Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042571 (vertigo)
 7,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on a 9-year-old boy who developed subependymal giant-cell astrocytoma associated with tuberous sclerosis. Apart from a few hypopigmented skin patches, the boy's clinical picture was completely unremarkable until the onset of diplopia and vertigo reflecting increased intracranial pressure. Magnetic resonance imaging of the brain revealed subcortical hamartomas in addition to the intraventricular tumour. Seizures and mental retardation were absent. The boy's mother had suffered from seizures during childhood and presented with typical skin lesions. The importance of thorough examination and regular follow-up even in patients with oligosymptomatic variants of tuberous sclerosis is emphasized.
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PMID:[Oligosymptomatic Bourneville-Pringle tuberous sclerosis of the brain with giant cell astrocytoma]. 175 57

Glioneuronal migration disorders of the brain evolve primarily due to aberration in neuronal migration, maturation and programming in the development of various topographic zones in the brain, following pathological alterations in glial and neuronal interactions. These are broadly referred as cortical dysplastic conditions. While these dysplastic conditions involving cerebral cortex present as drug resistant seizure disorder, those involving cerebellum present as mass lesions or slowly progressing vertigo. We report 17 cases, representing the histological spectrum of dysplastic, glioneuronal migration disorders which include, hemimegalencephaly (1), tuberous sclerosis (4), Sturge Weber Syndrome with focal dysplasia (1), Dysembryoplastic neuroepithelial tumor (7) and Lhermitte Ductos disease of cerebellum (2). The dysplastic neurons in varied stages of maturation showed neuronal cytoskeletal pathology similar to that in neuro degenerative diseases, especially when associated with cytomegaly. Similarly, cells exhibiting dual expression of glial and neuronal markers were noted in the cerebral dysplastic lesions. The dysplastic glial elements probably form the subependymal giant cell astrocytomas. Dysplastic neuronal elements form the nidus for DNT. When localized, surgical resection ameliorate the symptoms in many of these condition. Study of these conditions provide better insight into glioneuronal interaction and maturation of the brain.
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PMID:Glioneuronal migration and development disorders: histological and immunohistochemical study with a comment on evolution. 1257 93

Lamotrigine is a broadly effective antiepileptic drug in mono- and add-on therapy for children and adolescents with focal and generalized epilepsies. Some epileptologists consider lamotrigine as the drug of primary choice in older school children and adolescents because of its good tolerability (no increase of body weight, no impairment of cognitive functions, due to new data probably no teratogenic properties). Lamotrigine can be used with good efficacy in numerable epilepsy diseases, such as tuberous sclerosis, juvenile neuronal lipofuscinosis and Rett syndrome. The first studies show that lamotrigine is also effective in children under 2 years of age. For therapy of difficult-to-treat epilepsies the combination of lamotrigine with valproate has proved as especially useful. This clinical observation is supported by new results of animal experiments. The dose-dependant and typical CNS side effects vertigo, ataxia, nausea, tremor and diplopia are found most frequently. The rate of allergic skin rashes which was very high before 1998 has decreased markedly by new dosage guidelines and is now as low as in older antiepileptic drugs. Lamotrigine does not impair cognitive functions, especially not memory and language. It has mood-stabilizing features and may improve quality of life. In animal experiments lamotrigine shows antiepileptogenic and neuroprotective effects.
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PMID:[Optimizing epilepsy therapy in children and adolescents with lamotrigine]. 1603 48

Tuberous sclerosis complex is an autosomal dominant disorder affecting primarily the central nervous system, skin, and kidney caused by mutations in the TSC1 and TSC2 genes. Diagnosis is established with the identification of various neurocutaneous symptoms and multiple organ system hamartomas. The authors report on a 9-year-old patient with episodes of vertigo and headache followed by full spontaneous recovery. There was no history of seizures, mental retardation, or other neurologic sequelae, and psychomotor development was normal. Magnetic resonance imaging revealed pial angiomatosis of the left cerebellum and calcifications in the left parieto-occipital lobe consistent with the diagnosis of Sturge-Weber syndrome. At the age of 13, multiple renal angiomyolipomas and a single hypomelanotic macule were found, and subsequent imaging revealed several cortical tuberi. The diagnosis was confirmed by sequence analysis, which identified a novel missense mutation p.Ala460Thr in exon 13 of the TSC2 gene. Thus, mild tuberous sclerosis due to a hypomorphic mutation in TSC2 may present with isolated leptomeningeal angiomatosis.
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PMID:Unusual radiological presentation of tuberous sclerosis complex with leptomeningeal angiomatosis associated with a hypomorphic mutation in the TSC2 gene. 1925 84