摘要:

ABSTRACT –Juvenile myoclonic epilepsy (JME) is a special syndrome within the primary generalized epilepsies which is characterized clinically by irregular jerks of shoulders and arms (so‐called impulsive petit mal) after awakening and electroencephalographically by bilateral‐synchronous 4–6/s spike‐wave complexes, often in the form of multispike‐waves. The age of onset for this syndrome which occurs in 4–6% of all epilepsies is predominantly between 12 and 18 years. It mostly starts with isolated jerks which as a rule are soon followed by generalized tonic‐clonic seizures (TCS). Jerks and TCS are provoked by sleep deprivation and predominantly occur after awakening (awakening epilepsy). Sleep deprivation and photostimulation are also very efficient in provoking specific EEG patterns. Exogenous factors have no etiological significance. Genetic studies suggest a polygenetic mode of inheritance and a lower threshold of manifestation in women. JME can be controlled very well by valproate and/or primidone. A complete cure, nevertheless, does not seem to be possible. Within the group of primary generalized epilepsies beginning in adolescence JME is closely related nosologically to the syndrome of juvenile absences and the syndrome of pure grand m

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1985.tb00900.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

ABSTRACT –The serum‐valproate level of four patients with epilepsy was followed during pregnancy. A decrease in serum level occurred late in pregnancy and was followed by a pronounced increase in the first week after delivery.The maternal serum concentration of valproate was compared to that of the umbilical cord. The level in cord blood was 145–219% higher than that in maternal blood.The concentration of valproate in breast milk was found to be 5–10% of the maternal serum concentration. The serum concentration was measured in one breastfed child. The level was 7.6% of the maternal serum concentration.All children were healthy without any signs of intoxication or malformation.Based on our experience, pregnant patients treated with valproate must be carefully controlled especially during the last month of pregnancy and in the first two weeks after delivery. The amount of valproate excreted into the breast milk was negligible and should not prevent breast

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1985.tb00901.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

ABSTRACT –Seventeen patients suffering from myasthenia gravis were treated with plasma exchange. Serious complications were not encountered. The effect usually appeared on the second or the third day of treatment. In 12 patients with a total of 31 plasma exchange courses, the treatment resulted in a shift in Osserman group. Six of these had long‐standing remissions. Three patients responded moderately and two patients did not respond to plasma excha

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1985.tb00902.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

ABSTRACT –In patients with multiple sclerosis (MS) the spontaneous burst activity (BA) of peripheral blood mononuclear cells was related to the clinical course of the disease. In five patients clinical improvement was found while the BA was significantly increased (more than 300% of the controls). During the appearance of new or deteriorating signs and in the period without clinical changes, the BA was not at all or not markedly increased. In two patients without clinical improvement the BA did not reach levels above 300% of the controls. Our findings suggest that inflammatory reactions represented by the BA occur in the phase of clinical improvement. Since burst‐stimulating activity was found in the serum of MS patients, cytokines produced by activated immuno‐competent cells are assumed to cause the increased BA. A possible role of prednisone and ACTH on the BA of peripheral mononuclear leucocytes is disc

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1985.tb00903.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

ABSTRACT –Patients with amyotrophic lateral sclerosis were treated with high‐dose intravenous infusion of human leukocyte interferon for six days. Neuropsychological examinations were carried out before, during and after the treatment. Marked reversible dysfunction was detected in immediate memory functions, coordination of hand movements, and drawing. Motor perseveration, micrographia, and slowing of behaviour were also observed. Changes appeared four to 12 days after start of treatment, with the peak on days six to eight. Recovery was almost complete by day 15. Intellectual ability, as measured by three WAIS subtests, praxis of hand movements, visuognostic functions, speech, reading, writing, and calculation remained essentially unaffected.The profile of the neuropsychological deficits observed, the absence of defects typical of focal posterior cortical lesions, the simultaneously slowed electroencephalographic activity with frontal accentuation, and the increased central conduction times of brain stem auditory evoked potentials suggest frontobasal involvem

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1985.tb00904.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

ABSTRACT –Aphasia following metrizamide myelography has been reported infrequently. During a seven‐month period, we examined two patients who developed Broca's aphasia, apraxia of speech, oral‐buccal‐facial apraxia and neurogenic stuttering after intrathecal metrizamide administration. In each case, focal neurologic deficits were accompanied by clinical, electroencephalographic and radiologic signs of generalized neurologic disease. Serial speech and language evaluations initially revealed severe deficits that were largely resolved by the third day post‐myelography. Out‐patient follow‐up examinations demonstrated persistence of mild speech and language abnormalities in each case. Our findings suggest that metrizamide may cause longlasting neurologi

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1985.tb00905.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

ABSTRACT –Evoked potentials (SEP and BAEP) were measured daily in 18 patients who had suffered severe craniocerebral trauma, but could not be examined neurologically due to a barbiturate coma or neuromuscular blockade. The BAEP had only limited value as an indicator of the prognosis for these patients. The N20 component of the SEP appeared to be more valuable in this respect. Serial registration of the EPs was also of prognostic significance and proved to play an important role in the decision to stop or continue the barbiturate coma. In some patients serial measurement of the EPs also gave an indication of the presence of late posttraumatic intracranial mass lesions. EPs are useful for the monitoring of cerebral functions in patients who have suffered a contusion of the brain and can no longer be assessed clinicall

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1985.tb00906.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

ABSTRACT –A new case of Tangier disease is described. It is the 33rd case in world literature and only the 2nd in Italy.A 52‐year‐old man showed a widely spread neuropathy with facial diplegia, bilateral wasting of hand muscles and dissociated loss of pain and temperature sensation sparing the distal parts of the lower limbs. Clinical and laboratory data were typical of Tangier disease. A histological and ultrastructural study of the patient's superficial peroneal nerve and brevis peroneus muscle was carried out.A revision of the clinical and neuropathological aspects of the neuropathy of Tangier disease allowed our case to be included within a particular neurological description. Four patients with similar clinical characteristics had been noted previously. Clinical, morphological and biochemical data suggest the hypothesis that there are two different neuropathic forms of Tangier di

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1985.tb00907.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

ABSTRACT –Thirty‐four patients with advanced Parkinson's disease showing intolerance to therapeutic doses of L‐DOPA were treated with L‐DOPA plus carbidopa at two different proportions. Ten patients preferred medication containing 10% carbidopa while 24 preferred 25% carbidopa. The increase of carbidopa reduced gastrointestinal disturbances and psychiatric side effects related to L‐DOPA, but improvement of disability when measured according to objective tests was modest. Higher doses of carbidopa reduced peripheral DOPA metabolism and increased blood levels of tryptophan, 3‐OM‐DOPA and DOPA in relation to the admi

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1985.tb00908.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

ABSTRACT –Forty‐three male rotogravure printers with long‐term toluene exposure and 31 age‐ and sex‐matched offset printers without toluene exposure were examined in detail. Clinical, neurophysiological, neuropsychological and neuroradiological examinations and assessment of autonomic functions did not reveal any statistically significant differences between the groups. The results suggest that occupational long‐term exposure to toluene under these circumstances does not have clinically significant adverse effects on the nervous system. Exposure to toluene seemed to be associated with hea

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1985.tb00909.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY