Acute cerebellar syndrome complicating infectious mononucleosis.
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Biomedical subjects
Publications and source records attributed to G J Hankey.
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A woman with herpes simplex encephalitis (HSE) in the third trimester of pregnancy is described. She was treated with acyclovir and recovered completely to deliver a normal child per vaginam at term. She had no evidence of genital or disseminated herpes virus infection. This paper illustrates that (1) the outcome of HSE in pregnancy can be favorable both for the mother and the offspring, (2) early diagnosis and use of acyclovir therapy is essential for successful outcome, and (3) the use of acyclovir in the third trimester of pregnancy was not harmful to the mother or fetus.
The Landau-Kleffner syndrome is an unusual childhood disorder characterised by an acquired aphasia and a paroxysmal electroencephalographic abnormality with spike and spike and wave discharges which are mostly multifocal and unstable in their course of evolution. Two accessory symptoms are frequently observed: psychomotor or behavioural disturbances and epilepsy. Epileptic seizures are most often generalised convulsive or partial motor and invariably disappear before the age of 15 years. A 20 year old woman is described who has been followed since the rarely observed onset of the Landau-Kleffner syndrome at the age of 3 years. At 4 years of age she was totally unable to communicate through speech and her EEG showed almost continuous bitemporal spike and spike and wave discharges. Maximum doses of diazepam, phenytoin, carbamazepine and sulthiame were used without effect. From the age of 14 years her ability to communicate through spoken and written language has improved and her EEG now shows no epileptiform activity.
Despite the advance represented by nuclear magnetic resonance imaging, the diagnosis of multiple sclerosis (MS) remains clinical. It has been our impression that a diagnosis of MS reduces further critical neurological thought and assumes an air of certainty after a number of visits in the absence of any new evidence for the diagnosis. One of us (E.G.S-W.), as part of a nationwide epidemiological study, reviewed 387 patients in Western Australia who had been diagnosed as having MS. Three diagnostic sub-groups were identified: group A--318 patients considered to have MS, group B--32 patients with an alternative chronic neurological disorder, and group C--35 patients not considered to have a neurological illness. Patients in group C were predominantly females (89%) and nurses (34%) with an earlier age of onset of symptoms (mean 28 years, range 12-46 years). Frequent clinical features included monocular diplopia, tunnel vision, 'give-way' weakness and hemisensory loss of all modalities of sensation. The major clinical features of patients in group B, which were atypical for MS, included onset of non-remitting, progressive disease before age 35 years, localised disease, and normal optic nerves and eye movements. The results of this study serve to promote critical analysis of the accuracy of diagnosis of MS in individual cases.
Three parkinsonian patients are described who independently discovered that their gait was facilitated by inverting a walking stick and using the handle, carried a few inches from the ground, as a visual cue or target to step over and initiate walking. It is suggested that the "inverted" walking stick have wider application in patients with Parkinson's disease as an aid to walking, particularly if they have difficulty with step initiation and maintenance of stride length.
A one year prospective study was undertaken to evaluate the usage of the cranial CT scan in private neurological practice. The impetus for the study emanated from a general impression that patterns of referral to neurologists were changing with regard to the nature of the patients' condition and that a large number of patients had already had a cranial CT scan before neurological consultation. A total of 826 cases were reviewed. Sixty (7%) had had a recent cranial CT scan before consultation, and 90% of these cases were referred by the patients' general practitioners. A provisional diagnosis was attempted by the GP in 36% of cases, and 50% of these were correct. Ninety-five per cent of the CT scans were normal. Eighty-three (10%) patients were referred for cranial CT scan after neurological consultation. The neurologists' reasons for CT scanning included investigation of epilepsy (20%) and exclusion of a structural cerebral hemisphere lesion (16%), acoustic neuroma (10%) and other posterior fossa lesions (16%). Ninety-one per cent of these CT scans were normal. In all, 143 (17%) patients underwent cranial CT scanning; of these almost half (42%) had been referred for the CT scan by the general practitioner before neurological consultation. This study contrasts the CT scan referring patterns of general practitioners with that of a neurologist and questions the possible overuse of this facility.
A 70 year old man recovered from a left putamenal haemorrhage in 1984 with a residual mild right hemiparesis. In 1985 he presented following the sudden onset of inability to stand. The clinical findings of supranuclear ophthalmoplegia, bradykinesia and rigidity resembled those of progressive supranuclear palsy. CT scan revealed a recent haemorrhage deep in the right hemisphere white matter in addition to a low density change in the left basal ganglia reflecting the site of previous haemorrhage. The patient's course was uncomplicated and the ophthalmoplegia resolved. Bradykinesia and rigidity persist. This case illustrates an unusual clinical presentation of bilateral intracerebral haemorrhage with supranuclear ophthalmoplegia, bradykinesia and rigidity.
