Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neuropathologic findings in 2 cases of cerebral lymphomatoid granulomatosis with sequelae are presented. A 30-year old male with macular rash and pulmonary lymphomatoid granulomatosis responded to Prednisone terapy but developed acute intracranial hypertension with coma. A necrotizing hemorrhagic lesion was evident in the left putamen surrounded by diffuse and perivascular atypical lymphoplasmacytic infiltration. An 18-year old girl developed pulmonary lymphomatoid granulomatosis, diplopia, slurred speech and right hemiparesis. Brain scan, angiography and EEG suggested a left fronto-parietal mass assumed to represent lymphomatoid granulomatosis. She responded well to cerebral irradiation, intrathecal methotrexate and cytoxan but relapsed with seizures and increasing respiratory insufficiency. At autopsy, stigmata of cerebral lymphomatoid granulomatosis were absent but a parenchymatous degeneration consistent with disseminated necrotizing leukoencephalopathy following antileukemic therapy in children, was found.
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PMID:Cerebral lymphomatoid granulomatosis. A report of two cases, with disseminated necrotizing leukoencephalopathy in one. 100 3

We report a case of Fukuyama congenital muscular dystrophy with inflammatory infiltrate on muscle biopsy in an American girl of non-Japanese ancestry. The child was hypotonic, had decreased muscle strength in all extremities, and poor head control. Her mental and motor development were delayed. She developed generalized seizures at 19 months of age. Her muscle enzymes were abnormal; cranial computed tomography demonstrated hypoplasia of the cerebellum. Electromyogram was normal. Deltoid muscle biopsy documented scattered basophilic regenerating myofibers and focal atrophic fibers with focal increases of endomysial connective tissue, small endomysial foci of inflammatory cells, and occasional perimysial, perivenular lymphocytic infiltrates. Prednisone therapy produced some decrease in serum muscle enzyme levels.
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PMID:A variant of Fukuyama congenital muscular dystrophy in a non-Japanese child. 350 1

Four men and four women, aged 16 to 43, developed dural sinus thrombosis, five patients with acute lymphoblastic leukemia (L.A.L.) and three with non Hodgkin malignant lymphoma (N.H.M.L.). All the cases of L.A.L. were F.A.B.-2 subtype without any unusual hematological features. In 6 cases, the disorders occurred during the phase of therapeutical induction (E.O.R.T.C.-A.L.L.-H.R. protocol) at D5, D10, D15, D26, D30, D38, and in 2 cases during maintenance after a period of remission. All patients had received Vincristine and Prednisone, intrathecal Methotrexate in 5 cases, encephalic irradiation in 3 cases and L-Asparaginase in one case. Three women were taking contraceptive drugs. The neurological symptoms and signs were headache due to intracranial hypertension in 6 cases, Grand Mal seizures in 5 cases, focal seizures in 2 cases, a regressive hemiparesis in 4 cases, stupor in 3 cases. CT scan was abnormal in 4 cases, displaying oedema in 3 cases and an hemorrhagic infarction in 1 case. Angiography showed in all cases occlusion of the superior sagittal sinus in 7 cases and of the transverse sinus on 1 case. Six patients received anticoagulant therapy. Outcome was fatal in 3 cases: in 2 cases of L.A.L., the condition worsened rapidly after the onset and death was related to a tentorial herniation; in 1 case of N.H.M.L. death resulted from an intercurrent infection.
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PMID:[Dural venous sinus occlusions in hemopathies]. 385 30

We treated 116 children with ACTH or prednisone. Fifty-two had infantile spasms with hypsarhythmia, and 64 had other types of intractable seizures. ACTH completely controlled seizures in all patients with infantile spasms and hypsarhythmia and 74% of those with other types of seizures. Prednisone controlled 51% of patients with infantile spasms and none with other seizures. Serious side effects were minimal for both drugs, and recurrent seizures occurred in 40 to 50% of patients within 4 to 14 months after completion of therapy.
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PMID:ACTH and prednisone in childhood seizure disorders. 630 15

A 30-year-old man with long-standing localization-related epilepsy and mental retardation had seizures that were partially controlled with valproate (VPA) 500 mg four times daily. Routine examination showed severe thrombocytopenia with mild leukopenia and chronic low-grade hemolytic anemia. Pertinent laboratory results included positive ANA, rheumatoid factor, anti-NIA, circulating immune complexes, and antihistone antibody. The patient was treated with high dosage prednisone with partial improvement, but continued to have exacerbations at lower dosages. Fourteen months later, VPA was discontinued, and rapid improvement ensued. Prednisone was subsequently discontinued, and the patient has now maintained normal platelet counts for 18 months.
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PMID:Systemic lupus erythematosus associated with use of valproate. 811 40

Topiramate, a sulfamate-substituted monosaccharide (2,3:4, 5-bis-O-(1-methylethylidene)-beta-D-fructopyranose sulfamate), is a new antiepileptic drug, which has been approved for adjunctive therapy in adult patients with partial-onset seizures. Liquid-liquid extraction followed by flow-injection negative-ion electrospray mass spectrometry was evaluated as a means for the quantitative analysis of Topiramate in human plasma. Prednisone (1,4-pregnadiene-17-alpha, 21-diol-3,11,20-trione [15 microg/mL]) was used as the internal standard because its solubility and molecular weight are similar to those of Topiramate. Calibration curves for Topiramate were linear over a range of 1 to 30 microg/mL plasma (signal-to-noise ratio >4) and were highly reliable (r(2) = 0.994). This approach offers several advantages: (i) the extraction of Topiramate from human plasma using chloroform is simple and reproducible; (ii) the quantitative determination of Topiramate, in the presence of an internal standard, by flow-injection negative-ion electrospray mass spectrometry with selected-ion recording, is rapid and accurate and does not require chromatographic separation; (iii) the assay possesses adequate sensitivity (2-25 microg/mL) for the quantitative analysis of Topiramate in plasma from patients.
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PMID:Rapid approach to the quantitative determination of topiramate (2, 3:4,5-bis-O-(1-methylethylidene)-beta-D-fructopyranose sulfamate) in human plasma by liquid-liquid extraction and flow-injection negative-ion electrospray mass spectrometry. 1051 Apr 8

