Neuromyelitis optica with clinical and histopathological involvement of the brain.

Diagnostic criteria for neuromyelitis optica (NMO) state that there should be no active disease outside the optic nerves and spinal cord. However, several cases have been described with symptomatic brain involvement. We describe an autopsy case of a patient with NMO and symptomatic involvement of the brain. The histopathology of the brain lesions is typical for NMO, with extensive macrophage infiltration, including perivascular accumulation of large numbers of eosinophils. This is the first case that clearly shows that in NMO, the histopathology of the brain lesions is identical to that of the lesions in the optic nerves and spinal cord.

Neuropraxia of the cervical spinal cord following cervical spinal cord trauma: a report of five patients.

Neuropraxia of the cervical spinal cord is a rare condition which is almost exclusively reported in American football players following cervical hyperextension or hyperflexion trauma. In this entity-neurological symptoms of both arms and legs for a period of up to 15 minutes are observed with complete recovery. We report the characteristics of five patients not involved in contact sport activities with a neuropraxia of the spinal cord following cervical trauma. In four of the five patients, this syndrome was associated with a cervical canal stenosis. Surgical decompression was performed in two patients with progressive neurological symptoms after an initial period of recovery. The cases illustrates that although neuropraxia of the spinal cord is usually seen in athletes, also other persons may be at risk for developing this condition, especially when a preexisting spinal stenosis is present. Patients who experienced neuropraxia of the spinal cord should thus be evaluated carefully for the presence of cervical spinal cord abnormalities.

TIA, RIND, minor stroke: a continuum, or different subgroups? Dutch TIA Study Group.

The results of CT were studied prospectively in 606 patients with a transient ischaemic attack (TIA), 422 patients with a reversible ischaemic neurological deficit (RIND), and 1054 patients with a minor stroke, were all entered into a multi-centre clinical trial. CT scanning showed a relevant ischaemic lesion in 13% (95% confidence interval 10-16%) of TIAs, 35% (95% confidence interval 30-40%) of RINDs, and 49% (95% confidence interval 46-52%) of minor strokes (p less than 0.000001). Even within the 24 hour margin, relevant infarcts occurred more often with longer attacks, but were still found in some patients with attacks lasting less than a minute. The type and location of the infarcts were similar in the three groups. These findings suggest that the differences between TIAs, RINDs, and minor strokes are quantitative rather than qualitative.

alpha B-crystallin is present in reactive glia in Creutzfeldt-Jakob disease.

alpha-Crystallin is a major eye lens protein, composed of two types of subunits, alpha A and alpha B. The alpha A subunit is restricted to the lens, but alpha B-crystallin has recently also been detected in non-lenticular tissues, including the nervous system. With the use of a polyclonal antiserum directed against a synthetic C-terminal peptide of human alpha B-crystallin, the presence of alpha B-crystallin could be demonstrated immunohistochemically in astrocytes in the brains of patients with Creutzfeldt-Jakob disease (CJD). Most intensive localization was observed in the spongiotic tissue representing abundant progressively changed astrocytes in CJD. In age-matched control brains weak positive reaction was located in individual oligodendroglia cells and subpial astrocytes. Prominent increase of alpha B-crystallin in pathological glia in CJD may represent a response to stress.

Herpes simplex virus specific antibody determined by immunoblotting in cerebrospinal fluid of a patient with the Guillain-Barré syndrome.

The Guillain-Barré syndrome is often preceded by a herpes virus infection. Herpes simplex virus, however, has rarely been observed as the causative agent. A patient is described with a herpes simplex virus infection followed by a Guillain-Barré syndrome. Immunoblotting was used to detect herpes simplex virus-specific antibodies in serum and cerebrospinal fluid.

Neuromuscular investigations in retinitis pigmentosa.

Five patients with primary pigmentary dystrophy of the retina (retinitis pigmentosa) were tested on the integrity of their neuromuscular system. Clinical, electrophysiological and biochemical abnormalities were not found. A skeletal muscular biopsy was performed and histochemical, electron-microscopical and biochemical investigations were carried out. Only aspecific or minimal abnormalities were found. The mitochondrial functioning was especially explored and was found intact. Skeletal muscle examination is of no use in the study of retinitis pigmentosa.

