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Biomedical subjects

S F Berkovic

Publications and source records attributed to S F Berkovic.

At least 217 records · Page 12Linked to original sources
PubMed1986

Proximal motor neuropathy, dermato-endocrine syndrome, and IgG kappa paraproteinemia.

The association of monoclonal paraproteinemia, neuropathy, and dermato-endocrine disturbances is well recognized in Japan, and it also occurs in white patients. Neuropathy in such patients is classically distal and sensorimotor, and the paraprotein almost always contains lambda light chains. A 58-year-old white man presented with severe progressive proximal motor neuropathy, dermato-endocrine changes, and an IgG kappa paraprotein. Over a 2 1/2-year period, treatment with melphalan and prednisolone produced improvement in the neuropathy and resolution of dermato-endocrine features with a corresponding decline in the serum paraprotein concentration. Subsequent reappearance of the paraprotein, despite treatment, was associated with clinical relapse.

Endocrine System Diseases↗
PubMed1984

Metabolic disorders presenting as stroke.

In a series of 1460 consecutive patients admitted to an "acute" stroke unit, 10 patients (0.7%) were found to have a metabolic encephalopathy which mimicked stroke. Of these, three had hypoglycaemia, four had hyperglycaemia, three had hyponatraemia, and one had suffered from hypoxia. Metabolic disorders should be excluded in all patients with a diagnosis of suspected stroke, especially if the presentation is associated with a confusional state or focal seizures. The conventional symptoms and signs of the metabolic disorders may be minimal or absent. The relative rarity of these disorders among patients with suspected stroke is overshadowed by the importance of early recognition and treatment, in order to minimize morbidity and mortality.

Aged↗
PubMed1984

Striatocapsular infarction: large infarcts in the lenticulostriate arterial territory.

A specific form of deep cerebral hemisphere infarction was identified in 11 of 1,600 stroke register patients. Despite the CT finding of a deep striatocapsular lesion, these patients showed evidence of both cortical and capsular abnormalities. Angiography showed significant proximal carotid artery lesions as well as evidence of occlusive or obstructive lesions in the region of the T junction of the internal carotid artery. The combination of these pathologic entities would explain this particular clinical picture.

Aged↗
PubMed1984

Rubral tremor: clinical features and treatment of three cases.

Rubral tremor is characterized by a slow coarse tremor at rest that is exacerbated by postural adjustments and by guided voluntary movements. Some authorities have questioned whether it should be regarded as a distinct clinical entity. We observed three cases of rubral tremor following stroke and studied the movements by videotape recordings. The tremors were very similar in the three cases, and all showed plastic rigidity and dystonic posturing of the affected limbs. Rehabilitation difficulties out of proportion to the motor deficit were present, but all three patients responded favourably to treatment with levodopa-carbidopa. Lesions of the superior cerebellar peduncle, midbrain tegmentum or posterior part of the thalamus may cause this peculiar tremor, and it is probable that lesions of the red nucleus itself are not crucial for its production. The similarity of the clinical features and management problems of these cases suggests that it is valuable to regard rubral tremor as a specific clinical syndrome.

Aged↗
PubMed1984

Central pontine myelinolysis with widespread extrapontine lesions: a report of two cases.

Two cases of central pontine myelinolysis with extensive extrapontine lesions are described. Both occurred in patients with longstanding quadriplegia in whom severe hyponatraemia, occurring in a setting of respiratory infection, was rapidly corrected with hypertonic saline. The extrapontine lesions resembled those described in extrapontine myelinolysis, but the possibility that an anoxic-ischaemic component may have contributed is discussed. The clinical data in these two cases are consistent with the mounting body of clinical and experimental evidence that disturbances of sodium balance may be an important cause of central pontine and extrapontine myelinolysis.

Adult↗
PubMed1984

Experience with continuous ambulatory EEG monitoring.

The value of continuous ambulatory EEG monitoring in the management of patients with seizures was assessed. Eighty-seven patients were studied over a two-year period, and 198 recordings were made. In six patients data from the monitor record changed management significantly by suggesting a diagnosis other than that made clinically and by routine EEG. In a further 33 patients data strongly supporting the clinical diagnoses were obtained. In 47 patients no positive diagnostic data were obtained, and in one patient the data could not be reconciled with the clinical picture. The ambulatory EEG was particularly useful for detecting nocturnal fits, such as tonic seizures, and for differentiating genuine seizures from pseudoseizures. Limitations of the instrument include the presence of only four channels and some distortion and degradation of the EEG signal. In spite of these problems, the instrument was found to be a valuable aid in the evaluation of patients with seizures.

Adolescent↗
PubMed1983

Adrenomyeloneuropathy--clinical and biochemical diagnosis.

Adrenomyeloneuropathy (AMN) is an X-linked storage disease of very-long-chain fatty acids that presents as primary adrenocortical failure combined with spastic paraparesis and peripheral neuropathy. This disorder was diagnosed in three unrelated adult males. Definitive diagnosis was made by finding elevated very-long-chain fatty acids in plasma and skin biopsy samples. Biochemical characterisation of this disease has elucidated its genetics, clarified its relationship with adrenoleukodystrophy of children and other phenotypic variants, and allowed heterozygote identification, accurate genetic counselling and prenatal diagnosis.

