A new autosomal dominant pure cerebellar ataxia.

A kindred is described with a dominantly inherited "pure" cerebellar ataxia in which the currently known spinocerebellar ataxias have been excluded. In the eight subjects studied, a notable clinical feature is slow progression, with the three least affected having only a mild degree of gait ataxia after three or more decades of disease duration. Pending an actual chromosomal locus discovery, the name spinocerebellar ataxia (SCA)15 is expectantly applied.

Chronic inflammatory demyelinating polyneuropathy associated with membranous glomerulonephritis: case report.

A case of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) with Membranous Glomerulonephritis (MGN) is reported. This is the second case recorded in the literature and the article compares this case with the other reported case, including immunological implications.

Streptokinase for acute ischemic stroke with relationship to time of administration: Australian Streptokinase (ASK) Trial Study Group.

OBJECTIVES: To determine whether the administration of 1.5 million units of streptokinase intravenously within 4 hours of the onset of acute ischemic stroke would reduce morbidity and mortality at 3 months and whether outcomes may be better for those receiving therapy within 3 hours of stroke onset compared with those receiving it after 3 hours. DESIGN: Randomized, double-blind, placebo-controlled trial with 3-month follow-up. PARTICIPANTS: A total of 340 patients, aged 18 to 85 years, with moderate to severe strokes were randomized from 40 centers throughout Australia from June 1992 to November 1994. INTERVENTION: Administration of 1.5 million units of streptokinase or placebo intravenously in 100 mL of normal saline over 1 hour. MAIN OUTCOME MEASURE: Combined death and disability score (Barthel index <60) 3 months after the stroke. RESULTS: Using an intention-to-treat analysis with a combined death and disability score at 3 months after stroke as the primary end point, we found a nonsignificant overall trend toward unfavorable outcomes for streptokinase vs placebo (relative risk [RR] of unfavorable outcome, 1.08; 95% confidence interval [CI], 0.74-1.58) and an excess of hematomas (13.2%[12.6% symptomatic] in the treated group, 3% [2.4% symptomatic] for placebo [P<.01]). However, poor outcomes were confined to patients receiving therapy more than 3 hours after stroke onset (RR of unfavorable outcome, 1.22; 95% CI, 0.80-1.86). In contrast, among the 70 patients who were entered into the trial within 3 hours of stroke onset, there was a trend toward improved outcomes for those who received streptokinase (RR of unfavorable outcome, 0.66; 95% CI, 0.28-1.58), and this outcome pattern was significantly better than for those receiving therapy after 3 hours (P=.04). Streptokinase administration resulted in excess deaths in the group treated after 3 hours (RR, 1.98; 95% CI, 1.18-3.35), but not among those treated within 3 hours (RR, 1.11; 95% CI, 0.38-3.21). CONCLUSION: The administration of streptokinase within 4 hours of acute ischemic stroke increased morbidity and mortality at 3 months. While treatment within 3 hours of stroke was safer and associated with significantly better outcomes than later treatment, it showed no significant benefit over placebo. The timing of thrombolytic therapy for acute stroke is critical.

The use of high doses of adrenaline in paediatric cardiac resuscitation.

Adrenaline has been used in cardiac resuscitation for many years, yet until recently its mechanism of action and optimal dosage remained poorly investigated or understood. Recent guidelines suggest the use of higher doses of adrenaline at an early stage in paediatric resuscitation. This paper examines the use of adrenaline in paediatric resuscitation and studies the arguments in favour of using higher doses than previously accepted.

Focal cerebral ischemia and antiphospholipid antibodies: a case for cardiac embolism.

We report clinical, neuroimaging and immunological findings in seven women with antiphospholipid antibodies (APLA) and cerebral ischemia. Two patients had systemic lupus erythematosus (SLE) and five had the antiphospholipid syndrome (APS). Autopsies were done in 3 women who died acutely with focal neurological deficits. Evidence for cerebral embolism was found in all patients: a) pathology demonstrated multiple cerebral infarctions and cerebral emboli from underlying non-bacterial thrombotic endocarditis (NBTE) in the patients who died. b) Three patients had thickened mitral valves and embolic cerebral occlusions were identified with cerebral angiography. c) In one patient, echocardiography detected a thrombus on the posterior leaflet of a prolapsing mitral valve at the time of ictus. Our data provide further evidence to implicate valvular endothelium in the genesis of cerebral ischemia in some patients with APLA.

