HTLV-I associated myelopathy in a Seychellois immigrant.

OBJECTIVE: To describe the clinical and laboratory features of human T-lymphotropic virus type I (HTLV-I) associated myelopathy in an immigrant from the Seychelles. CLINICAL FEATURES: A slowly progressive myelopathy has been recently diagnosed in a 64-year-old woman who emigrated to Australia from the Seychelles in 1957. Sphincter disturbance and back pain were the first manifestations, followed by gait disturbance. Neurophysiological investigation supported the clinical diagnosis of a myelopathy and radiological investigations revealed no structural cause. Serum antibodies to HTLV-I were detected by enzyme-linked particle agglutination and the presence of antibodies to individual HTLV-I gene products in the serum was confirmed by western blot. The virus was detected in a culture of the patient's peripheral blood mononuclear cells by antigen capture assay and by sequencing a polymerase chain reaction product amplified from the env gene. INTERVENTION AND OUTCOME: The patient was advised of the nature and prognosis of her illness. Oral corticosteroids were tried without benefit. CONCLUSIONS: The prevalence of HTLV-I infection is low in Australia although it may be endemic in some Aboriginal communities. Most infections are asymptomatic but the chronic neurological disease associated with HTLV-I infection has now been shown to exist in this country. HTLV-I infection should be considered in the aetiology of myelopathy without another obvious cause.

Thrombolytic therapy in vertebrobasilar occlusion.

Since 1983 at the Alfred Hospital 4 patients with thrombotic or embolic vertebrobasilar occlusions have been treated with intra-arterial streptokinase (SK) infusions for the effects of persisting brainstem ischaemia despite anticoagulation with heparin. In 3 cases there was immediate and dramatic neurological improvement, in all cases associated with arteriographically demonstrated reperfusion of a blocked vessel. Two of these patients suffered further thromboembolic vertebral or basilar artery occlusions (3 days and 2 years later) but recovered fully without further thrombolytic therapy. The other patient was given intra-arterial SK 12 days after an apparently completed brainstem stroke: the therapy failed to cause reperfusion of a vertebral occlusion or produce any clinical improvement. Complications from the therapy were nausea requiring the termination of the SK infusion in one case, easily controlled bleeding from a recent surgical wound, and a clinically insignificant haemorrhagic transformation of cerebellar infarction in a third. The benefits of thrombolytic therapy in vertebrobasilar ischaemia and the dose of streptokinase required are discussed.

Intraventricular infusion of Dopamine in Parkinson's disease.

A patient with severe end-stage Parkinson's disease and troublesome fluctuations in motor function was treated with a long-term intraventricular infusion of dopamine. There was modest improvement in speech and mentation and there was smoother control of motor symptoms that was superior to that achieved by conventional oral medications.

Obstructive hydrocephalus caused by multiple sclerosis.

A case is reported of obstructive hydrocephalus secondary to the mass effect of a plaque of acute demyelination in the brainstem. The literature on the CT scan appearances in multiple sclerosis is briefly discussed in relation to this case.

Transient ischaemic attacks: controversies in treatment. A review.

The methods available to treat transient ischaemic episodes and to prevent stroke are discussed, with particular reference to the use of aspirin.

Pyridoxine neuropathy.

A case of sensory neuropathy in a young woman due to long-term ingestion of pyridoxine, with subsequent recovery, is described. Pyridoxine neuropathy may occur after the long-term ingestion of doses as low as 200 mg a day. Because of its widespread use in the community, both the general public and the medical community need to be aware of this recently described complication of megavitamin therapy.

Cryptococcal infections of the central nervous system: a ten year experience.

Twenty cases of cryptococcal CNS infection treated at the Alfred and Fairfield Infectious Diseases Hospitals from 1975 to 1985 were reviewed. A predisposing immunological deficit was present in 40% of the cases and nearly half had evidence of pulmonary involvement. Severe headache was an almost universal presenting feature but fever and meningismus were not. Measurement of CSF cryptococcal antigen and CSF culture were far more reliable diagnostic markers than Indian ink smears. Cerebral CT scanning identified abnormalities in nearly 30% of cases, including 2 with cystic lesions and 2 with mass lesions. Combination therapy with amphotericin B and 5-fluorocytosine was used as first line treatment. Ventricular shunts were required for 2 patients with hydrocephalus, and persistently raised intracranial pressure often required frequent lumbar punctures and corticosteroids for control. Mortality was 30% and correlated with the presence of impaired conscious state, hydrocephalus or other neurological deficit, underlying immunodeficiency and low CSF glucose levels.

Neurological sequelae of lightning stroke.