Addison Disease↗
PubMed1983

Basilar artery dissection.

Dissection of the basilar artery caused sudden coma and death in a 40-year-old man. Atypical clinical features were explained at necropsy. A ventral dissection of the artery within its outer layers resulted in destruction of the pontine tegmentum with sparing of the basis pontis. An unsuspected defect in the internal elastic lamina in the left internal carotid artery was also found suggesting a more generalised disorder of arterial walls. Basilar artery dissection should be considered in the diagnosis of coma in young people.

Adult↗
PubMed1983

Hepatotoxicity of sodium valproate.

Repeated clinical and biochemical evaluations of liver function were performed on 60 patients taking sodium valproate. No clinical evidence of liver disease was found. Biochemical abnormalities were frequently found, but they were far more common in patients treated with multiple anticonvulsants than in patients on valproate alone. A control group of patients taking phenytoin or carbamazepine monotherapy also had a high incidence of biochemical abnormalities. A group of patients started on valproate therapy 6 years previously was followed up. No evidence of liver disease was found in this group. It was concluded that biochemical abnormalities of liver function in patients receiving valproate are not progressive, and in many cases relate to the intake of other drugs. The finding of abnormal liver function tests is not necessarily an indication for ceasing valproate.

Adolescent↗
PubMed1983

Absence status in adults.

Twelve cases of adult absence status were seen over 7 years. Clinically the disorder was characterised by long periods of clouding of consciousness with myoclonic phenomena of varying degrees of severity. The EEGs showed sustained spike-wave, polyspike and rhythmic slow activity. In one exceptional patient bizarre hemitonic seizures were documented clinically and on EEG. Diagnosis of the condition was frequently delayed and the histories of most patients showed episodes of obscure confusional behaviour, undiagnosed over the previous months or years. Psychiatric disturbances of various types were surprisingly common, contributing to diagnostic difficulty. Treatment with conventional anticonvulsants was often disappointing. In contrast, sodium valproate and clonazepam were found to be very effective.

Adult↗
PubMed1983

Carotid plaques and retinal emboli: a clinical, angiographic and morphological study.

The angiographic criteria for the significance of a carotid lesion and the contribution of embolic versus haemodynamic factors in stroke pathogenesis are currently unresolved. This study evaluated correlations between the clinical features, presence of retinal emboli, angiographic appearance of the carotid bifurcation and morphology of the surgically excised plaque in 32 consecutive patients with symptomatic carotid lesions. Retinal emboli were found in 22% of patients, including some without a history of amaurosis fugax. The carotid plaques were complicated by ulceration, intraplaque haemorrhage and/or intraluminal debris in 91% of cases. The degree of carotid stenosis seen at operation correlated well with the angiographic estimate. Plaque complications could not be accurately predicted angiographically, however. The high frequency of plaque complications, and the presence of retinal emboli, suggested that embolisation was the likely pathogenetic mechanism of stroke in most of these patients.

Arteriosclerosis↗
PubMed1982

Infectious mononucleosis encephalitis. Possible therapeutic response to vidarabine.

A comatose patient with seizures and focal neurological signs was given vidarabine because herpes simplex encephalitis was a likely diagnosis. Investigations showed that the illness was due to infectious mononucleosis. Although recovery is usual in infectious mononucleosis encephalitis, the complete clinical and neuropsychological recovery documented in this severe case raises the possibility that vidarabine may have exerted a therapeutic effect.

Adolescent↗
PubMed1982

Focal seizures and systemic metabolic disorders.

Repetitive focal seizures were associated with hyperglycemia in three patients, and with hypoxia in another patient. Autopsy in one case and computed tomography (CT) scans in two patients failed to reveal relevant focal cerebral disease. Awareness of the occurrence of focal seizures in metabolic disorders, especially non-ketotic hyperglycemia, should be increased as early recognition is vital for successful treatment. Metabolic encephalopathies can cause focal seizures with or without underlying focal cerebral pathology.

Aged↗
PubMed1977

Acetylation of histones in isolated avian erythroid nuclei.

1. Suspensions of avian erythroid nuclei, of high purity, were prepared. Acetylation of histones was observed when nuclei were incubated in the presence of [1-14C]acetyl CoA, but not in the presence of sodium [3H]acetate. 2. The acetylation reaction was very heat labile and reproduced the in vivo reaction with high fidelity. The reaction was strongly inhibited by divalent cations and cysteine. 3. Studies, in which intact cells were pre-incubated with cycloheximide prior to the isolation of nuclei, suggested that histone acetylation in isolated erythroid nuclei was largely independent of histone synthesis. 4. The pH profile suggested the presence of at least two histone acetyltransferases, with pH optima at 8.0 and 8.6. Acetylation of histone H4 appeared to be favoured at pH 8.0. 5. Studies on histone acetylation in isolated nuclei should be very useful in correlating observations on histone acetylation in vivo, with experiments using purified histone acetyltransferases.

Acetyltransferases↗