Search

Subcortical arteriosclerotic encephalopathy is a chronic vascular dementia with hydrocephalus characterized clinically by: (i) subacute focal neurological deficit; (ii) acute strokes; (iii) dementia; (iv) motor signs and pseudobulbar palsy; (v) hydrocephalus; (vi) persistent hypertension and systemic vascular disease; and (vii) a lengthy course. The pathogenesis is most probably ischaemic change related to subacute hypertensive encephalopathy. The pathological changes include severe central nervous system disease characterized by loss of white matter with gliosis, and arterial and arteriolar sclerosis of small penetrating cerebral blood vessels. ThediHerential diagnosis includes vascular pseudobulbar palsy, multi-infarct dementia and senile dementia (Alzheimer's disease). Treatment includes blood pressure control as well as management of other factors known to affect vascular disease (diabetes mellitus).

The Eaton-Lambert syndrome is a non-metastatic manifestation of oat-cell carcinoma of the bronchus, although it has been reported in patients with carcinoma at other sites. The clinical picture is usually on~ of subacute muscular fatiguability with weakness and wasting affecting the proximal parts of the limbs and trunk, but occasionally the external ocular and bulbar muscles are involved. The weakness is often myasthenic, but it has been observed that muscle power may in fact increase after brief exercise. The pathogenesis, diagnosis, electrophysiological features and some aspects of therapy are reviewed.

The Tolosa-Hunt syndrome. or painful ophthalmoplegia. is a rare condition caused by a granulomatous nonspecific process at the level of anterior cavernous sinus. superior orbital fissure and orbital apex. The syndrome is characterized by pain behind. above or around the eye. involvement of the cranial nerves which pass through the cavernous sinus, spontaneous remissions and exacerbations. and a favourable response to steroid therapy. Recognition of this condition is important because administration of !?teroids may prevent residual damage.

A 48-year-old hypertensive man with signs and symptoms of pseudobulbar paralysis was Ultimately found to be sLiffering from the operculum syndrome. Recognition of this syndrome is important in that: (a) it may be caused by a localized cortical lesion amenable to treatment; (b) some of the symptoms carry a good prognosis as opposed to those in pseudobulbar paralysis; and (c) the operculum syndrome usually has a-more favourable prognosis since there is no associated mental impairment or sphincter disturbances.

The restless legs syndrome is a condition of unknown aetiology characterized by nocturnal paraesthesiae in the lower limbs,an irreversible tendency to move the limbs, pain in the distribution of the paraesthesiae, and psychiatric disturbances. The syndrome may occur at any age and in either sex and usually runs a course over many years with spontaneous improvements and exacerbations. An association with iron deficiency has been suggested but in most cases no apparent cause has been found, and treatment at present is symptomatic with analgesics and sedatives. The differential diagnosis includes phenothiazine-induced akathisia and meralgia paraesthetica.

Treatment of a 27-year-old Black man with writer's cramp with a combin'ation of sodium valproate (Epilim) and baclofen (Lioresal) resulted in dramatic improvement of symptoms and signs. The possible mechanism of action of these drugs is discussed. This combination should be tried in the initial management of this syndrome.

A case of parkinsonism associated with neurosyphilis, which improved markedly after appropriate treatment, is described, Although neurosyphilis is a rare cause of parkinsonism, it should be considered when parkinsonism appears in the 30-50-year age group, particularly if other anomalous neurological findings are present. Adequate therapy may improve the patient's condition and prevent further progression

In two blood-related Libyan siblings aged 3 and 6 years with psychomotor retardation, attacks, hyperkinetic behaviour and lack of co-ordination of differentiated motor functions, CT scanning revealed a cyst in the pellucid septum and Verga's ventricle, combined with deficient corpus callosum, hydrocephalus and microcephalia.