Neuromuscular pathology in hereditary gelsolin amyloidosis.

Hereditary gelsolin amyloidosis (AGel amyloidosis) is a systemic disorder reported worldwide in kindreds with a G654A or G654T gelsolin gene mutation. The clinically characteristic peripheral nerve involvement has been poorly characterized morphologically, and its pathogenesis remains unknown. We studied peripheral nerve and skeletal muscle biopsy or autopsy specimens of 35 patients with a G654A gelsolin gene mutation.

Is EEG useful in assessing patients with acute encephalitis treated with acyclovir?

EEG has been used widely in diagnosing encephalitis, as it demonstrates rather typical abnormalities, especially in herpes simplex virus encephalitis (HSVE). We analysed 204 EEG recordings from 98 consecutive acyclovir-treated patients with acute encephalitis between 1984 and 1994. Periodic complexes (PC) in the acute phase predicted poor outcome (Kendall tau 0.

Unilateral hyperfusion in brain-perfusion SPECT predicts poor prognosis in acute encephalitis.

We studied 88 patients with acute encephalitis using hexamethylpropyleneamine oxime and single photon emission computed tomography (SPECT). All patients had been initially treated with intravenous acyclovir. The etiology could be disclosed in 37 patients (42%), which included 15 patients with herpes simplex encephalitis, 7 with varicella-zoster encephalitis, and 29 with other encephalitides (Mycoplasma, adenovirus, influenza, rotavirus, rubella, Epstein-Barr, arbovirus, syphilis, and tuberculosis).

Significance of the second somatosensory cortex in sensorimotor integration: enhancement of sensory responses during finger movements.

The functional significance of the second somatosensory cortex (SII) is poorly understood. However, lesion and cortical stimulation studies indicate that SII may be involved in sensory aspects of tactile learning and in movement control. In the present study, we explored a possible role of SII in sensorimotor integration in humans using a multichannel magnetometer.

Acute effects of 1,1,1-trichloroethane inhalation on the human central nervous system.

The object of this study was to examine the immediate nervous effects of variable 1,1,1-trichloroethane (TCE) exposure combined with physical exercise. The effects on the quantitative electroencephalography (EEG), visual evoked potentials (VEP) and body sway were analyzed. Nine male volunteers were exposed to either a stable or a fluctuating exposure pattern with the same time-weighted average concentration of 200 ppm (8.

Activation of ipsilateral primary sensorimotor cortex by median nerve stimulation.

We report evidence for activation of ipsilateral primary sensorimotor cortex (SMI) after median nerve stimulation recorded with magnetoencephalography (MEG). We measured somatosensory evoked magnetic fields (SEFs) to median nerve stimulation with a 122-channel helmet-shaped magnetometer in 10 healthy subjects. In five, the magnetic field patterns suggested long-latency activation of the ipsilateral SMI.

Neuromuscular dysfunction after surgical treatment of oral cancer.

Surgical treatment of oral cancer usually includes radical dissection of the neck, in which the sternocleidomastoid muscle is sacrificed. In modified neck dissection the accessory nerve is spared, but may be severed by e.g.

Neuropathy in familial amyloidosis , Finnish type (FAF): electrophysiological studies.

We report, for the first time, electrophysiological findings in the Finnish type of familial amyloidosis (FAF), a gelsolin-related form of systemic amyloidosis. Electromyography, nerve conduction studies, and blink reflex examinations were performed in 30 patients (age range 27-74 years). Cranial nerve involvement was detected in all, and peripheral nerve involvement in the majority of patients.

Neurological recovery after liver transplantation: a prospective study of 22 patients.

A group of 22 liver transplantation patients were examined pre- and postoperatively using clinical neurological, neurophysiological and neuroradiological methods. After the operation improvement was observed in neurological symptoms, and in neuropsychological and neurophysiological test results. Our study shows that liver recipients have a high prevalence of nervous system dysfunction and that successful transplantation is followed by significant improvement.

Tetanus caused by occupational accidents.

The role of occupational accidents as a source of tetanus is poorly known. In Finland, during the period 1969-1985, 28 (26%) of the 106 cases of tetanus were caused by occupational accidents, 16 of which occurred in agriculture and forestry. Twenty-one of the patients were men and seven were women.

Metabolic control and progression of complications in insulin-dependent diabetic patients after kidney transplantation.

