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Biomedical subjects

A Dan

Publications and source records attributed to A Dan.

41 records · Page 3Linked to original sources

Chronic sensorimotor polyneuropathy associated with cerebello-extrapyramidal manifestations in a patient with tomaculous neuropathy.

Clinical, electrophysiological and morphological (sural nerve and gastrocnemius muscle biopsies) data of a 57-year-old man with a chronic sensorimotor polyneuropathy of Charcot-Marie-Tooth type associated with a progressive cerebello-extrapyramidal syndrome are reported. Patient's family data were negative. Nerve structural and ultrastructural examinations revealed the morphological picture of a tomaculous neuropathy. The association of different clinical syndromes and the specificity of the tomaculous neuropathy are discussed.

Basal Ganglia Diseases↗

Degenerative neuromuscular diseases with a late onset. Clinical, electrophysiological and morphological study.

This study carried out clinical, electrophysiological and morphological investigations (sural nerve and gastrocnemius muscle biopsies) in a group of 47 patients with neuromuscular disease of a certain or supposed degenerative origin and a late onset (over the age of 30 yrs.). It aimed the evidence of the eventual particularities of such diseases with a delayed onset. The equal involvement of sexes, the insidious onset, the clinical picture similar to that of the corresponding diseases with an onset at the usual age were observed. Regardless of the age, some interesting associations of the polyneuropathy with other diseases or its presence with in these diseases (Parkinsonism, Addison's disease, multiple symmetrical lipomatosis, etc.) were noticed, too. Electrophysiological examinations showed no particularities. Neither did the muscular morphological picture in most of the cases presenting neurogenic lesions with a moderate intensity. The sural nerve biopsy evidenced in 70% of the cases a moderately intense neuropathy of an axonal type ("dying back"), with or without secondary lesions of segmental demyelination and with the signs of a live regenerative activity.

Adult↗

Tomaculous neuropathy with unusual clinical aspects.

Tomaculous neuropathy represents the morphological substrate of the recurrent familial neuropathy with liability to pressure palsies. Some ultrastructural changes characterizing the tomaculous neuropathy can occur as incidental aspects in other different neuropathies. Few tomaculous neuropathy cases with clinical aspect of chronic polyneuropathy without paretic episodes have been mentioned in the literature. In the present work, we report four cases who offered the morphological surprise of a true tomaculous neuropathy with 15-37% of the teased fibres bearing tomaculae sized: 55-106 microns/20-23 microns, on the background of a demyelinating neuropathy with 25-56% of the teased fibres showing segmental de- or remyelination. The clinical and electrophysiological diagnoses of these 4 patients were: HSMN type I (2 cases), HSMN type VIII (polyneuropathy associated with a cerebello-extrapyramidal syndrome -1 case), and a neurogenic scapuloperoneal syndrome (1 case). The specificity of the tomaculous neuropathy is discussed.

Adolescent↗