Finger sudorometry and assessment of the sudomotor drive.

Sudorometry of the finger was carried out using the ventilated capsule method, the aim being to use the level of relative humidity within the sudorometer as an indirect measure of the sudomotor drive. Subjects inserted a finger through a diaphragm of a finger-shaped, temperature-controlled chamber which also contained the humidity sensor. Manoeuvres known to alter the sudomotor drive produced changes in chamber humidity. The relative humidity within the sudorometer became constant after local anaesthesia of the digital nerves and after upper limb sympathectomy, suggesting that fluctuations in the sudorometer output were dependent upon an intact autonomic nervous system. In an environment in which temperature was controlled and arousal effects from the process of measurement were minimised, chamber humidity always increased during a Stroop test, providing a rapid means of indirectly assessing sudomotor drive mechanisms.

Multiple sclerosis: report of a case in a Papua New Guinean.

Multiple sclerosis is rare in equatorial countries and has not been diagnosed in the indigenous population of Papua New Guinea. We describe the clinical features of a young Papua New Guinean with optic neuritis and a myelopathy which we believe to be due to multiple sclerosis.

Variability of repeated nerve conduction studies.

We have determined the variability of repeated measurements of sensory nerve action potential (SNAP) and compound muscle action potential (CMAP) amplitude and motor and sensory conduction velocity (MCV and SCV) and examined the extent to which limb temperature is responsible for the variability. We made 10 serial measurements of SNAP, CMAP, MCV and SCV in each of 3 nerves in a single normal subject. The coefficients of variation for MCV and SCV ranged from 2.0% to 6.7% and the proportion of the variance due to temperature was 0.3-56%. The coefficients of variation were much greater for serial measurements of compound action potential amplitude. We used the standard deviations for serial measurements in each nerve to calculate the number of subjects required to detect a difference of 1/msec between the means of two sets of measurements with a power of 90%.

Social, neurological and cognitive disorders in alcoholics.

OBJECTIVES: To construct the social, neurological and neuropsychological profiles of patients with suspected alcohol related brain damage, and to test the hypothesis that cognitive dysfunction develops in alcoholics before signs of alcohol related neurological disease. DESIGN: The study design comprised: a retrospective analysis of patients' records; a comparison of patients' demographic and social variables with age-matched population data from the 1986 Census; and a comparison of neuropsychological test scores of male patients with suspected alcohol related brain damage and age-matched controls. SETTING: The Neuropsychology Unit of Royal Prince Alfred Hospital, a tertiary referral teaching hospital. PARTICIPANTS: All patients (n = 641) referred to the Neuropsychology Unit for assessment of suspected alcohol related brain damage from July 1, 1987 to June 30, 1989, and a control group (n = 93) consisting of healthy volunteers who drank no more than 40 g of alcohol per day. RESULTS: Of the patients, 515 (80%) were men and 410 (64%) were 55 years or younger; their median daily alcohol consumption was 180 g. Compared with the general population the patients were significantly more likely to be in receipt of social security payments, to be separated, divorced or widowed and living in rented or temporary accommodation. However, the proportion of professional and skilled individuals was the same as in the general population. Cerebellar degeneration (38%), peripheral neuropathy (34%) and seizures (14%) were the most frequent neurological disorders. Frontal lobe dysfunction (58%) and short-term memory loss (32%) were the most frequent cognitive abnormalities. Dementia was relatively uncommon (4%). The mean age of male patients with alcohol related brain damage but no neurological disorders (43 years) was significantly less than the mean age of those with neurological disease (54 years; P less than 0.001). In the former, neuropsychological tests of frontal lobe function showed significant impairment compared with control subjects (P less than 0.001), despite normal intelligence. CONCLUSIONS: Cognitive impairment in alcoholics frequently takes the form of frontal lobe dysfunction and may be relatively subtle, requiring a neuropsychological examination for diagnosis. Signs of cognitive impairment may precede those of alcohol related neurological disorders by more than ten years.

