A predominantly left upper limb sensory neuronopathy as a manifestation of a metastatic neuroendocrine malignancy.

An 83-year-old woman presented with a predominantly left upper limb sensory ganglionopathy. She was found to have metastatic, poorly differentiated, neuroendocrine malignancy. Anti-neuronal antibodies were not detected.

Creutzfeldt-Jakob syndrome presenting as epilepsia partialis continua.

Creutzfeldt-Jakob disease (CJD) is an uncommon rapidly progressive neurological disorder which can have protean clinical presentations. We report an autopsy-proven case of CJD presenting initially as epilepsia partialis continua but then developing the typical clinical features of CJD over several weeks. CJD should be considered in the differential diagnosis of new onset epilepsia partialis continua in adults.

Behavioural changes due to pontine and extrapontine myelinolysis.

OBJECTIVE: To report a case of central and extrapontine myelinolysis with unusual behavioural manifestations that developed after slow (not more than 0.5 mmol sodium per litre per hour) correction of hyponatraemia. CLINICAL FEATURES: A 51-year-old Caucasian woman with hyponatraemia caused by psychogenic polydipsia experienced a delayed onset of behavioural changes with some ataxia, but no pyramidal signs, after correction of the hyponatraemia. INTERVENTION AND OUTCOME: The hyponatraemia was corrected with a combination of hypertonic saline and fluid restriction at the currently recommended rate of not more than 0.5 mmol of sodium per litre per hour. CONCLUSION: Central and extrapontine myelinolysis may develop after slow correction of hyponatraemia. Behavioural manifestations may be the most prominent clinical feature.

Saccade onset and offset lambda waves: relation to pattern movement visually evoked potentials.

The lambda (lambda) wave is an occipital EEG potential which occurs when saccadic eye movements are made against an illuminated contrast background. There is some disagreement concerning the presence of sub-components to the lambda-wave, and its relationship to visually evoked potentials. In the present study, lambda-waves were recorded with saccades of different durations (30-110 ms) and compared to VEPs associated with pattern movements of similar durations and velocity. It was found that the lambda-wave consisted of a saccade onset component with positive sub-components at 59 and 100 ms after saccade onset, and a saccade offset component with a positive potential at 74 ms after saccade offset. With small saccades of 30 ms duration or less, these components superimposed to form a single lambda-wave. In the case of pattern movement VEPs, a movement onset component of latency 110 ms following movement onset, and a movement offset component at 89 ms after movement offset, were identified. The similar behaviour of the lambda-wave and VEP under these conditions supports the view that the lambda-wave is a visually evoked potential resulting from movement of the visual field across the retina during a saccadic eye movement.

Dystonia and intracerebral calcification: pseudohypoparathyroidism presenting in an eleven-year-old girl.

Pseudohypoparathyroidism is an uncommon cause of movement disorders. We describe the case of an otherwise well 11-year-old girl, who presented with dystonia and was subsequently shown to have the classical metabolic abnormalities of pseudohypoparathyroidism. Cranial computerised tomographic scanning (CT scanning) showed intracerebral calcification of the basal ganglia and frontal lobes.

Crossed facilitation and post-contraction depression of abductor pollicis brevis motor neurons.

Crossed facilitation of abductor pollicis brevis (APB) motor neurons by voluntary contraction of the contralateral APB was investigated using F response analysis. The integrated F response EMG showed a mean 4-fold increase during contraction. This enhancement was maintained until the onset of subjective fatigue and then declined. After isometric or isotonic contraction of the ipsilateral APB there was a significant depression of F responses with a progressive return to baseline values over a 6 minute period. The significance of the findings is discussed.

Weakness of head turning in hemiplegia: a quantitative study.

The strength of head turning was measured in normal subjects and in patients with hemiplegia of vascular origin. In normals the strength of head turning to the two sides was not significantly different. In most patients with a cortical, capsular or high brainstem lesion the strength of head turning away from the side of the lesion was reduced. In patients with a medullary lesion the strength of head turning towards the side of the lesion was reduced. The significance of these findings is discussed in relation to the cortical control of the sternocleidomastoid muscle and other muscles involved in the movement of head turning.