Two cases of patients struck by lightning are described. One instance occurred while the victim was using the telephone; the other victim received a direct lightning strike to the head. Both patients had acoustic shock injuries and one developed a cerebral infarct. The mechanisms of these injuries are discussed and the relevant literature is reviewed.

Paramedian thalamic and midbrain infarction: the 'mesencephalothalamic syndrome'.

Paramedian infarction in the region of the thalamus and upper midbrain may produce a wide range of neuro-ophthalmological, behavioural and motor abnormalities. The paramedian arteries arise from the first part of the posterior cerebral artery, also known as the basilar communicating artery. The particular arterial topography and its anatomical variation may result in unusual combinations of clinical signs, and infarction may be bilateral in some cases. Diagnosis is often aided by CT scanning and magnetic resonance imaging. Both atherosclerotic occlusion and embolism are thought to be responsible for these particular syndromes. We describe 3 cases that illustrate some of the various clinical features and underlying anatomical vascular arrangements which may be seen in this condition. Thalamic dementia was present in one case with evidence of bilateral thalamic infarction. A complex ophthalmoplegia and hemiparesis were seen in another case, and the third case had a combination of thalamic dementia and ophthalmoplegia.

Granulomatous angiitis of the central nervous system.

Granulomatous angiitis of the nervous system is a rare necrotizing vasculitis that is largely confined to the central nervous system. The case of a 43-year-old patient is presented and the clinical presentation, diagnostic difficulties and treatment are discussed. Patients present in their fourth decade with diffuse and focal neurological symptoms and signs. No single test is diagnostic; however, cerebrospinal fluid lymphocytosis and raised protein levels, changes on cerebral angiography, and leptomeningeal biopsy may confirm the diagnosis. Although the administration of corticosteroid agents has prolonged the survival of the patient, a progression of the disease has been the rule. The administration of corticosteroid and cyclophosphamide agents on alternate days may lead to clinical remission of the disease.

Paraneoplastic optic neuritis and external ophthalmoplegia.

A 58 year old man developed bilateral optic neuritis and external ophthalmoplegia nine months before presentation with a small cell carcinoma of the lung. There was no evidence of central nervous system metastases and his ocular symptoms responded to corticosteroid therapy.

Wernicke's encephalopathy after prolonged fasting.

Wernicke's encephalopathy has been associated rarely with fasting. We describe a middle-aged patient who presented in a subacute fashion with features of thiamin deficiency after marked weight reduction. Associated features were chest pain, possibly related to cardiac involvement, and a proximal myopathy. The Wernicke-Korsakoff syndrome has been described in the literature for around 100 years. However, the classic triad of ataxia, ophthalmoplegia and impaired conscious state that occurs in a person with chronic alcoholism is by no means its exclusive presentation.

Availability of drug assay results and dosage advice improves antiepileptic care in a specialist neurology outpatient clinic.

The incidence of grand mal seizures, distribution of drug levels relative to the therapeutic range and the incidence of side effects were monitored before and after the provision of same-day preconsultation assay results (phenytoin and carbamazepine) to an epilepsy clinic. A total of 300 patients completed the study. The proportion of patients fitting fell from 23.3% at entry to 10% at review (P less than 0.01) while the incidence of side effects fell from 30.7% to 17.3% (P less than 0.01) and the proportion of phenytoin levels in the therapeutic range rose from 41% to 58% (P less than 0.01). Similar patterns were observed in 111 patients who entered the study during a control observation period. The provision of appropriate support services (assay results and dosage advice) to specialist epilepsy clinics allows improvement in the management of epilepsy.

Absence status: a report of two cases.

Two known epileptics presented with abnormal behaviour. One was inattentive, aggressive and ataxic. The other carried out quite complex, though purposeless activity. EEG's revealed that the patients' behavioural abnormalities were due to uncontrolled seizures. Absence Status is an uncommon condition and its various manifestations can lead to delays in its recognition and treatment. It should be considered as a possible diagnosis in any patient who presents with recurring episodes of confusion.

Postanoxic action myoclonus (Lance-Adams syndrome) responding to valproate.

A patient with postanoxic action myoclonus (Lance-Adams syndrome) was severely disabled with this movement disorder. Valproate sodium was administered orally, with complete resolution of the myoclonus. This favorable response has been maintained for two years. Excessive yawning, the only side effect encountered, was dose related and was abolished with the addition of pimozide to the drug regimen.

Primary hyperparathyroidism presenting as a proximal myopathy.

A 46-year-old female presented with a three year history of progressive weakness. Asymptomatic apart from proximal myopathy, multi channel screening and radiological features indicated primary hyperparathyroidism with severe metabolic bone disease. Removal of a parathyroid adenoma led to improvement of muscle strength and regression of bony and metabolic changes.