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We report on a neuropathological analysis of two cases of TSP/HAM originating from Brazil. These two cases had, respectively, an evolution of 13 and 40 years. The main neuropathological findings consisted of spinal cord atrophy, mainly the lower thoracic cord, diffuse degeneration of the white and grey matter, rare foci of mononuclear and perivascular cuffs, and hyaline hardening of arteriolae. The supraspinal structures were normal, excepting for a slight gliosis in the cerebellum. An analysis on the long evolutive cases as described in the literature is outlined in this study. Relatamos a análise neuropatológica de dois casos de PET/MAH originários do Brasil. Estes dois casos tinham, respectivamente, uma evolução de 13 e 40 anos. O principais achados neuropatológicos consistiam de atrofia da medula espinhal, principalmente da medula torácica baixa, degeneração difusa das substâncias branca e cinzenta, raros focos de infiltrado mononuclear e perivascular, e endurecimento hialínico das arteriolae. As estruturas supra-espinhais eram normais, exceto por uma ligeira gliose do cerebelo. Uma análise dos casos de longa evolução descritos na literatura é salientada neste estudo.

The serine proteinase tissue-type plasminogen activator (t-PA) and the metalloproteinase gelatinase B (MMP-9) have recently been demonstrated in MS lesions. Both enzymes are interconnected in an enzyme cascade which contributes to destruction of the blood brain barrier and demyelination and both enzymes are inhibited by D-penicillamine. Metacycline was shown in in vitro experiments to inhibit gelatinase B. The combination of peroral D-penicillamine plus metacycline was evaluated in a double-blind placebo-controlled way in two groups of 10 patients suffering from secondary progressive multiple sclerosis. The major objectives of this pilot trial were to examine the safety of this combination and the possibility of blinding, while the effect on disease progression was considered as a secondary endpoint. Over a follow-up period of 1 year and in this selected patient group, there was no significant improvement in the Expanded Disability Status Scale score (EDSS) as compared with that of the placebo-control group. Toxicity was too high to consider additional trials with this combination of metalloproteinase inhibitors. Although peroral treatment is by most MS patients acknowledged as a major improvement in treatment compliance, one has to await the development of more selective and efficaceous protease inhibitors than those used in the combination therapy described here.

A 21 year old female patient developed Sudeck's atrophy of the right foot secondary to a chronic Achilles tendinitis. The condition was complicated by the occurrence of painful muscle spasms in the right leg and incontinence of urine. The spasms had characteristics of both a tonic ambulatory foot response and a spinal flexor reflex. The movements disappeared during sleep. Regional anaesthesia of the right leg made the spasms disappear both in and outside the region of anaesthesia. Backaveraging of the EEG showed the involuntary spasms to be preceded by a cortical potential similar to a readiness potential, indicating a cortical potential similar to a readiness potential, indicating a cortical component in the pathophysiology of the muscle spasms complicating Sudeck's atrophy.