1/649. An unusual case of Lafora body disease. A case is described in which non-convulsive status epilepticus (NCSE) prompted further investigation leading to the diagnosis of Lafora body disease (LBD). The onset of NCSE was temporally related to the withdrawal of sodium valproate and introduction of carbamazepine, which may have been precipitating factors. NCSE has not previously been reported in LBD. Implications for its drug management are discussed. ( info) |
| CASE REPORT: A 53-year-old cocaine user was on chronic therapy with theophylline, albuterol, and ipratropium for asthma and nifedipine for hypertension. Acute asthma treatment that increased the serum theophylline to 35 micrograms/mL was associated with tonic clonic seizures followed by bizarre, lateralized posturing. Electroencephalogram seizure activity lasting 10 days was consistent with nonconvulsive status epilepticus, Complex Partial, type II. theophylline was considered the probable instigator of this underdiagnosed neurologic disorder. ( info) |
3/649. MRI abnormalities associated with partial status epilepticus. OBJECTIVE: To report neuroimaging findings in patients with complex partial status epilepticus. BACKGROUND: During status epilepticus, neuroimaging may be used to exclude other neurologic conditions. Therefore, it is important to identify the neuroimaging features that are associated with status epilepticus. In addition, MRI characteristics may provide insight into the pathophysiologic changes during status epilepticus. methods: The history and neuroimaging examination results of three patients with complex partial status epilepticus were reviewed. Studies obtained during status epilepticus included diffusion-weighted MRI (DWI), MR angiography (MRA), postcontrast T1-weighted MRI, T2-weighted MRI, and CT. Follow-up MRI was obtained in two patients, and autopsy results were available for the third. RESULTS: Some of the MRI and CT findings during partial status epilepticus mimicked those of acute ischemic stroke: DWI and T2-weighted MRI showed cortical hyperintensity with a corresponding low apparent diffusion coefficient, and CT showed an area of decreased attenuation with effacement of sulci and loss of gray-white differentiation. However, the lesions did not respect vascular territories, there was increased signal of the ipsilateral middle cerebral artery on MRA, and leptomeningeal enhancement appeared on postcontrast MRI. On follow-up imaging, the abnormalities had resolved, but some cerebral atrophy was present. CONCLUSIONS: The radiologic characteristics of status epilepticus resemble those of ischemic stroke but can be differentiated based on lesion location and findings on MRA and postcontrast MRI. The MRI abnormalities indicated the presence of cytotoxic and vasogenic edema, hyperperfusion of the epileptic region, and alteration of the leptomeningeal blood-brain barrier. These changes reversed, but they resulted in some regional brain atrophy. ( info) |
4/649. Truly a team effort. Jane was a healthy 16 year old girl who attended a high school dance and subsequently had a grand mal seizure--her first! She was taken home, developed a decreasing level of consciousness and was admitted to the local hospital, where it progressed to status epilepticus. We will describe the classifications of seizures including status epilepticus, which demands the highest level of clinical expertise and attention to preventative medicine, for a desirable outcome. During the eleven months of care a massive multi disciplinary team approach was instituted which extended across borders. Jane's story demonstrates a truly Neuroscience team effort from acute care to a rehabilitation center to home. ( info) |
5/649. 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. ( info) |
6/649. Epileptogenic mineralization: pathological variants with good prognosis. Four examples of grossly mineralized lesions of the brain, causing seizures, are described; they include 2 hamartomas, a choristoma (novel associations), and an idiopathic "brain stone." Each was detectable with routine roentgenograms, and the seizures were ameliorated by surgical therapy. ( info) |
7/649. camphor toxicity. camphor ingestion led to status epilepticus in a 20-month-old girl who required intubation and ventilation. She was treated with intravenous valium and phenobarbitone, and nasogastric activated charcoal. She made a complete neurological recovery. A number of products containing a high concentration of camphor were found to be available in the local community. ( info) |
8/649. Unusual presentation and course of hiv-1 progressive encephalopathy. The present report concerns a vertically human immunodeficiency virus type 1 (hiv-1)-infected 7-year-old child, in whom a neurodegenerative disease occurred after an acute neurologic disorder that was in all likelihood symptomatic of hiv-1 encephalitis. At the steady state the neurologic disease fulfilled the accepted criteria of HIV-related progressive encephalopathy of childhood and was characterized by involvement of multiple neural systems and subcortical dementia. The neurologic disease displayed, however, atypical presentation and course, and its acute focal onset led the authors to postulate an acute and direct involvement of the brain in hiv-1 infection. The correlation between the cliniconeuroradiologic data and levels of HIV-rna in the cerebrospinal fluid and the response to different antiretroviral treatments are also discussed. ( info) |
9/649. Clinical experience of three pediatric and one adult case of spike-and-wave status epilepticus treated with injectable valproic acid. Subclinical or nonconvulsive status epilepticus may cause severe postmorbid neurologic dysfunction. It is, therefore, critical to rapidly identify and treat these cases. The recent availability of injectable valproic acid (Depacon) provides an additional method for treatment of status epilepticus, although studies concerning its effectiveness are not widely available in the literature. We report four cases (three pediatric, one adult) of patients who presented to us in status epilepticus. All had previously failed more than one other common method of treatment for this condition. Treatment with injectable valproic acid resulted in the elimination of all clinical indications of status epilepticus as well as a return to the baseline EEG condition in all four cases. Seizure types included focal, multifocal, and generalized spike and wave forms, suggesting potential benefit from injectable valproic acid treatment in a wide range of status epilepticus patients. We present these cases for review. ( info) |
10/649. Persistent occipital electrographic status epilepticus. A 13-year-old girl of normal intellect had clinically silent occipital electrographic status epilepticus that persisted for more than 3 years. neurologic examination and cranial magnetic resonance imaging were entirely normal. [18F]Fluorodeoxyglucose positron emission tomography demonstrated a hypermetabolic focus in the right occipital lobe. Electrographic status lasting years can be seen in epilepsia partialis continua. However, the absence of focal clinical seizures, nonprogressive course, and normal magnetic resonance imaging study seen in this patient are not features characteristic of epilepsia partialis continua. ( info) |