[Sensory trigeminal neuropathy in systemic diseases: 4 cases with study of the trigeminofacial reflex]. / Les neuropathies trigéminales sensitives des maladies systémiques: 4 cas avec étude du réflexe trigémino-facial.

Isolated trigeminal nerve affections can occur during the course of various connective tissue diseases, particularly scleroderma and mixed connective tissue lesions. Four cases are reported: a patient with systemic scleroderma, one with atrophic polychondritis, one with Gougerot-Sjögren's disease and one with atypical and frustes connective tissue lesions. The mechanisms of onset and lesional location in these neuropathies are poorly understood. A blink reflex study by electrical stimulation of the supraorbital nerve was carried out in these 4 patients to determine the site of lesions. Response was normal in 1 case suggesting a lesion of a distinct branch of the supraorbital nerve. In 2 cases the anomalies of the early response were strongly suggestive of a peripheral, truncal or radicular lesion. In the last patient the early response was normal and latencies in tardive responses of the stimulated side were in favor of a central lesion of the spinal root or spinal nucleus of the trigeminal nerve. Clinical characteristics of some reported cases of neuropathy of trigeminal nerve also appear to point to a central lesion.

[Familial thenar amyotrophy of carpal origin]. / Amyotrophies thénariennes familiales d'origine carpienne.

Thenar amyotrophy of carpal origin was found in two sisters aged respectively 49 and 59 years and in a 75 year-old woman and her 56 year-old daughter. The literature contains reports on about 15 cases of familial carpal tunnel syndrome. The clinical features were sensory symptoms in most patients but there was also cases with amyotrophy. The coexistence in a same family of several cases of carpal tunnel syndrome is not by itself, evidence of a genetic factor.

[Neuroendocrine syndrome: pseudomyopathic spinal amyotrophy with gynecomastia related to the X chromosome. Kennedy's syndrome. 3 cases]. / Un syndrome neuro-endocrinien: l'amyotrophie spinale pseudo-myopathique avec gynécomastie liée au chromosome X. Syndrome de Kennedy. Trois observations.

Three new cases of X chromosome-linked spinal muscular atrophy associated with gynaecomastia are reported. They were concordant with the description given in about 15 published reports: predominantly proximal muscle weakness and atrophy, fasciculations in the face and tongue, areflexia and slowly progressive course. In two of our patients nerve biopsy showed axonal lesions. All these patients had gynaecomastia. The relationship between the neurological and endocrine syndromes is discussed, but no firm conclusion can yet be drawn.