1/44. Does the tuberous sclerosis complex include intracranial aneurysms? A case report with a review of the literature.BACKGROUND: tuberous sclerosis is a protean, genetically determined disease that may involve any organ or tissue and lead to a great number of symptoms and clinical features. OBJECTIVE: diagnosis can be very difficult in cases with incomplete manifestations (formes fruste) lacking the classic signs of the disease. MATERIALS AND methods: We report a case fulfilling the diagnostic criteria for tuberous sclerosis (shagreen patches, hypomelanotic macules, renal cysts and angiomyolipomas, and "migration tracts" in the cerebral white matter) in association with a giant intracranial aneurysm, but lacking mental retardation, epilepsy and facial angiofibroma. RESULTS: Fourteen other cases of tuberous sclerosis and intracranial aneurysms, all but one without any clear sign of polycystic kidney disease, were found in the literature. CONCLUSION: We suggest that vascular dysplasias in general and aneurysms (mainly intracranial) in particular can be added to the other non-primary diagnostic features for the clinical diagnosis of tuberous sclerosis.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
2/44. Attempted child-stealing: post-ictal psychosis and psychological distress.Baby-stealing is probably one of the more taboo crimes in our society. Its connection with psychological distress, while generally acknowledged, is not always well understood. In this paper we present an unusual and complex case of a woman who attempted to steal young babies while in a state of extreme psychological distress following epileptic seizures. While the post-epileptic state was characterized as 'psychotic' there were clear psychological antecedents to explain the form in which the postical psychosis was expressed. Treatment strategies included modification of medication regime and use of cognitive therapy to ameliorate the impact of postictal confusion.- - - - - - - - - - ranking = 6keywords = tea (Clic here for more details about this article) |
3/44. The coordinated psychosocial and neurologic care of children with seizures and their families.SUMMARY: As the medical and surgical management of epilepsy continues to advance, issues associated with the quality of life of patients and their families can be addressed. Whenever associated with other handicaps, such as learning disabilities, attentional or behavioral disorders, and problems in psychological adjustment, dual-diagnosis issues must be identified. To provide comprehensive care for children with epilepsy, a team approach to psychosocial assessment and treatment must be provided and coordinated with neurologic care. When the age-related needs in the life stage of the individual and family are identified, the best possible adaptation of the patient and his or her family can be supported.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
4/44. Diaper burn: accident, abuse, or neglect.A 7-year-old, female with mental retardation and seizure disorder was admitted with burns. Reportedly, her brother who was 12 years old was cooking dinner for himself. A towel caught fire. He threw the towel over his head, and the patient's diaper ignited. She had partial thickness second-degree burns over her right elbow, upper abdomen, both inner thighs, and buttocks with multiple blistering, which was estimated to be 7% of the total body surface. The child abuse team took a diaper similar to the one the child was wearing at the time of injury and found that it easily ignited and melted in several seconds. This is the first medically reported burn case due to a disposable diaper.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
5/44. Postcallosotomy seizure outcome in hemiconvulsion-hemiatrophy-epilepsy syndrome.Recently, three patients with hemiconvulsion-hemiplegia-epilepsy syndrome who underwent callosotomy were monitored for more than four years. All patients had atrophy of the right brain hemisphere with left hemiparesis and seizures. Two cases were probably the result of traumatic intracranial hemorrhage and one was due to an unknown cause. Wada tests were done in cases 1 and 2, which showed spared motor function of the atrophic hemisphere, supporting the choice of callosotomy instead of hemispherectomy. Patient 1 had atypical absence seizures and asymmetric generalized tonic seizures before surgery, the latter of which decreased by about 60% after callosotomy. Patient 2 had simple partial seizures of the motor type and complex partial seizures, the latter of which were also induced by touch (somatosensory-induced reflex epilepsy). This patient's complex partial seizures disappeared completely, but the simple partial seizures remained unchanged. Patient 3 had generalized tonic seizures, simple partial seizures of the sensory type and complex partial seizures. After surgery, the frequency of the generalized tonic seizures decreased more than 90%. The simple partial seizures of the sensory type remained unchanged. There were two new types of seizures after surgery, simple partial seizures of the motor type and brief generalized myoclonic jerks. All patients had significant reductions in numbers of seizures of more than 50%.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