Steroids are often an effective treatment for the West's syndrome. There have been few reports of steroid use in children with epilepsy outside the first year of life. I report my experience with prednisone for the treatment of older children with intractable epilepsy. Twenty-eight children (17 boys, 11 girls) aged 18 months to 10 years with intractable epilepsy were studied. Prednisone 1 mg/kg/day for 12 weeks (6 weeks daily and 6 weeks alternate therapy) was prescribed in addition to their regular antiepileptic medications. The parents kept seizure diaries, and the patients were regularly assessed for seizure frequency and side effects. The follow-up period was for 1 to 5 years. Thirteen patients (46%) became seizure free on prednisone and another 18 (40%) had a significant decrease in seizure frequency. Five patients (19%) had no change in seizure frequency. The best outcomes were seen in the absence group in which six out of seven patients became seizure free and in the Lennox-Gastaut syndrome group in which seven out of 10 became seizure free. Side effects were uncommon and included weight gain in five patients and aggression in four patients. Prednisone therapy is a safe and effective adjunctive treatment for epilepsy. It should be considered as an alternative treatment for older children with intractable generalized epilepsy who have failed conventional antiepileptic therapy.
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PMID:Prednisone therapy in pediatric epilepsy. 1277 Jun 72

Landau-Kleffner syndrome and its variants such as continuous Spike-Wave Discharge during Sleep (CSWS) are progressive epileptic encephalopathies of childhood. The treatment of this unusual group of patients is controversial. We describe our experience in treating patients with Landau-Kleffner syndrome and CSWS with corticosteroids. The patients received Prednisone 1 mg/kg/day for 6 months, 1 year, then yearly. Follow-up was for 1-10 years (mean 4 years). Ten patients, 3 females, 7 males were studied. Age of onset ranged from 2 to 11 years (mean 7.5 years). Eight patients manifested Landau-Kleffner syndrome, and two had CSWS. Most patients had seizures (8/10); however, two patients did not have clinical seizures. MRI was normal in all patients. SPECT scan was abnormal in four patients, normal in three, and not available in three. All but one patient manifested significant improvement in language, cognition, and behaviour, which continued after the corticosteroid trial. Side effects were few (4/10) and transient and consisted of weight gain (2), behavioral change (1), and hypertension (1). Corticosteroids are a safe and effective treatment for patients with Landau-Kleffner syndrome and CSWS. Most patients had improvement in language, cognition, and behaviour after treatment. Side effects are few and reversible, and benefits appear long lasting. Corticosteroids should be considered as a treatment option in children with Landau-Kleffner syndrome and CSWS.
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PMID:Corticosteroids for the treatment of Landau-kleffner syndrome and continuous spike-wave discharge during sleep. 1586 29

Neuromuscular sarcoidosis is recognized with increasing frequency. Although reported to occur in only 5% of patients with sarcoidosis, major centers are reporting up to 20% neuromuscular involvement. While the majority of patients with neurologic involvement present with 7 (th) nerve palsy, the reported frequency of sarcoidosis of the brain, meninges, spinal cord, and muscles is rising. The diagnosis of neuromuscular sarcoidosis is usually accepted when a patient with previous biopsy-proven sarcoidosis presents with a compatible neurologic syndrome. In addition to cranial nerve abnormalities, these syndromes include leptomeningitis, seizures, mass lesions of brain and spinal cord, pituitary dysfunction, and neuropathies. Occasionally, the typical intrathoracic, ocular, cutaneous and lymph node presentations of sarcoidosis are not present or are unrecognized and the diagnosis is established by biopsy of neuromuscular tissue. Biopsy exhibiting granulomas is not specific and requires absence of evidence of known granulomagenic agents plus presence of multiorgan disease. An isolated granuloma in neural tissue without other organ involvement is not diagnostic of sarcoidosis. Treatment of neuromuscular sarcoidosis is usually prolonged. Facial palsy often improves spontaneously. Prednisone is still the drug of choice. Most authorities agree that 30 to 40 mg qd is sufficient with concomitant azathioprine or cyclophosphamide.
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PMID:Neuromuscular sarcoidosis. 1608 46

The authors report a case of 36-year-old woman presented with epileptic seizures and headaches. Magnetic resonance imaging (MRI) revealed an enhancing lesion with surrounding edema and mild mass effect in the left frontal lobe. Stereotactic brain biopsy demonstrated intraparenchymal granulomas surrounding S. mansoni eggs. Praziquantel was started (60mg/kg of body weight, in a single dose), followed by Prednisone (80mg/day) for seven days to treat the cerebral edema. The patient's symptoms resolved following medical treatment and the follow-up MRI yielded normal findings.
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PMID:Pseudotumoral form of cerebral Schistosomiasis Mansoni. 2496 Jul 95


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