An uncommon pattern of polyneuropathy induced by lifetime exposures to drift containing organophosphate pesticides.

The natural history of chronic peripheral polyneuropathy following lifetime low-level organophosphate (OP) exposure was investigated. A pilot study (1984-1987) conducted in rural communities in Israel detected subtle reversible in-season changes in nerve conduction patterns of 17 field workers out of 214 residents exposed to seasonal drift containing OP's. We examined 60 individuals (males: 50/60; 83.3%) from the original cohort still residing (more than 40 years) in the same communities. Exposure assessment was based on reports by Israeli institutions and the Bureau of Statistics. Information on personal status, work experience, exposures and symptoms was collected by questionnaires. The nervous system was systematically studied, evaluating cortical upper motor neurons, corticospinal tracts, lower motor neurons and peripheral nerves. Electrophysiological studies included conduction velocities, amplitudes and distal latencies of sensory and motor median, ulnar, tibial and sural nerves; F-waves for proximal nerve functions; thermal and pain thresholds for small thinly-myelinated and non-myelinated fibers; transcranial magnetic stimulation for large fibers. Clinical and electrophysiological features of Carpal Tunnel Syndrome were found in 18% of the subjects, atypically in males only. Fingertips' tingling correlated with both axonal and myelin-dependent parameters (lower wave amplitudes and prolonged latency periods, respectively) in the sensory median nerves bilaterally. OP exposure significantly correlated to prolonged distal latency in the right median sensory nerve (r=0.29; p=0.052; n=45) and lower wave amplitude in the right sural nerve (p=0.031). These findings attest to subtle, predominantly sensory peripheral polyneuropathy following lifetime low-level exposures to drifts containing OP.

Use of the Stroop phenomenon as a diagnostic tool for malingering.

AIMS: To assess a computerised version of the Stroop test for detection of malingering of cognitive deficit. METHODS: Sixty subjects were assessed using this test. Twenty had cognitive deficits due to brain damage of miscellaneous aetiologies. Ten were healthy, not acquainted with the test, and were asked to simulate cognitive impairment. Another 10 simulators were psychology students trained in the use of the test. Twenty healthy subjects served as controls. Results were analysed for reaction time, error percentage, and the Stroop effect. RESULTS: There was a significant difference in reaction time among groups, showing a direct relation of age among control subjects, and also longer reaction time in patients with brain damage than in controls. Controls and patients with brain damage showed a clear Stroop effect. Simulators had a significantly prolonged reaction time, increased error percentage, and inverted or absent Stroop effect. This alteration of the Stroop effect is never present in organic cognitive deficits and seems to be a characteristic pattern of feigning, independently of knowledge of the test. CONCLUSION: This technique is recommended as a valuable tool to detect feigned cognitive impairment.

Slow magnetic stimulation of prefrontal cortex in depression and schizophrenia.

1. The authors used transcranial magnetic stimulation (TMS) of pre-frontal cortex to study mood changes in 10 depressed patients and 10 schizophrenic patients. 2. A slow rate of stimuli was used, one per 30 seconds; maximal intensity of about 2 Tesla was given for 30 stimuli, 15 on each side of the brain. 3. No side effects were seen and at least three depressed patients and two schizophrenic patients appeared to improve, at least transiently. 4. These results suggest that rapid rate TMS may not be necessary to elicit mood effects.

Polymyositis, arthritis, and proteinuria in a patient with adult celiac disease.

A 37-year-old woman developed polymyositis and arthritis concomitantly with proteinuria and watery diarrhea. Repeated duodenal biopsies and serological evaluation established the diagnosis of adult celiac disease. Treatment with gluten-free diet resolved all clinical and laboratory abnormalities. We believe that this is the first report of adult celiac disease presenting as a multisystem disease involving kidneys, joint, and muscles.

Fatal familial infantile glycogen storage disease: multisystem phosphofructokinase deficiency.

An infant girl of consanguinous Bedouin parents suffered from fatal early onset of progressive generalized muscle weakness. Her older brother suffered from similar weakness and cardiomyopathy, which led to his death at the age of 21 months. A muscle biopsy performed on the propositus at the age of 9 months was PAS-negative, and showed nonspecific myopathic changes. A second muscle biopsy, performed at 21 months of age, a few days before her death, and postmortem study of heart and liver, disclosed excessive extralysosomal glycogen storage and reduced phosphofructokinase-1 (PFK-1) activity. Because the genes encoded for PFK-1 in liver and muscle are located on separate chromosomes, the reduced enzyme activity in both tissues could not be related to a single mutation for this enzyme. Activity of 6-phosphofructose-2-kinase (PFK-2), a recently discovered physiological activator to all PFK-1 isozymes, was normal in the liver. The possibility that this multisystem PFK-1 deficiency may be related to the absence of a yet unknown activator, common to all PFK-1 isozymes, is discussed.

