[Mixed connective tissue disease (Sharp syndrome) with involvement of the trigeminal nerve and facial paralysis]. / Connectivite mixte (syndrome de Sharp) avec atteinte trijéminée et paralysie faciale.

The authors report a case of mixed connective tissue disease with involvement of the fifth and seventh cranial nerves. While involvement of the trigeminal nerve is well-known, that of the seventh nerve is much less so. However, seventh nerve involvement is beginning to be recognized in other connective tissue diseases such as scleroderma. Neurological manifestations in Sharp syndrome are reviewed.

[Hereditary degenerative spinocerebellar diseases in Tunisia with manometric studies in bladder disorders (author's transl)]. / Les hérédodégénérescences spino-cérébelleuses en Tunisie, avec étude cystomanométrique des troubles sphinctériens. 204 cas de maladies de Friedreich, de Pierre marie et de Strumpell-Lorrain.

During a survey conducted in Tunisia in 1978, 204 cases of hereditary degenerative spinocerebellar diseases were discovered among members of 117 families. The cases included 109 patients with Friedreich's ataxia, 28 with Piere Marie's heredo-ataxia, 20 with Strumpell-Lorrain's disease, and 47 with intermediary forms. The latter group included incomplete forms of Friedreich's and Pierre Marie's diseases. The onset or progression of the disease was linked to a febrile episode in 25 p. cent of the cases. Emphasis is placed on the presence of bladder sphincter disorders in approximately one third of the patients with Friedreich's or Pierre Marie's diseases. Manometric studies in 17 cases demonstrated the presence of normal bladders in 4 cases, hypertonicity of the bladder in 5 patients, and hypesthesic retention-type bladders in 5 other cases. In 3 patients the disorder was difficult to classify. These results show that sphincter disorders should not constitute a criterium for exclusion of the diagnosis of spinocerebellar degeneration.

[Congenital and sporadic atrophy of the granular layer of the cerebellum with secondary pontocerebellar degeneration (author's transl)]. / Atrophie congénitale et sporadique de la couche des grains du cervelet avec dégénérescence ponto-cérébelleuse secondaire.

The primary interest of the case reported here of congenital atrophy of the granular layer of the cerebellum (Norman type) lies in the presence of an associated pontocerebellar degeneration, interpreted to be secondary (transsynaptic and retrograde) to the granular involvement and due to the length (42 years) of the evolution. Secondly, the sporadic character of this case is not in favor of a genetic disorder. By analogy with Herringham and Andrewes cat ataxia due to a perinatal virus infection (Margolis and Kilham), it can be hypothesized that granular cerebellar atrophy in man is related to an injury incurred at a "favorable" moment in histogenesis, that is, between the 3rd and 6th month of fetal life.

Olivopontocerebellar atrophy with velopharyngolaryngeal paralysis: a contribution to the somatotopy of the nucleus ambiguus.

The authors report a case of olivopontocerebellar atrophy (OPCA) with velopharyngolaryngeal paralysis. The cerebellar syndrome appeared in a 66 year-old woman and ran its course until her death at 75. The velopharyngolaryngeal paralysis occured two years after the beginning of the cerebellar symtomatology and was limited for 6 months to a Gerhardt syndrome. Postmortem examination showed typical lesions of OPCA, and on serial sections of the medulla a massive loss of neurons in the lower two thirds of the nucleus ambiguus, bilaterally. The association of OPCA with velopharyngolaryngeal paralysis is exceptional. The anatomical findings in this case contribute to the somatotopy of the nucleus ambiguus in man by demonstrating the location of the velopharyngolaryngeal centers in this formation. The upper third plays only an accessory role in the velopharyngolaryngeal functions, and in the two lower thirds one finds, from the oral to the caudal extremity, first the velopharyngeal, then the laryngeal centers.

[Paralysis of the 3d cranial nerves in diabetes and common oculomotor vascularization]. / Paralysies de la IIIe paire au cours du diabete et vascularisation du moteur oculaire commun.

The purpose of this paper is to present explanations for various patterns of IIIrd cranial nerve involvement in diabetes mellitsus, based on its vascularisation. Three clinical cases of diabetes with numerous attacks of IIIrd nerve paralysis are reported. The following patterns were observed: (1) isolated; (2) associated with Vth nerve involvement and; (3) associated with invovlement of the Vth, IVth, VIth, and occasionally IInd and VIIth nerves. An anatomical study of IIIrd nerve vascularisation demonstrates three territories which could correspond to the 3 patterns of clinical expression. The arterial branches to the IIIrd nerve give off no collaterals in the posterior region of the circle of Willis. In the supra-cavernous region, vascularisation of the IIIrd nerve may be associated with that of the IVth. Eventually, vascularisation of the IIIrd nerve in the intracavernous region is associated with that of the Vth, IVth, VIth, and occasionally IInd and VIIth cranial nerves. Thus, a painless paralysis of the IIIrd nerve (isolated or associated only with an involvement of the IVth) would predictably be related to a vascular disturbance limited to the first or second portion of this nerve, whereas a painful paralysis of the IIIrd nerve, without or with associated involvement of other cranial nerves, would relate to a vascular distrubance in the intra-cavernous region. The anatomo-clinical relationships that have been presented: (1) support the vascular basis of IIIrd nerve paralysis in diabetes; (2) explain the various clinical patterns of IIIrd nerve involvement in that disorder and; (3) act as a model which can be applied to the study of ischemic pathology in other cranial nerves and other etiologies.