Jackson Braham

Creutzfeldt-Jakob Disease: Focus among Libyan Jews in Israel

A countrywide search for Creutzfeldt-Jakob disease in Israel disclosed 29 cases with onset between 1963 and 1972. Incidence in various ethnic groups varied in the narrow range of 0.4 to 1.9 per million population except among Jewish immigrants from Libya, among whom the incidence was 31.3 per million. An extraordinary excess of Creutzfeldt-Jakob disease exists in this ethnic group.

Subacute spongiform encephalopathy and its relation to Jakob-Creutzfeldt disease: report on six cases

An account is given of six cases of Jakob-Creutzfeldt disease confirmed at necropsy. The rapid fatal outcome within three months was typical of the variety designated subacute spongiform encephalopathy (Nevin-Jones). The characteristic EEG changes were found to be of great value in making an early diagnosis and, together with akinetic mutism and myoclonus, constituted an easily recognizable picture. Treatment with idoxyuridine was ineffective in one case in which electronmicroscopy of brain biopsy material supported the diagnosis.

Decreased erythrocyte glutathione peroxidase activity in multiple sclerosis.

The mean activity of glutathione peroxidase (GSH‐PX) in erythrocytes of 22 Israeli‐Jewish patients with multiple sclerosis (19.3 ± 4.5 U/gHb) was significantly lower than in a control group of 30 Jewish patients with various neurological disorders (24.3 ± 5.1 U/gHb). This observation confirms a similar finding of a decreased activity of GSH‐Px in erythrocytes of multiple sclerosis patients in Denmark (Shukla et al. 1977). These results are discussed in relation to the possibility of selenium deficiency and to the recently described genetic polymorphism and ethnic variation of GSH‐Px activity in human red cells. It is concluded that additional investigations are required in order to elucidate the cause of the decreased activity of this enzyme in red cells of patients with multiple sclerosis.

Progressive supranuclear palsy: Electromyographic examinations of eye muscles

Electromyographic examination of vertical and lateral extraocular muscles was carried out in five patients suffering from progressive supranuclear palsy, and incapable of performing voluntary vertical eye movements. No evidence of a lower motor neurone lesion or paradoxical innervation of eye muscles was noted.

Methotrexate Treatment in Dermatomyositis

Two patients with dermatomyositis were treated with methotrexate, given intravenously. They were unresponsive to corticosteroids. The patients were bedridden and paralysed and one seemed to be in the terminal phase of the disease. The response to courses of methotrexate treatment was satisfactory and sustained over a 2-year follow-up period; muscle enzyme studies indicate that the muscle disease is no longer active. Although steroid therapy alone may be effective, its usefulness is unpredictable and may lead to dependence and undesirable side effects. Our experience with these two patients suggests that a combination of the two drugs may be justified as initial treatment in severe cases.

Oral phenylalanine and tyrosine tolerance tests in Parkinsonian patients.

Reduction of dopamine concentrations in the brains of patients with Parkinsonism, together with reported clinical improvement after the administration of dihydroxyphenylalanine, has led to the hypothesis that impaired hydroxylation of tyrosine may be associated with the disease. To test this hypothesis oral loading tests with L-phenylalanine and tyrosine were carried out in patients and controls. After phenylalanine lower blood levels of this were found in Parkinsonian patients than in controls, but tyrosine levels were the same. After tyrosine lower levels of this were also found in patients compared with controls. It is suggested that these findings indicate a decreased rate of tyrosine utilization in Parkinsons disease together with intestinal malabsorption; the latter is supported by the finding of abnormal D-xylose tolerance in these patients.

Periodic ataxia: An unusual non-familial variation with paroxysmal EEG features

SummaryA 19-year-old youth suffered from periodic attacks of ataxia and dysarthria. Abnormally high IgG and IgA levels were found in the CSF. The length of the episodes of ataxia, absence of family history and the presence of generalised paroxysmal features in the EEG constitute a combination which is not believed to have been recorded previously.Carbamazepine was ineffective but temporary clinical and electroencephalographic improvement followed the administration of ATCH. Acetazolamide therapy has resulted in prolonged remissions from attacks.ZusammenfassungEin 19jähriger Mann litt an periodischen Anfällen von Ataxie und Dysarthrie. Die IgG- und IgA-Werte im Liquor waren erhöht. Die episodische Ataxie, die fehlende Familienanamnese und die paroxysmalen Eigenschaften im EEG sind eine Kombination, die unseres Wissens bisher nicht beschrieben wurde.Carbamazepin war wirkungslos, doch ACTH brachte eine vorübergehende klinische und elektroencephalographische Besserung. Behandlung mit Acetazolamid resultierte in längeren Remissionen der Anfälle.

Hypokalemic periodic paralysis associated with multiple sclerosis.

A case is reported of a 46-year old male who has been suffering from recurrent episodes of quadriplegia, characteristic of hypokalemic periodic paralysis, for 28 years. During this period he has developed typical signs and symptoms of multiple sclerosis. The association of hypokalemic periodic paralysis and multiple sclerosis is extremely unusual.

Persistent coma and papilledema in parathion poisoning

SummaryA case of parathion poisoning in a young man is described; unusual features were coma persisting for 2 months till death, hemiplegia and gross papilledema associated with edema of the brain.ZusammenfassungDie Parathion-Vergiftung eines jungen Mannes wird beschrieben; ungewöhnliche Begleiterscheinungen waren zweimonatiges, bis zum Tode anhaltendes Koma, Hemiplegie und ausgeprägte Stauungspapillen verbunden mit Gehirnödem.

Chronic progressive and relapsing-remitting multiple sclerosis: two diseases?

In their article in your June issue, on immune complexes in multiple sclerosis patients (l), Henneberg et al. refer to the difference they had previously noted, between relapsing-remitting and chronic progressive types of the disease, namely the presence in the latter of sera1 antibrain antibodies, absent in the former (2). This difference, they now report, could not be attributed to the formation of immune complexes, and suggest that it might be inferred that the two clinical varieties are in fact really two different diseases. However, modern techniques, notably the extensive use of MRI, have shown that clinical relapses seem to bear little relationship to the ongoing pathological process, with many lesions appearing continuously in areas and numbers out of all proportion to the clinical manifestations (3-5). With this evidence in mind it could perhaps be that there is after all only one disorder, always chronic and progressive in the sense of ongoing basic pathology, but differing clinically in certain victims because of some as yet undiscovered defensive factor which prevents the majority of new lesions from evolving further to a stage which has a negative effect on neural function. The absence of antibrain antibodies would then possibly reflect in some way the nature of the mechanism responsible for this difference in the clinical progress of the two varieties, or in other words, whether this difference is due to the basic nature of the disease process (two different pathogenic agents) or to the state of the victim (one pathogenic agent, two different host reactions). This is the question raised by the evidence revealed by the MRI changes referred to above. The relative state of defence against exacerbations in the pregnant woman, as opposed to susceptibility to exacerbations in men exposed to minor infections as referred to by VanLambalgen et al. (8) could perhaps be regarded as being at least compatible with different responses to an identical aetiological background. It must also be remembered that in many patients the character of abrupt serious but temporary clinical deteriorations may eventually be replaced by a chronic progressive course. Minor changes in MRI, indistinguishable visually from those seen in MS, have also been noted in a rather surprising proportion of healthy young individuals (6). The questions has been raised (7) whether this means that clinically silent MS is far more ubiquitous than has previously been suspected. Could the same background of non-development of antibrain antibodies, be the explanation for this phenomenon? Also, the racial and geographical aspects of MS are fairly well defined; these could be expected to show differences if there are two distinct diseases.