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Biomedical subjects

C D Lambert

Publications and source records attributed to C D Lambert.

14 recordsLinked to original sources
PubMed1992

Stiff-person syndrome.

The stiff-person syndrome is a disorder of persistent, painful muscle contractions predominately affecting the axial musculature. We describe a patient with this disorder and review its pathophysiology. Molecular biologic and immunologic techniques have recently added to the understanding of the mechanism of this disorder. Association with diseases such as diabetes, vitiligo and hypothyroidism have strengthened the auto-immune nature of this syndrome. Auto-antibodies against glutamic acid decarboxylase (GAD), an intraneuronal enzyme, have been implicated in the etiology of this unique disease. Therapeutic intervention with agents such as benzodiazepines that modify central GABAergic activity have demonstrated significant benefit in patients with stiff-person syndrome.

Female↗
PubMed1984

Visual loss secondary to a giant aneurysm in a patient with tuberous sclerosis.

We report a fifty three year old man who presented with progressive visual loss. Investigation disclosed a giant aneurysm with optic nerve compression, which was successfully treated surgically. The patient had typical skin lesions of tuberous sclerosis, but did not suffer from a seizure disorder or mental retardation. In the past he had had an enucleation for glaucoma secondary to a retinal phakoma. Cerebral aneurysms are discussed in relation to the tuberous sclerosis disease process. After review of the literature, we propose that the aneurysms are secondary to dysplastic change in the vessel walls, this is in keeping with the abnormal tissue proliferations in this disease.

Carotid Artery Diseases↗
PubMed1978

Unusual presentation of traumatic extracranial internal carotid artery aneurysm.

A young man presented with apparent transient ischemic attacks following a motorcycle accident in which he sustained minor injuries only. Computerized axial tomography demonstrated a small right frontal infarct, and angiography revealed an aneurysm of the right internal carotid artery in its extracranial course. This was thought to be traumatic in origin.

Adult↗
PubMed1977

Skeletal muscle pathology in chronic heart block.

Skeletal muscle samples, mainly from the deltoid, were studied morphologically and histochemically in 35 patients with chronic heart block and from nine elderly control subject. The average age of the first group was 67-7 (range 11-94) years. Abnormalities were present in 20 cases, no difference being found between patients with idiopathic and secondary heart block. In 15 samples there was increased oxidative enzyme activity in some muscle fibres, and in six there was fibre type grouping. Six had unexplained type 11 fibre atrophy and two had a predominance of type 1 fibres. Tubular aggregates were conspicuous in one biopsy specimen, and in another, rod bodies were found. Minor abnormalities were also seen in the control group. In the absence of any consistent pattern many of these changes were attributed to ageing.

Adolescent↗
PubMed1976

Systemic embolism in chronic sinoatrial disorder.

We attempted to estimate the prevalence of systemic embolism in patients with chronic sino-atrial disorder. In a group of 100 patients, evidence of embolism was found in 16, of whom 15 had the bradycardia-tachycardia syndrome. In 712 controls with chronic complete heart block, who were matched for age and sex, embolism had occurred in only 1.3 per cent (P less than 0.001). A second group of 41 patients with chronic ventricular bradycardia and atrial flutter or fibrillation had an embolic prevalence of 7.3 per cent, which was also greater than that in the controls (P less than 0.05). All patients with sinoatrial disorder in whom systemic embolism developed were over 54 years of age; multiple episodes occurred in six. The risk of embolization remains even if the bradycardia-tachycardia syndrome is replaced by stable atrial fibrillation. Impaired atrial function appears to be a key factor in predisposing to intracardiac thrombosis, and paroxysmal supraventricular tachycardia increases the risk of subsequent embolization.

Age Factors↗
PubMed1976

Neurological associations of chronic heart block.

A large group of patients with chronic heart block was studied for evidence of neurological disorder. Six out of 892 patients were found who had neuromuscular disease related to the conduction disturbance. In four patients, cardiac involvement appeared selectively to affect the conducting tissues. Three of these patients had a scapuloperoneal syndrome, the fourth, the oculocraniosomatic syndrome. In the remaining two patients, one with limb girdle dystrophy and the other with dystrophia myotonica, cardiomyopathy was present in addition to the conduction disturbance.

Adolescent↗
PubMed1976

Neurological aspects of sinoatrial heart block.

The symptoms of 100 patients with chronic cardiac sinoatrial disorder were analysed. The most common presenting features were syncope in 34 cases and dizziness in 22 cases. Over three-quarters of the patients had cerebral ischaemic symptoms at some stage of the disease. Diagnostic difficulties are often encountered and are illustrated by two case histories. Although sinoatrial disorder has been described in association with neuromuscular diseases, only one such example was found in this series. The patient had a limb girdle dystrophy with cardiomyopathy and diffuse disease of the cardiac conducting system. Muscle biopsy samples taken from 11 patients with idiopathic sinoatrial disorder were normal showing no evidence of subclinical muscular disease.

Adolescent↗
PubMed1975

Familial late onset heart block: a clinico-pathological study.

A family is reported in which complete heart block developed late in life, apparently inherited as an autosomal dominant trait. Two octogenarian brothers had symptomatic complete heart block, and their younger relations asymptomatic conduction abnormalities. Neurological disease was present in one proband; investigations suggested that this was a fortuitous association.

Age Factors↗
PubMed1974

Microglioma with paraproteinaemia.

A case is reported in which a solitary intracerebral tumour of lymphoreticular cells (microglioma, reticulum cell sarcoma) was associated with the presence of a serum paraprotein. Extensive investigation failed to show any evidence of extracerebral reticulosis. Response of the paraprotein to treatment of the tumour is described.

Aged↗