Growth of tracheal anastomoses: advantage of absorbable interrupted sutures.

Growth of the trachea after complete transection and anastomosis was studied in four groups of 1-month-old New Zealand white rabbits. The trachea was transected at the fifth cartilaginous ring and then anastomosed with continuous 6-0 polypropylene (Prolene) (group 1), interrupted 6-0 polypropylene (group 2), continuous 6-0 polydioxanone (PDS) (group 3), or interrupted 6-0 PDS (group 4). The animals were followed up for 90 to 103 days (mean follow-up, 95 days). At the time the animals were killed, body weight had increased 125% (1.2 to 2.7 +/- 0.18 kg). Growth of the trachea was assessed at the time of death. Results from this study suggest that growth of a tracheal anastomosis is retarded in a growing animal model. The degree of resultant stenosis was significantly less when an absorbable suture material (PDS) and an interrupted suturing technique were used.

Effect of social drinking on neuropsychological performance.

The effects of social drinking on neuropsychological function have been assessed in a group of healthy male volunteers. Subjects were divided into three groups according to their daily alcohol consumption: (1) 40 g or less (n = 93), (2) 41-80 g (n = 22), (3) 81-130 g (n = 16). Group 1 had been drinking at the present level for a mean of 12.6 years, group 2 for 16.9 years and group 3 for 15.1 years; the differences are not significant. There are no significant differences on any neuropsychological tests variables between groups 1 and 2. However, subjects in group 3 were found to perform at a significantly lower level than groups 1 and 2 on the Rey Auditory Verbal Learning Test, the Austin Maze, and the Little Man and Spatial Memory Tests of the Bexley Maudsley Automated Psychological Screening Test. The pattern of deficits found in heavy social drinkers is less severe but otherwise similar to that found in alcoholics.

Disorders of the autonomic nervous system: Part 2. Investigation and treatment.

Autonomic function may be adequately tested with noninvasive tests of sympathetic and parasympathetic pathways, including: the response of blood pressure to change in posture and isometric contraction, heart rate response to standing, variation in heart rate with respiration, Valsalva ratio, sweat tests, and plasma noradrenaline measurements. Abnormal results in two or more of these tests indicate autonomic dysfunction. Intraarterial catheterization and tests of vasomotor function are usually required only in doubtful cases or for research purposes. Treatment of autonomic dysfunction is focused primarily on bladder control and control of orthostatic hypotension. Orthostatic hypotension is best treated with physical measures, pharmacologically with 9-alpha-fluorohydrocortisone and dihydroergotamine mesylate. A number of other agents may be tried but results have been less effective.

Disorders of the autonomic nervous system: Part 1. Pathophysiology and clinical features.

Autonomic dysfunction may result from diseases that affect primarily either the central nervous system or the peripheral autonomic nervous system. The most common pathogenesis of disturbed autonomic function in central nervous system diseases is degeneration of the intermediolateral cell columns (progressive autonomic failure) or disease or damage to descending pathways that synapse on the intermediolateral column cells (spinal cord lesions, cerebrovascular disease, brainstem tumors, multiple sclerosis). The peripheral autonomic nervous system may be damaged in isolation in the acute and subacute autonomic neuropathies or in association with a generalized peripheral neuropathy. The peripheral neuropathies most likely to cause severe autonomic disturbance are those in which small myelinated and unmyelinated fibers are damaged in the baroreflex afferents, the vagal efferents to the heart, and the sympathetic efferent pathways to the mesenteric vascular bed. Acute demyelination of the sympathetic and parasympathetic nerves in the Guillain-Barré syndrome may also cause acute autonomic dysfunction. Although autonomic disturbances may occur in other types of peripheral neuropathy, they are rarely clinically important.