A case-control study of Parkinson's disease in urban population of southern Israel.

BACKGROUND: In recent years, an increased prevalence of Parkinson's disease (PD) in southern Israel was observed. The aim of this study was to determine which exposures are associated with PD in the urban population of this region. METHODS: Ninety-three PD patients living in towns were compared to 93 age and sex matched controls. A previously validated questionnaire, including demographic data, education, data on exposures, previous diseases, family history and habits, was administered. RESULTS: In multivariate logistic regression analysis, it was found that history of work in construction sites was the strongest predictor of PD risk, followed by exposure to pesticides. In contrast, there was a negative association with smoking and history of mechanical factory employment. When the same statistical analysis was limited to association of PD with smoking, pesticides and construction work, the latter was found to be the strongest risk factor. CONCLUSION: The risk factors for PD in this population are work on a construction site and exposure to pesticides.

Transient hemichorea/hemiballismus associated with new onset hyperglycemia.

OBJECTIVE: To describe three patients suffering from transient hemichorea/hemiballismus associated with hyperglycemia, review previous reports and propose a possible pathophysiological explanation for this phenomenon. RESULTS: Our original cases and previously reported ones reveal a uniform syndrome: mostly female patients (F/M ratio of 11/2), 50-80 years old, usually with no previous history of diabetes mellitus (9/13), develop choreic or ballistic movements on one side of the body over a period of hours. Serum glucose levels are elevated. In most of the patients, a lowering of the blood sugar level reverses the movement disorder within 24-48 hours. CONCLUSIONS: We believe that the combination of a recent or old striatal lesion (causing increased inhibition of the subthalamic nucleus) and hyperglycemia (causing decreased GABAergic inhibition of the thalamus) may be responsible for the appearance of this unilateral hyperkinetic movement disorder. Undiagnosed diabetes mellitus should always be suspected in patients who develop hemiballistic or hemichoreic movements. When hyperglycemia is detected and corrected, the movement disorder usually resolves within two days and may not require symptomatic therapy with dopamine receptor antagonists.

Murine typhus presenting as subacute meningoencephalitis.

Murine typhus is a febrile systemic illness, presenting with headache and undulating fever. Neurological involvement is considered a rare complication. During 1994 and 1995, 34 patients admitted to our hospital were diagnosed as having murine typhus. Five of these patients presented with a syndrome of subacute "aseptic" meningitis or meningoencephalitis. Three had bilateral papilloedema and 2 had focal neurological signs. None had a rash or other systemic findings suggestive of rickettsial disease. The diagnosis was based on serum and cerebrospinal fluid serology and on prompt response to doxycycline therapy. These cases suggest that neurological involvement in murine typhus is more common than previously suspected and that murine typhus should be included in the differential diagnosis of subacute meningitis in endemic areas.

Cefuroxime-induced encephalopathy.

We describe four patients who developed an encephalopathic syndrome characterized by obtundation or stupor, myoclonic jerks, and asterixis in association with cefuroxime therapy. Three patients had renal failure. These cases suggest that cefuroxime in overdose or in conventional doses in patients with renal failure can cause a reversible encephalopathy. This syndrome may have been unrecognized because it usually occurs in severely ill patients with additional causes for encephalopathy.

A case-referent study of extrapyramidal signs (preparkinsonism) in rural communities of Israel.

BACKGROUND: In previous studies we reported an increased prevalence of Parkinson's disease in several kibbutzim of Southern Israel (cluster kibbutzim). Subsequent studies revealed a significant prevalence of subjects presenting extrapyramidal signs (preparkinsonism) in the same kibbutzim. On follow-up worsening of these signs was observed in some of the older subjects, some of them actually being diagnosed as suffering from 1-Dopa responsive Parkinson's disease. The current study was designed to evaluate possible etiologic factors for the development of preparkinsonism. METHODS: 317 subjects over the age of 40, living in five kibbutzim were examined and interviewed. 95 subjects presenting extrapyramidal signs were compared with 95 control subjects. They were matched for age, sex and length of residence in the kibbutz. Odds ratios were computed to identify exposure variables for logistic regression analyses. Detectors for carbamates and organic phosphates were applied at different sites of these kibbutzim. RESULTS: The severity and frequency of the extrapyramidal signs were higher in the older age groups, more in the "cluster", than in other kibbutzim. A very strong association was found between field crop work exposure, particularly cotton, and preparkinsonism (p = 0.0007) and a slightly weaker association for landscape work. The detectors picked up abundant pesticide traces (carbamates and organic phosphates) in the residential areas fairly distant from sites of aerial spray. CONCLUSIONS: We assume a chronic passive exposure of the residents in these kibbutzim to pesticides, in addition to any occupational exposures.

Dynamics of parkinsonism-Parkinson's disease in residents of adjacent kibbutzim in Israel's Negev.