Certain clinical features are known to correlate with patient outcome in acute stroke, but the potential of cranial CT scan as an aid to predicting recovery after stroke remains unclear. In a prospective study, 178 acute stroke patients admitted to Royal Perth Hospital were assessed by 4 neurologists; 150 cranial CT scans were performed and these were reviewed by an independent radiologist. CT lesion characteristics were cross-tabulated against clinical indices of patient outcome. Lesions greater than 50 cm3 in volume were associated with a 23% mortality compared to a 5% mortality for lesions smaller than 50 cm3. The mortality rate from intracerebral haemorrhage was 25% compared to 9% for other disease. Of patients whose CT scan showed a mass effect, 35% had an altered mental state at discharge compared to only 2% of those without a mass effect. Of the patients with deep white matter lesions, 35% had an altered mental state at discharge compared to only 13% of patients with lesions in other sites, and only 50% were discharged home compared to 77% of patients with lesions in other sites. CT scan features that correlated significantly with a poor outcome following acute stroke were large lesion size, intracerebral haemorrhage, mass effect and deep white matter lesions.
The first reported case of disseminated histoplasmosis in Western Australia is described. The diagnosis was delayed because the clinical presentation closely resembled that of tuberculosis, the initial histoplasma serology was negative, and constant severe thrombocytopenia delayed efforts to obtain tissue required for culture and histology. Despite adequate therapy, relapse occurred on one occasion.
Carotid endarterectomy is currently indicated for patients with severe symptomatic carotid stenosis because it halves the risk of stroke. However, it is expensive and potentially risky, and is performed unnecessarily in most of these patients. In order to improve the cost effectiveness of carotid endarterectomy, other predictors of stroke, in addition to the presence of recent focal neurological symptoms and the degree of carotid stenosis, need to be identified. Further research into the nature of the carotid atherosclerotic plaque and the clustering of systemic factors (infectious, inflammatory and vascular risk factors) that trigger inflammatory and morphological changes in the asymptomatic plaque and predispose it to rupture (with subsequent symptomatic thromboembolism) may yield powerful predictors of stroke which, when combined with the degree of stenosis and presence of focal neurological symptoms, may improve patient selection for carotid endarterectomy and its cost effectiveness.
We prospectively examined 128 patients with acute first-ever stroke to determine the prevalence of swallowing disorders, the diagnostic accuracy of our clinical assessment of swallowing function compared with videofluoroscopy, and interobserver agreement for the clinical and videofluoroscopic diagnosis of swallowing disorders and aspiration. We found clinical and videofluoroscopic evidence of a swallowing disorder in 51% [95% confidence interval (CI) 42-60%] and 64% (95% CI 55-72%) of patients, respectively, and aspiration in 49% (95% CI 40-58%) and 22% (95% CI 15-29%) of patients, respectively. The optimal clinical criteria for detecting videofluoroscopic evidence of a swallowing disorder and aspiration were any clinical evidence of a swallowing disorder (sensitivity 73%, 95% CI 62-82%; specificity 89%, 95% CI 76-96%), and any clinical evidence of aspiration (sensitivity 93%, 95% CI 76-99%; specificity 63%, 95% CI 53-72%). The interobserver agreement between two speech pathologists for the clinical diagnosis of a swallowing disorder (kappa: 0.82 +/- 0.09) and aspiration (kappa: 0.75 +/- 0.09) was good, and between a speech pathologist and radiologist for the videofluoroscopic diagnosis of a swallowing disorder (kappa: 0.75 +/- 0.09) and aspiration (kappa: 0.41 +/- 0.09), it was good and fair, respectively. Although clinical bedside examination underestimates the frequency of swallowing abnormalities and overestimates the frequency of aspiration compared with videofluoroscopy, it may still offer valuable information for the diagnosis of swallowing impairment. Long-term follow-up studies are required to determine the independent functional significance of the findings of the bedside and videofluoroscopic examinations in predicting the occurrence of important outcome events such as aspiration pneumonia.
For more than 20 years, moderately raised concentrations of total homocysteine have been associated with an increased risk of atherothrombotic vascular events but only recently has evidence mounted to suggest that the association may be causal. The association is independent of other factors, it is fairly consistent across many studies, it is strong and dose-related, and it is biologically plausible. However, the evidence needs to be strengthened by a systematic review of all comparable studies and the demonstration, in randomised trials, that lowering total homocysteine is followed by a significant reduction in atherothrombotic vascular disease. In addition, the measurement of total homocysteine needs to be standardised. If these can be achieved then total homocysteine measurement will become another useful marker of vascular risk, multivitamin therapy will be another therapeutic option for people at risk of atherothrombotic vascular disease, and fortification of food with folic acid will rise high on the political and public health agenda.