[Flunarizine--a new agent for migraine prevention. Results of a double-blind comparison with placebo]. / Flunarizin--ein neues Mittel zur Migräneprophylaxe. Ergebnisse eines Doppelblindvergleichs mit Plazebo.

Seventeen patients with common or classical migraine were prophylactically treated with 10 mg flunarizine daily whereas 18 patients received a placebo during a 3-month randomized double-blind study. Globally, flunarizine was significantly superior to the placebo. Only three patients felt that flunarizine had been useless and the investigator also guessed the medication code correctly in all but these three cases. Beyond a 1-month starting period the frequency of the migraine attacks became significantly lower with flunarizine than with the placebo. The mean monthly number of attacks was respectively 3.3 and 3.8 before the study and 1.4 and 3.2 during the study. The limited scale of the trial precludes a judgment as to whether one type of migraine would respond better to flunarizine than the other. Side-effects were negligible, weight gain being considered rather a secondary gain than an untoward consequence of treatment.

Flunarizine, a new preventive approach to migraine. A double-blind comparison with placebo.

Seventeen patients with common or classic migraine were prophylactically treated with 10 mg flunarizine daily, whereas 18 patients received a placebo during a 12-week randomized double-blind study. In the gross flunarizine was significantly superior to the placebo. Only 3 patients felt that flunarizine had been useless and the investigator also guessed the medication code correctly in all but these 3 cases. After a 1-month starting period the difference between flunarizine and placebo in reducing the frequency of the migraine attacks became statistically significant in favour of flunarizine. The mean monthly number of attacks was respectively 3.3 and 3.8 before the study and 1.4 and 3.2 during the study. The limited scale of the trial precluded a judgment as to whether one type of migraine would respond better to flunarizine than the other. Side-effects were negligible, weight gain being secondary to the therapeutic effect rather than an untoward consequence of treatment.

Multivariate logistic analysis of risk factors for stroke in Tilburg, The Netherlands.

By means of a case-control study conducted between October 1, 1978, and July 31, 1981, in Tilburg, The Netherlands, various characteristics and events, including personal data, health-related behavior, and medical history, were evaluated as risk factors for stroke. The study subjects included 132 stroke patients and 239 age- and sex-matched control patients interviewed at the two city hospitals. To assess joint effects and possible interactions, and to control for multiple confounding factors, a series of multivariate logistic models for matched data were studied. From this analysis, it appeared that hypertension, acute myocardial infarction, cardiac arrhythmias, transient cerebral ischemic attacks, obesity, physical activity during leisure time, education of head of household, and Rhesus factor were all significant stroke risk factors. These risk determinants demonstrated a multiplicative effect in general; however, the influence of some variables on stroke risk was not constant with age (hypertension, acute myocardial infarction, cardiac arrhythmias, obesity, and Rhesus factor) and sex (hypertension and education of head of household). The relationship of diabetes mellitus to stroke slightly decreased and became nonsignificant after adjustment for factors besides age and sex. Stroke risk was not associated with cigarette and alcohol use, family history of stroke and related disorders, marital status, and ABO blood typing.

Epidemiology of stroke in Tilburg, the Netherlands. The population-based stroke incidence register: 2. Incidence, initial clinical picture and medical care, and three-week case fatality.

The results of a prospective population study of stroke in Tilburg are reported. The average annual over-all incidence (per 100,000 population) for the period October 1, 1978-September 30, 1980 was 174 for total cases, 145 for first-ever cases. Males and older persons were at greater risk for a stroke. The vast majority of strokes were thrombo-embolic infarctions. Hemiplegia was the predominant neurological deficit resulting from an insult. Patients with speech problems, as opposed to those without, demonstrated more extreme motor deficit when such occurred. Seventy-four per cent of stroke cases had hypertensive blood pressure readings shortly after onset. Most patients were under hospital care during the initial phase of their attacks. The presence of such handling was related to specific patient characteristics. Three-week stroke case fatality was 30% for all patients registered, and varied significantly by age, sex and diagnostic type.