Patient survival and progression of complications were monitored for 3 years after kidney transplantation in 29 type-1 diabetic patients. Ten age-matched, non-diabetic kidney-transplanted patients served as controls. Five diabetic patients died during follow-up (three cardiovascular events, two infections), three diabetic patients had a non-fatal myocardial infarction and four developed cerebrovascular complications after transplantation.

Encephalopathy and neuropathy in end-stage liver disease before and after liver transplantation.

The nervous system involvement of 8 patients with end-stage liver disease was evaluated by means of clinical neurological, neuropsychological, neurophysiological and neuroradiological investigation before and 6-12 months after a successful liver transplantation. Preoperatively, all subjects (7 women, 1 man; mean age 40 years, range 30-54 years) exhibited decreased muscle strength and 2 patients manifested clinical signs of polyneuropathy. In neuropsychological tests, slight visuoconstructive apraxia, and disturbances of verbal memory and cognitive function were observed.

Sustained high plasma 5-aminolaevulinic acid concentration in a volunteer: no porphyric symptoms.

The pathogenesis of the acute porphyric attack is not known. One hypothesis is that porphyrin precursors, especially 5-aminolaevulinic acid (ALA), are toxic for neuronal tissue. This was tested by infusing ALA in a male volunteer after a loading dose at a rate of 50-80 mg h-1 for 92.

Obstructive sleep apnoea syndrome in morbidly obese patients.

Twenty-seven morbidly obese patients (13 men and 14 women) with body mass index greater than or equal to 40 kg m-2 were examined. The mean age of the subjects was 36.9 +/- 8.

Misuse of zopiclone and convulsions during withdrawal.

It has been documented that benzodiazepines have the potential to cause dependence and withdrawal reactions, including convulsions. However, the available data concerning zopiclone, a nonbenzodiazepine hypnotic, are insufficient. The present study describes the case of a 36-year-old man who repeatedly misused zopiclone, in daily doses of 60-90 mg.

Electroencephalographic findings during experimental human exposure to m-xylene.

Aromatic hydrocarbon solvents, used widely in industry, cause central nervous system symptoms in exposed workers. Acute effects of m-xylene were studied in nine voluntary subjects exposed experimentally to stable or varying concentrations of m-xylene at rest or while exercising. Each subject participated in four exposure and two control sessions in a single-blind fashion.

Electroneuromyographic sequelae of tetanus, a controlled study of 40 patients.

To assess the peripheral nervous system involvement caused by tetanus 40 patients recovered from tetanus and their sex and age matched referents were submitted to electroneuromyography (ENMG). Clinical symptoms and signs of peripheral neuropathy were found in 6 patients, 3 of whom had other obvious aetiology than tetanus. EMG was examined of the masseters and three muscles in the extremities.

Electroencephalography in tetanus.

To assess the brain involvement caused by tetanus, we examined the EEGs of 39 patients recovered from tetanus and those of 39 sex- and age-matched controls. The patients had significantly more frequent and more severe EEG abnormalities than their controls. The most marked difference was in the occurrence of diffuse EEG abnormalities (P less than 0.

Changes induced by short-term xylene exposure in human evoked potentials.

Nine healthy male volunteers were exposed to m-xylene for 3 h in the morning and 40 min in the afternoon with a 40-min break in between. The atmospheric m-xylene concentrations were either stable at 8.2 mumol/l (200 ppm) or they fluctuated (5.

Neurophysiological approaches to the detection of early neurotoxicity in humans.

Various neurophysiological methods, including electroencephalography, electromyography, nerve conduction velocities, and evoked potential techniques, have been used to detect early signs of neurotoxicity in humans. These methods have been applied to groups of occupationally exposed workers and their referents in epidemiologic studies, to patients with suspected or proven diseases after long-term work in toxic environment, and to human subjects during or after experimental exposure. The main body of knowledge arises from epidemiologic studies of occupationally exposed subjects, and several chemicals widely used in industry have been shown to be neurotoxic.

The effect of exposure to high and low frequency hand-arm vibration on finger systolic pressure.

Twenty-three patients with hand-arm vibration exposure and diagnosed vibration syndrome were given a thorough clinical and neurophysiological examination, together with finger strain gauge plethysmography. Eleven of the patients were forest workers regularly using chain saws (low frequency vibration exposure), and twelve were metal grinders (higher frequency vibration exposure). Both groups had significantly lower finger blood pressures than healthy controls, and comparisons between the groups indicated that the mean values tended to be lower in the grinders.