Bulbo-pontine paralysis with deafness: the Vialetto-Van Laere syndrome.

A Caucasian girl developed slowly progressive sensory neural deafness and bulbar and spinal muscle weakness typical of the Vialetto-Van Laere syndrome. As the condition progressed the major disabilities became dysphagia, respiratory muscle weakness and postural hypotension. Treatment with gastrostomy feedings, oxygen and fludrocortisone acetate produced worthwhile functional improvement.

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.

Clustering of Parkinson's disease points to environmental etiology.

On three adjacent kibbutzim in the Negev (southern region) of Israel, 13 cases of Parkinson's disease were found in a population of 592 persons who were at least 40 yr of age. There were no clinical (or other) findings that distinguished these Parkinson's disease cases. Long-term residence is characteristic of this population. During the past 40 yr, water has been supplied to these persons via wells from a common aquifer. On the basis of local age-specific incidence data, no difference in age distribution was found between clustered and nonclustered cases. The incidence of Parkinson's disease is about five times greater in each of the three kibbutzim than in the remainder of the region. The three kibbutzim in the cluster use similar agricultural chemicals, as do other kibbutzim. Although associations with rural residence and well water use have been reported elsewhere, clusters of this sort have not been reported. Clusters strongly suggest that common environmental factors exist. Drinking water and agricultural chemicals are the most likely common environmental factors.

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.

Inclusion body myositis in post-poliomyelitis muscular atrophy.

A 38-year-old male developed a new muscle weakness in his left thigh 35 years after having acute paralytic poliomyelitis with residual right distal leg weakness and atrophy. EMG studies showed widespread denervation in proximal and distal muscles regardless the clinical involvement. Muscle biopsy from an affected muscle showed the findings of inclusion-body myositis consisting of perivascular and interstitial mononuclear infiltration, sarcoplasmic granular inclusions with membranous whorls and typical filamentous inclusions in several myonuclei. This raises the possibility of inclusion body myositis in other cases of progressive post-poliomyelitis muscular atrophy, especially those with perivascular infiltration of mononuclear cells in the muscle biopsy.

Peripheral neuropathy and cerebellar syndrome associated with amiodarone therapy.

A 52-year-old woman developed symmetric sensorimotor polyneuropathy and cerebellar syndrome after 24 months of amiodarone treatment. Sural nerve biopsy revealed mild demyelination and many inclusion bodies in the axoplasm and in Schwann cell cytoplasm. Improvement in her condition was evident 6 months after discontinuing amiodarone.

Myophosphorylase deficiency: the course of an unusual congenital myopathy.

A 59-year-old man had proximal weakness and wasting that started in early childhood. EMG was "myopathic," serum CK activity was increased, and muscle biopsy showed accumulations of glycogen. Biochemical studies revealed elevated glycogen concentration and absence of myophosphorylase activity. This unusual presentation of a long-standing, painless, and quite static weakness due to myophosphorylase deficiency represents another example of clinical heterogeneity.

Neurologic complications after gastric restriction surgery for morbid obesity.

We report the occurrence of neurologic complications in 23 patients who underwent gastric restriction surgery for the treatment of morbid obesity. Complications occurred 3 to 20 months after surgery. All the patients had had protracted vomiting for the first 3 months after the operation. The following syndromes were found: chronic or subacute symmetric polyneuropathy (12 patients), acute severe polyneuropathy (1 patient), burning feet syndrome (2 patients), meralgia paresthetica (3 patients), myotonic syndrome (1 patient), posterolateral myelopathy (2 patients), and Wernicke-Korsakoff encephalopathy (2 patients). The patients suffering from burning feet syndrome and those with Wernicke-Korsakoff encephalopathy showed a clear improvement after parenteral thiamine treatment. As to the rest of the patients, the occurrence of the complications seems to be linked to nutritional causes, although no such deficiencies were detected.

Vitamin B12 deficiency neuropathy: sural nerve biopsy study.

A 40-year-old woman developed neuropathy and myelopathy with vitamin B12 deficiency without anemia. Motor and sensory nerve conduction velocities were moderately decreased. Sural nerve biopsy showed the coexistence of demyelination and axonal degeneration.