tongue decrease the quality of daily life especially in the elderly. Therefore, we should pay more attention to it and try to treat it earlier.
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PMID:[Case of an elderly woman with dementia showing episodic involuntary movement of the tongue]. 2480 76


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