Aphemia after a penetrating brain wound: a case study.

The speech of a patient with aphemia (pure anarthria) resulting from a penetrating brain wound was studied using linguistic and acoustic observations as well as electromyographic recordings from four labial muscles. The results are discussed in relation to phonetic disintegration's syndrome and apraxia of speech which, respectively, enhance linguistic disorders and motor programming disturbance.

[Memory disorders following rupture of anterior communicating artery aneurysm. 22 cases]. / Troubles mnésiques après rupture d'anévrysme de l'artère communicante antérieure. 22 cas.

Twenty-two patients who underwent surgery for ruptured aneurysm of the anterior communicating artery were investigated by a battery of neuropsychological tests, including memory evaluation tests. According to the results obtained at these tests the patients were divided into three groups: 1) 7 patients had no memory disorders; 2) 10 patients presented with memory disorders affecting memory; their complaints concerned behavior, and the memory deficit was discovered at neuropsychological examination; 3) 5 patients presented with an obvious amnesic syndrome, and the tests confirmed a severe learning deficit and a severe forgetting while recognition remained normal in 4 patients. Age, residual lesions at CT and electrophysiological signs of focalization correlated well with the residual neuropsychological disorders. Leaving aside quantitative aspects, the memory deficits observed in the second and third groups seemed to be qualitatively different and might correspond to different anatomical and functional sequelae.

[Magnetic resonance imaging in horizontal oculomotor paralysis caused by infarction]. / L'imagerie par résonance magnétique dans les paralysies de l'oculomotricité horizontale par infarctus.

Four patients with focal brainstem ischemic strokes and various types of horizontal oculomotor disturbances have been studied clinically and radiologically. One had a six nerve palsy, one a unilateral internuclear ophthalmoplegia, one a Fisher's one-and-a-half syndrome, and one a paramedian pontine reticular formation syndrome with a sixth nerve palsy. In all patients a C.T. Scan and a Magnetic Resonance Imaging (MRI) were obtained. The MRI study was performed on a CGR Magniscan 5000 with a superconducting magnet of 0.5 Tesla. In all patients a hypersignal in T2 weighted images was shown and corresponded to the brainstem infarct. MRI allowed accurate delineation of the lesion and clinico-radiologic correlations in three patients. But the low specificity of MRI does not permit to distinguish edema from necrosis, gliosis or demyelination in a region with a pathological MRI signal. In one patient this low specificity and perhaps partial volume effects decreased the accuracy of the clinico-topographic correlation.

[Cutaneous lesion associated with multiple endocrine neoplasms type 2A (Sipple's syndrome). An early clinical marker]. / Atteinte cutanée associée à la néoplasie endocrinienne multiple de type 2A (syndrome de Sipple). Un marqueur clinique précoce.

We report the association of a cutaneous lesion with multiple endocrine neoplasia type 2A (MEN 2A) in three patients from a French family. These lesions are very similar to those previously described in an Italian and an American MEN 2A family and called cutaneous lichen amyloidosis. In all three families the patients presented with a pruritic and pigmented cutaneous lesion localized unilaterally on the upper back. However, in the French family the patients also complained of paroxysmal pain in the same area, in which we could elicit a touch hypoesthesia and pain hyperesthesia. Such an association of cutaneous and neurological features in the upper back is known as Notalgia Paresthetica (NP). NP is believed to represent a neuropathy of the posterior dorsal nerve rami. Unlike the two previously reported families, the histological, immunohistochemical and ultrastructural analysis of the skin biopsies of the French patients did not show any amyloid material. This suggests that the presence of amyloid may not be a constant feature of the cutaneous lesions associated with MEN 2A. We consider these lesions as a form of dorsal neuropathy rather than a cutaneous lichen amyloidosis. Whatever their origin, these cutaneous lesion usually precede the appearance of the neoplastic lesions of MEN 2A. They may act as an early clinical marker that must be searched for in each subject at risk for MEN 2A. In addition, all patients presenting with NP should be screened for MEN 2A.

Cutaneous lesion associated with multiple endocrine neoplasia type 2A: lichen amyloidosis or notalgia paresthetica?

Three patients of a French family demonstrated an association of multiple endocrine neoplasia type 2A (MEN 2A) with a pruritic scapular skin lesion. The lesions are similar to those described as familial cutaneous lichen amyloidosis in unrelated MEN 2A and medullary thyroid carcinoma families, but histological, immunohistochemical, and ultrastructural analysis of skin biopsies from each patient in the French family did not show amyloid deposition. The topography of the lesion follows dermatomes C8-D3. The patients report not only pruritus but also paresthesia and hyperalgesia, and one showed touch hypoesthesia and pain hyperesthesia in the area of the lesion. Such an association of cutaneous and neurological features suggests notalgia paresthetica (NP), a neuropathy of the posterior dorsal rami nerves. We thus suggest that the cutaneous lesions associated with MEN 2A might be secondary to pathology in the neural crest-derived dorsal sensory nerves. The amyloid, when present, would be secondary to scratching. We propose that patients presenting with familial NP be suspect for MEN 2A.