Peripheral autonomic surface potential. A quantitative technique for recording sympathetic conduction in man.

Using EMG equipment with time locked recording and standard averaging facilities we were able to record a previously poorly defined skin potential. The potential, termed by us the peripheral autonomic surface potential (PASP), was recorded from the palmar surface of the hand and the plantar surface of the foot in 30 normal subjects. The PASP usually consisted of a biphasic potential with an initial negative and a later positive peak. It was elicited by randomly timed electrical stimuli over the median nerve at the wrist. The mean palmar PASP latency was 1.52 +/- 0.13 s and the mean plantar PASP latency was 2.07 +/- 0.16 s. The mean palmar and plantar PASP amplitudes were 479 +/- 105 microV and 101 +/- 40 microV, respectively. A mean sympathetic conduction velocity was calculated to be 1.28 +/- 0.18 m/s. In 5 normal subjects a similar technique was used to record a PASP from the volar surface of the left middle finger. Iontophoresis of atropine into the skin under the recording site abolished the PASP in all subjects. Palmar and plantar PASPs were recorded in 10 diabetic patients. The amplitudes (palmar 179 +/- 158 microV, plantar 17 +/- 16 microV) were significantly reduced compared to normals (P less than 0.001). Five patients with unilateral lumbar or cervical sympathectomy were studied. The PASP was absent or markedly reduced on the side of the sympathectomy in all patients.

Ocular and ocular motor aspects of primary thalamic haemorrhage.

As part of a correlative study of the neuro-ophthalmological abnormalities in thalamic haemorrhage, we report preliminary findings in six patients with this condition diagnosed by clinical and CT brain examinations. Five patients were middle-aged and hypertensive. Two had Horner's syndrome, and three anisocoria. Three patients had prominent mesencephalic dysfunction. There was pathological confirmation of the lesion in one case. The pathogenesis of the signs of midbrain disturbance in these patients is uncertain, but may comprise pressure, traction or hypoxia, separately or in combination. There was no positive correlation between the estimated size of the demonstrated haemorrhage and the severity of the neuro-ophthalmological abnormality.

Peripheral autonomic surface potential: a quantitative technique for recording autonomic neural function in man.

Using EMG equipment with time-locked recording and standard averaging facilities we were able to record a previously poorly studied skin potential. The potential, termed by us by the peripheral autonomic surface potential (PASP), was recorded from the palmar surface of the hand and the plantar surface of the foot in 20 normal subjects. The PASP consisted of a biphasic potential with an initial negative and a later positive peak. The mean palmar PASP latency was 1.51 +/- 0.19 seconds and the mean plantar PASP latency was 2.09 +/- 0.24 seconds. The mean palmar and plantar PASP amplitudes were 485 +/- 100 microV and 102 +/- 45 microV respectively. A mean central sympathetic conduction velocity was calculated at 1.2 +/- 0.21 m/s. In two patients with unilateral lumbar sympathectomy the ipsilateral plantar PASP was absent in one and of considerably reduced amplitude in the other. Both palmar and plantar PASPs were absent or of reduced amplitude in five patients with diabetes mellitus. The study indicates that the PASP may prove a useful quantitative test of central and peripheral autonomic function.

Brainstem auditory evoked responses in hereditary spinocerebellar ataxias.

Brainstem auditory evoked responses were recorded in 18 patients with spinocerebellar ataxia, seven with Friedreich's ataxia, four with late-onset cerebellar degeneration, and seven with olivopontocerebellar atrophy. All patients had normal hearing. BAERs were abnormal in 10 cases (58%). Five of the seven patients with Friedreich's ataxia (71%) had grossly abnormal BAERs with wave I only being identified. Five of the patients with olivopontocerebellar atrophy (71%) had abnormal BAERs, whereas all patients with cerebellar degeneration had normal BAERs. The results indicate abnormalities of the brainstem auditory pathways in patients with spinocerebellar ataxias and show that the test is of value in the differential diagnosis of this group of disorders.