From serial studies of clinical findings and Webster's scores, we determined the incidence of Parkinson's disease and the pattern of progression of the components of parkinsonism in residents older than 40 in rural settlements with unusually high rates of Parkinson's disease in southern Israel (Negev). In 1988, the authors reported on three adjacent kibbutzim at which were identified 13 cases of Parkinson's disease in a population of 413 persons older than 50 years. In the region as a whole, there were 143 additional cases among a population of 64,185 who were 40 years of age or older. The age-standardized prevalence ratio for the kibbutz population was 5.43. By 1993, 2 of the persons with Parkinson's disease had died, and 8 new cases had been diagnosed. The age-standardized prevalence ratio in 1993 was 8.09. The crude incidence ratio for persons older than 40 was 3.4 per 1000 per year. Webster's score is used as an estimator of the severity of pre-parkinsonism. In the normal population of elderly persons from other areas in the Negev, only 20 of 110 had a score greater than 0 (11, 8, and 1 scored 1, 2, and 3, respectively). When the Webster's score was first applied systematically-in 1989 to asymptomatic persons from the kibbutzim who were older than 40-46 of 104 had scores of 2 or more, and some subjects also had impaired vibration sense. The tests were repeated by different observers on a casual sample in 1991 and 1993. Of the persons older than 60 in 1989 (n = 14), the scores increased from an average of 2.3 in 1989 to 3.9 in 1991 and to 5.7 in 1993. In a younger sample (n = 7), the mean scores were 2.3 in 1989, 3.6 in 1991, and 2.9 in 1993. These results are evidence of an age-specific peak in incidence and prevalence in the 70- to 79-year-old age group and may represent a cohort exposure phenomenon. The results may also reflect the critical role of age in the transformation of pre-parkinsonism to Parkinson's disease. Finally, in this population with high initial rates of Parkinson's disease, there was evidence of pre-parkinsonism in about half of those older than 40, and active progression of pre-parkinsonism in those older than 60. Therefore, this group is an ideal population to test for effects of preventive interventions.

The effect of chronic one-eye patching on ocular myoclonus.

Ocular myoclonus developed in our 34-year-old patient 4 months after massive brain stem hemorrhage due to eclampsia. On chronic patching of the left eye, the vertical pendular nystagmus in the fixating right eye disappeared, whereas the covered eye was esotropic. While fixating, the left eye showed horizontal pendular nystagmus. With both eyes fixating, the right eye presented markedly vertical pendular nystagmus and the left eye a horizontal pendular nystagmus. It seems that chronic patching of one eye has a beneficial modulating effect on the vertical pendular nystagmus in the fixating eye.

Spontaneous vertical eye movements associated with pontine lesions.

Ocular bobbing, dipping and "reversed" ocular dipping were observed and recorded in two patients presenting a severe neurological symptomatology: quadruplegia and coma in one and locked-in syndrome in the other. CT scan showed a pontine infarction in both these patients. This is the first time that the ocular dipping and "reversed" ocular dipping are related to such an anatomic structure.

A possible harmful late effect of methylprednisolone therapy on a time cluster of optic neuritis.

Clinical records of all patients (n = 26) suffering from optic neuritis during the last 16 years re-evaluated in relation to treatment. The patients were treated in 3 ways: with prednisone; with "pulse" megadoses of methylprednisolone; and untreated. A possibly frightening conversion to multiple sclerosis was seen in the group of patients with methylprednisolone, compared with the other 2 groups.

Clustering of Parkinson's disease in southern Israel.

On three adjacent kibbutzim (collective rural communities) in the Negev (Southern Israel) 13 parkinsonian patients were found among a population of 592 persons 40 years or older. The clinical picture was not different from that of patients from other areas with idiopathic parkinsonism. Long term residence in the kibbutzim is characteristic of this population. In the past most of the drinking water has been supplied by wells from a common aquifer. From other patients with Parkinson's disease in the Negev, we estimated the age-specific incidence for the region. The incidence is about five times greater in each of these kibbutzim than in the remainder of the Negev. Although associations with rural residence and well water use have been reported elsewhere, clusters of this sort have not been reported. They strongly suggest that a common environmental factor exists.

Motor neuron disease in textile factory workers.

Five cases of motor neuron disease occurred during the period 1983-1987 in 4 small and medium-sized textile factories. These cases were 45% of 11 motor neuron disease patients diagnosed in this period in a population of about 300,000 persons. This observations may be relevant to toxic occupational hazards in the pathogenesis of motor neuron disease.

Saccadic intrusions in internuclear ophthalmoplegia.

Saccadic intrusions disrupted forward fixation in four patients with bilateral internuclear ophthalmoplegia (INO). Three had multiple sclerosis and one had brainstem infarction. Sporadic bursts of monocular abducting saccades were recorded in either eye. Each saccade was followed by a slower return to midposition. The presence of the INO prevented conjugate saccades of the fellow eye. The adducting phases were faster than convergence eye movements or the glissadic smooth eye movements that sometimes follow saccades. Dynamic overshoot of the abducting saccadic phase may contribute to the relatively rapid adducting phase. The saccadic intrusions of INO are recognized clinically as monocular horizontal oscillation; low-amplitude intrusions are evident only during funduscopy. This distinctive dyskinesia is considered a sign of involvement extrinsic to the medial longitudinal fasciculi in patients with INO.

Cerebral square wave jerks.

Abnormal saccadic intrusions consisting of frequent sporadic horizontal saccades followed, after an interval, by corrective saccades occurred in 70% of 17 patients with acute or chronic focal cerebral lesions. These square wave jerks were significantly lower in amplitude than those in cerebellar system disease. The metrics of these jerks were uniform regardless of the site of cerebral damage. Mean durations approximated the reaction time for saccadic refixations triggered by visual feedback. Very short-latency corrective saccades in some patients are attributed to internal (nonretinal) feedback of eye position errors. Low-amplitude cerebral square wave jerks can be detected clinically by funduscopy.