Cases reported "Status Epilepticus"

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1/5. The role of cranial MRI in identifying patients suffering from child abuse and presenting with unexplained neurological findings.

    OBJECTIVE: The aim of this study was to demonstrate the usefulness of cerebral MRI to detect possible child abuse in children with unexplained neurologic findings. METHOD: Between 1990 and 1997, 208 children were referred for suspected physical child abuse to the Child Protection Clinic of Ste-Justine Hospital, a tertiary care pediatric hospital. Among them, 39 children presented initially with neurological findings. For 27 of them, the CT Scan results prompted the diagnosis of child abuse. However, in 12 children, even if a CT-Scan was performed, the diagnosis and/or the mechanisms of the neurologic distress remained obscure. Investigation was completed with MRI study in those 12 cases. RESULTS: MRI findings were diagnostic for physical abuse in eight cases. A diagnosis of child abuse was made in two more cases by a combination of MRI and skeletal survey findings. In one case, MRI was suggestive but the diagnosis of child abuse could not be confirmed. One case was misinterpreted as normal. CONCLUSIONS: MRI is the test of choice to rule out child abuse when faced with a child presenting unexplained neurologic signs lasting for few days. The fact that MRI can better differentiate collections of different ages makes this imaging test particularly useful in identifying cases of child abuse. These results, however, always have to be integrated in a well conducted multidisciplinary clinical approach.
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2/5. catatonia mimicking nonconvulsive status epilepticus.

    Nonconvulsive status epilepticus (NCSE) and catatonia share many clinical features and distinguishing between them on the basis of the physical examination may be difficult and even impossible. Although several reports have been made of NCSE simulating catatonia, there are no published cases of catatonia mimicking NCSE. We report a 24-year-old woman whose initial examination was notable for a fixed stare, no response to voice or command, gaze preference, tonic head posturing, constant stereotypic chewing movements, profuse foamy salivation, and dramatic response to the parenteral administration of benzodiazepines (BZDs). She was initially misdiagnosed as having NCSE. EEG, however, was normal. We believe this is the first published report of catatonia simulating NCSE. The EEg may be more useful than the clinical examination in distinguishing NCSE from catatonia.
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3/5. Defusing status epilepticus.

    The risk of brain neuron death supersedes that of physical harm due to muscle spasms. In fact, immediate induction of neuromuscular paralysis impedes monitoring of anticonvulsant therapy, which can be empiric but should follow a predetermined, sequential protocol so that seizures can be terminated quickly.
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4/5. Complex partial status epilepticus in childhood.

    We report 5 pediatric patients (2 male, 3 female; age range: 4-8 years) with complex partial status epilepticus (CPSE). Four patients had previous illnesses and mild motor or mental retardation. In 2 patients, CPSE was induced by inappropriate management or selection of antiepileptic drugs. Clinical features varied and automatisms were observed in 3 patients. In 1 patient, decreased physical tone with syncope and impaired consciousness with amaurosis were observed. The episodes of CPSE were continuous in 3 patients and recurrent in 2 patients. In 4 patients, ictal electroencephalographic (EEG) findings, including video-EEG analyses of 3 patients, demonstrated persistent focal epileptic features. Intravenous diazepam abolished CPSE in 3 patients with brief periods of definite EEG localizations remaining. In 4 patients, seizure prognoses were favorable after appropriate treatments; in 1 patient, seizures were intractable even after antiepileptic drug administration.
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5/5. Monozygotic twins with MELAS-like syndrome lacking ragged red fibers and lactacidaemia.

    Typical cases of MELAS present a combination of clinical and neuroradiological features, lactacidaemia, and ragged red fibers (RRFs) in striated muscle. We have observed a MELAS-like syndrome in monozygotic twins. They developed seizures typically in conjunction with physical exertion, sleep deprivation or febrile episodes. stroke-like episodes occurred usually during seizures. In twin 2 the course was fatal at age 20 years. Neuroradiological findings were typical of MELAS. plasma lactate was normal in both. CSF lactate was normal in twin 1 and normal/elevated in twin 2. RRFs were not seen in muscle biopsies of the twins. Complex I activity was reduced in muscle in twin 1. brain tissue removed at epilepsy surgery in twin 2 showed the presence of mitochondrial angiopathy. The commonest mitochondrial dna mutation in MELAS, at base pair 3243, was absent. Lactacidaemia and mitochondrial myopathy with RRFs constitute part of the diagnostic criteria of MELAS. However, the absence of these features does not exclude mitochondrial disorder with the serious manifestations of MELAS (seizures and stroke-like episodes) as seen in these twins.
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