Neuropathy associated with Brescia-Cimino arteriovenous fistulas.

We performed a clinical and electrophysiologic study of median and ulnar nerve function to determine the frequency of neuropathy in 21 patients who had unilateral Brescia-Cimino arteriovenous fistulas and who were undergoing maintenance hemodialysis. Seven patients had symptomatic median or ulnar neuropathy in the arm with the fistula, and abnormalities of motor and/or sensory nerve conduction were found in all of these patients. Of the 14 asymptomatic patients, nine had electrophysiologic evidence of median and/or ulnar neuropathy in the arm with the fistula. Evidence of subclinical median or ulnar neuropathy was also found in the contralateral extremity in 11 of the 21 subjects. Statistically significant differences were found for median and ulnar sensory nerve action potential latencies and motor conduction velocities and for the median distal motor latency between the arms with and without fistulas in the group as a whole, and the mean interarm differences for these values were statistically significant.

Cerebrospinal-fluid abnormalities associated with migraine.

Cerebrospinal-fluid (CSF) abnormalities have been reported infrequently in patients with migraine. Four cases of CSF pleocytosis and raised pressure found in young women during the course of an attack of protracted prodromal migraine, which was followed by complete recovery, are reported. In the absence of any other detectable cause, the CSF changes were attributed to the effects of the migrainous process on meningeal vascular permeability.

Guillain-Barré syndrome and pemphigus foliaceus associated with D-penicillamine therapy.

The Guillain-Barré syndrome and pemphigus foliaceus occurred simultaneously in a patient on long-term treatment with D-penicillamine. It is proposed that both conditions may have developed as a result of a disturbance of immunoregulation caused by D-penicillamine.

Neuroleptic malignant syndrome.

Three cases of the neuroleptic malignant syndrome, which complicated treatment with haloperidol, fluphenazine and, in one case, with several drugs, are described. All three patients recovered and, in two, levodopa appeared to modify the course of the condition. In one patient, myoclonus was present and has persisted. The pharmacological mechanisms involved in this syndrome are discussed.

Brainstem auditory evoked responses and quantitative saccade studies in multiple sclerosis: a comparative evaluation.

Brainstem auditory evoked responses (BAERs) and quantitative saccadic eye movement studies provide information on the integrity of pathways traversing the brainstem. To compare the relative yield of abnormalities with the two techniques, the findings in a group of 46 patients with clinically definite, probable or suspected multiple sclerosis, in whom both procedures were performed, were analysed. BAERs were abnormal in 18 cases (39%), the abnormalities being subclinical in 10 (22%). One or more saccade parameters (reaction time, velocity, accuracy) were abnormal in 31 cases (67%), the abnormalities being subclinical in 19 (41%). When the results of both procedures were taken into account, the overall incidence of abnormalities was 76% and the overall incidence of subclinical abnormalities was 52%. Abnormalities were found with both techniques in 15 cases and with neither technique in 12 cases. Sixteen cases with normal BAERs had an abnormal saccade study, but only three cases with a normal saccade study had abnormal BAERs. It is concluded that BAERs and quantitative saccade studies have a complementary role in the investigation of patients with multiple sclerosis.

Relapse of herpes simplex encephalitis after acyclovir therapy.

Acyclovir is a new and well tolerated anti-viral agent whose role in the treatment of herpes simplex encephalitis (HSE) is still being evaluated. Although relapse of adenosine arabinoside treated HSE has been documented, this paper describes the recurrence of encephalitis after treatment with the recommended course of Acyclovir. A more extended course of Acyclovir produced a rapid and sustained improvement with minimal residual neurological deficit. We emphasise the need for more prolonged treatment and an awareness of the possibility of relapse when using Acyclovir for HSE.