6/44. Purple glove syndrome caused by oral administration of phenytoin.A severely handicapped boy had been treated with phenytoin and his seizures were controlled well. At 10 years of age, a pharmacy gave about 1000 mg of phenytoin instead of the prescribed 100 mg of the drug per day. Several hours after the initial administration, the patient became drowsy and his hands and feet turned dark purple with marked swelling. Four days later, his mother stopped administering the phenytoin to him and took him to hospital. After fluid therapy was started, the swelling and discoloration of both his hands and feet improved gradually and disappeared 11 days after drug discontinuation. Purple glove syndrome is defined as the edema, discoloration, and pain occurring in the distal limb where intravenous phenytoin has been administered. This might be the first report of purple glove syndrome caused by the oral administration of a large quantity of phenytoin.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
7/44. Theoretical and practical considerations in the psychological and educational assessment of the student with intractable epilepsy: dynamic assessment as an adjunct to static assessment.Assessing the student with intractable epilepsy requires skill not only in evaluating cognitive problems, but also detecting seizures and discovering how to adapt instruction to minimize their negative impact on learning. Ironically, assessment efforts are seen as compromised by the occurrence of seizures during testing, when determining how seizure events may interfere with learning and the instructional modifications that are necessary to cope with them, should be a key part of assessment. A dual approach to assessment is recommended that combines the identification of cognitive deficits with an evaluation of how recurring seizures may prevent the student from engaging in instruction. Without also evaluating the student's response to instruction, teaching to specific cognitive needs is limited by insufficient knowledge about how to keep the student involved in instruction when seizures occur. Static assessment evaluates cognitive functioning at the time of testing, without changing the way that the student learns and responds. By engaging the student in teaching/learning sessions, dynamic assessment explores how the student best learns despite cognitive deficits and the disruptive effect of seizures. This paper includes a description of the authors'experience in using dynamic assessment as an adjunct to static assessment in evaluating a student with intractable epilepsy.- - - - - - - - - - ranking = 2keywords = tea (Clic here for more details about this article) |
8/44. Severe, isolated thrombocytopenia under polytherapy with carbamazepine and valproate.It is not known whether polytherapy with carbamazepine (CBZ) and valproate (VPA) increases the risk of thrombocytopenia. A 67-year-old woman with symptomatic epilepsy since age 6 years, classified as Lennox-Gasteau syndrome, developed severe, isolated thrombocytopenia of 5 GIGA/L (normal: 150-360 GIGA/L) after being on CBZ (1200 mg/day) for 15 days and additional VPA (300 mg/day) for 5 days in combination. After discontinuation of CBZ and VPA and two thrombocyte transfusions, the thrombocyte count normalized within 3 days. Because she had been taking VPA for only 5 days in addition to CBZ, it could not be confirmed whether it was CBZ alone or the combination of CBZ and VPA that was responsible for the severe thrombocytopenia.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
9/44. Non-alcoholic steatohepatitis induced by carbamazepine and variegate porphyria.A 42-year-old woman presented with acute bullous skin lesions and angio-oedema that had developed 3 months after initiation of treatment with carbamazepine for epilepsy. Chromatographic analysis of urinary porphyrins was compatible with variegate porphyria. This was manifested initially by neurological symptoms that were mistaken for epilepsy and later by cutaneous symptoms also. Histological findings excluded hepatic porphyria, but revealed severe fatty changes thought to be caused by idiosyncratic metabolism of carbamazepine. While the porphyrinogenicity of carbamazepine is well known, the presence of variegate porphyria has not been reported. The toxic hepatic effects of the drug on hepatic cytochrome P-450, which is involved in haem metabolism, could have aggravated the pre-existent porphyria, provoking the onset of skin lesions.- - - - - - - - - - ranking = 4keywords = tea (Clic here for more details about this article) |
10/44. Bielschowsky bodies (Lafora bodies of Bielschowsky type): report of a case associated with Rosenthal fibers in the brain stem.Bielschowsky bodies are an uncommon type of polyglucosan body. Similar to Lafora bodies, they are characteristically identified within neuronal perikarya and neurites. However, they lack the diffuse distribution of Lafora bodies, and instead are typically restricted to the external pallidum, often in association with status marmoratus or atrophy of the putamen. Fewer numbers of Bielschowsky bodies have also been identified in other areas such as the substantia nigra, putamen and inner globus pallidus. We report an additional case with Bielschowsky bodies in an 18-year old female with cerebral palsy. This case demonstrated multifocal Bielschowsky bodies and abundant Rosenthal fibers in the midbrain and pons. To our knowledge the association of Bielschowsky bodies with this peculiar distribution of Rosenthal fibers has not previously been reported.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
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