An evaluation of risk factors for stroke in a Dutch community.

The objective of the present epidemiological study was to reevaluate the relationship of various suspect risk factors to stroke within a Dutch community. The Tilburg study lends support to findings in other countries concerning the greater stroke risk of persons with high blood pressure, heart disorders, and diabetes mellitus. Significant interactions of both age and a history of myocardial infarction with hypertension on stroke risk were noted. The presence of overweight and possible transient ischemic attacks (TIA) were found related to stroke onset. No increase in stroke risk was associated with cigarette smoking. The extent of leisure time physical activity during one's lifetime was found inversely related to one's chances for stroke.

Kearns syndrome: a heterogeneous group of disorders with CPEO, or a nosological entity?

In connection with 4 new cases of Kearns syndrome (multisystem form of mitochondrial CPEO), the condition was found to be present in slight to oligosymptomatic form in all 4 families. The marker symptom in subclinical patients was nearly always ptosis (sometimes very slight) and occasionally diabetes. In the literature other endocrine disorders, retinal anomalies, deafness, growth disturbances, etc., have been noted as subclinical symptoms in former generations. Heredity appears to be autosomal dominant in these 4 families, with very variable expressivity. The possibility that one gene is responsible for the disease seems to be plausible, but the marked variation in expressivity suggests a modifying influence of other alleles; in this sense, therefore, one may speak of multifactor inheritance. Supporting facts could also be found in the literature, where there was autosomal dominant heredity of the disease-carrying gene, but for its complete expression 'amplifying' factors (alleles) were needed. The pleiotropia of the disease-carrying gene is explained by a mitochondrial disorder of various organs. On the basis of the heredity, therefore, Kearns syndrome is not a syndrome but a disease. The most serious, most progressive and most extensive (multisystem) variant of Kearns disease is the infantile form, known as the 'Kearns-Sayre syndrome. When the expressivity of the disease is less extensive it usually occurs later in life and is less progressive: the adult form of Kearns disease.

Epidemiology of stroke in Tilburg, The Netherlands. The population-based stroke incidence register: 1. Introduction and preliminary results.

We present the methodology and some preliminary findings of a population-based stroke incidence register operating in Tilburg (about 150,000 persons), The Netherlands. From October 1, 1978 to March 31, 1979, 152 new strokes occurred giving an estimated overall incidence (per 100,000 population) for total and first attacks of 202 and 162, respectively. No major sex difference in stroke risk (all ages) was noted, although males did have a significantly higher incidence at 55--64 years of age. Stroke incidence increased strikingly with age. Thromboembolic infarcts comprised 83% and intracranial hemorrhage 13% of attacks. Males and females differed little in distribution of stroke types. The proportion of cerebral infarction was higher among older than younger cases. The opposite was true regarding intracranial hemorrhage. Embolic infarcts constituted a significantly higher proportion of cases at 65--74 years of age than at other ages. Hemiplegia and speech deficits dominated the clinical picture at onset. The 3-week case fatality for all strokes was 26%. Both a lowering of consciousness (found among 38% of cases during the first 24 hours) and a history of prior stroke were found related to a reduction in survival. Of patients surviving 3 weeks 25% were completely dependent and 25% partially dependent upon others for personal care.

Myoclonic disorders of spinal origin.

Data in the literature on spinal myoclonic disorders are still scanty; little has been done to ensure an adequate classification; little is known about the pathophysiology of these disorders. Three patients with spinal myoclonic jerks are described, with detailed reference to electromyographic findings. On the basis of the case reports so far available, a classification into five subgroups is suggested of spinal myoclonic jerks: 1) after cord injuries; 2) associated with intramedullary cord tumours; 3) associated with intramedullary or extramedullary cysts; 4) associated with radiculomyelitis or myelopathy; 5) associated with affections of the alpha motoneurons. A number of hypotheses on the pathogenesis of this disorder are briefly discussed: peripheral or intraspinal sprouting, degeneration of alpha motoneurons or interneurons, reduction of the dendritic tree size, and involvement of the gamma system.