Granulomatous angiitis of the central nervous system. Case report.
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Adult↗
Biomedical subjects
M E Alexianu
Publications and source records attributed to M E Alexianu.
Particular neurological aspects in vascular autoimmune diseases. II. Cryoglobulinemic vasculitis and polyarteritis nodosa.
In this second part we present our clinical neurological observations in the other 8 cases: 5 presenting systemic vasculitis of cryoglobulinemic etiology, and 3 with polyarteritis nodosa with central and peripheral neurological manifestations.
Adult↗
Particular neurological aspects in vascular autoimmune diseases. I. Rheumatoid purpura, Stevens-Johnson syndrome, rheumatoid arthritis and systemic lupus erythematosus.
This study presents particular clinical manifestations in 7 patients with autoiMmune diseases: rheumatoid purpura with right crural nerve paresis (1 case), Stevens-Johnson syndrome with encephalomyeloradicular syndrome (1 case) and left Wallenberg syndrome (1 case), rheumatoid arthritis with right parieto-occipital syndrome (1 case) and Gowers local panatrophy (1 case), systemic lupus erythematosus with confusional state and meningeal syndrome (1 case) and left ictal hemiplegia (1 case). The importance of neurological clinical manifestations at the onset or during the evolution of the autoimmune diseases is emphasized.
Adult↗
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↗
The role of immune processes in amyotrophic lateral sclerosis pathogenesis.
Despite many efforts, the etiopathogenesis of ALS remains unknown. During the last decade evidence for an autoimmune involvement in motoneuron degeneration and death has remarkably increased. Multiple reports have documented significant expression of proteins associated with immune function in affected areas of ALS patients. Two animal models of immune-mediated motoneuron destruction have been developed that closely resemble clinical, electrophysiological and morphological features of human ALS. Inflammatory foci within the spinal cord, and IgG at the neuromuscular junction as well as within upper and lower motoneurons found in the animal models support the role of autoimmune mechanisms of motoneuron destruction in this model. IgG from ALS patients and from the animal models can passively transfer physiological changes at the neuromuscular junction in mice. That ALS IgG interact with calcium channels and induce an alteration of their function is now electrophysiologically and biochemically evident. Furthermore, it has been documented that motoneurons may be selectively vulnerable since they have a deficient calcium buffering capacity. Although further research efforts are necessary to elucidate the interaction of the ALS antibodies with the calcium channel function and how defective calcium handling by the motoneurons is important in their degeneration, the current data strongly suggest the involvement of autoimmune mechanisms in ALS etiopathogenesis.
Amyotrophic Lateral Sclerosis↗
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↗