[Aphasia caused by isolated lesion of the semi-ovale centre: contribution of the measurement of cerebral blood flow]. / Aphasie par lésion isolée du centre semi-ovale: approt de la mesure du débit sanguin cérébral.

Sudden onset stroke occurred in a right-handed vietnamese woman speaking, reading and writing french fluently. When first seen in our department, the patient had mild right facial paresis and non fluent atypical aphasia. CT scan and MRI showed a left subcortical infarct in the superficial territory of the middle cerebral artery; only white matter of the semiovale centre was involved. Neurological examination revealed linguistic impairment resembling transcortical motor aphasia, with unusual stuttering, hypophonia, occasional semantic paraphasias and phonological reading and writing abnormalities. Non verbal cognitive function, gestural and buccofacial praxes were normal. Cerebral blood flow study by SPECT was consistent with left sylvian functional deactivation.

[Congenital syphilitic meningomyelitis manifested in the adult]. / Méningo-myélite syphilitique congénitale révélée à l'âge adulte.

We report a case of neurosyphilis of the spinal cord in a 38-year-old-man presenting with urinary incontinence and Argyll-Robertson sign. Anamnesis revealed the congenital origin of the disease. Urodynamics findings were consistent with a conus medullaris involvement and CSF study revealed a specific lymphocytic meningitis. A specific spinal arteritis was found by angiography. A quarterly 15 to 20 days course of intravenous penicillin was then instituted during 2 years. A specific treatment of urinary symptoms resulted in complete disappearance of sphincter dyssynergia. Antibiotic therapy was followed by clinical improvement and regression of most of the CSF abnormalities.

[Perineal neuralgia]. / Les névralgies périnéales.

Ninety cases of chronic perineal pain of neurological origin are reported. Alcock's canal syndrome, consecutive to damage of the pudendal nerve in the ischiorectal fossa, is the most frequent of these neuralgias. It is characterized by burning pain or paraesthesia increased in sitting position and relieved by standing up. The specific treatment is CT-guided infiltrations of the pudendal nerve. Other neurological causes are spinal cord lesions (notably tumours of the conus medullaris), sacral meningoradiculitis (perineal herpes zoster), plexitis and pudendal nerve neuritis. In some cases the responsibility of perineal stretching neuropathy may be considered. In all patients, electrophysiological exploration of the perineum (detection of perineal floor muscles, sacral latency, somatosensory and motor evoked potentials of the pudendal nerve) are necessary to confirm the aetiological diagnosis and guide neurological investigations.

[Right hemiplegia and spatial neglect with apraxia and agraphia without aphasia in a right-handed patient]. / Hémiplégie droite et négligence avec apraxie et agraphie sans aphasie chez une patiente droitière.

A 65 year-old right-handed woman was admitted after the sudden onset of a right dense hemiplegia. C.T. showed a large left infarction in the middle cerebral artery territory. There was a slight anosognosia and neglect of the right space without confusion. She had aprosodia but no aphasia. On the other hand, there was a severe apraxia and all the components of Gerstmann's syndrome were present. This suggests an unusual sattering of hemispheric functional dominances.

[Perineal neuropathy due to stretching and urinary incontinence. Physiopathology, diagnosis and therapeutic implications]. / Neuropathie périnéale d'étirement et incontinence urinaire. Physiopathologie, diagnostic et implications thérapeutiques.

Perineal nerve injury due to stretching is caused by excessive traction on the distal motor branches of the pudendal nerve that innervate the perineum and anus. These injuries can occur in a number of morbid conditions (prolapsus, anorectal dyschezia, pelvic surgery) and induce denervation of the pelvic floor that very probably modifies the resistance of the sphincters. Stretch injuries should be considered when discussing physiotherapy or surgery for urinary incontinence. Diagnosis can be established by electrophysiologic studies of the perineum, particularly by measurements of distal pudendal nerve motor latencies. The authors report a series of sixty patients with stretch-induced neuropathy.

[Alcock's canal syndrome and perineal neuralgia]. / Syndrome du canal d'Alcock et névralgie périnéale.

Fifteen cases of perineal neuralgia are reviewed, the lesion arising from a canal syndrome due to compression of the pudendal nerve in the ischiorectal fossa (Alcock's canal syndrome). The clinical characteristic of the pain syndrome was its postural nature with the existence of a true Tinel sign (increased pain on sitting). Diagnosis was confirmed in all cases by a perineal electrophysiological which showed peripheral neurogenic signs on examination of perineal muscles and an increase in sacral evoked potentials latencies (latency of bulbocavernous or clitorido-anal reflexes, cortical somesthetic evoked potential from pudendal nerve). Treatment was infiltration of cortisone derivatives into the pudendal nerve canal, under CT guidance because of the difficulty of infiltrating the pudendal nerve by an external perineal approach. Results were satisfactory in 9 of the 15 patients.

[Polyneuropathy and solitary bone plasmacytoma. A new case]. / Polyneuropathie et plasmocytome solitaire osseux. Une nouvelle observation.

The authors report a case of acute polyneuropathy revealing a solitary osseous plasmacytoma with osteo-dense and osteolytic bone lesions. Initially, the rapid progression of the sensory and motor loss led to treatment by plasma exchanges and irradiation of the plasmacytoma. Four months later, despite a significant improvement of the neurological condition, serum protein electrophoresis continued to show a peak of monoclonal immunoglobulin. Chemotherapy with cyclophosphamide and prednisone was administered for one year whilst the neuropathy continued to regress. This case, which presents many classical features of plasma cell dyscrasia (polyneuropathy with albumino-cytological dissociation, radiological osseous condensation, low concentrations of lambda light chain protein), illustrates some unusual features of solitary plasmacytomas associated with peripheral neuropathy: the young age of our patient, an acute progression of the neuropathy in the early stages, tumoral localisation in the diaphysis of a long bone.

[Value of evoked sacral potentials in studying bladder sphincter disorders in peripheral neuropathies and central nervous system diseases. A study of 110 cases]. / Intérêt des potentiels évoqués sacrés dans l'étude des troubles vésico-sphinctériens des neuropathies périphériques et des affections du système nerveux central. Etude de 110 cas.

The sacral spinal cord is one of the main sites of integration of bladder and anal sphincter and sexual function. Clinical tests (bulbocavernous reflex, perineal reflexes) are often inadequate and urodynamic investigations (cystomanometry, urecholine test, Susset's test) are sometimes unhelpful. The integrity of this structure is essential for normal sphincter function and disease at this level (or of its afferent or efferent limbs) produces a peripheral type of disorder. Sacral evoked potentials are an electrophysiological means of testing the bulbocavernous reflex by external stimulation of the dorsal nerve of the penis or clitoris and recording by a needle bipolar electrode in the striated muscle of the sphincter or the bulbocavernous muscle. An objective study of the sacral reflex are (S2, S3, S4) can thus be performed. One hundred and ten patients have undergone this investigation. Normal latency established in a series of 26 healthy volunteers was less than 42.1 ms. Prolongation of this interval is a sign of disease of the reflex are and correlated with urinary or sexual problems in 46 cases of clinical or electrical peripheral neuropathy. The reflex time was normal in all patients with CNS disease (32 cases) and an impotence of psychological origin (6 cases).

[Myopathy, osteomalacia, and congenital hypoalbuminemia (author's transl)]. / Myopathie. Ostéomalacie et hypoalbuminémie congénitale.

A myopathy revealed the presence of a vitamin-sensitive osteomalacia in a patient with congenital hypoalbuminemia. The severe myopathy and osteomalacia recovered after treatment with 25-hydroxycholecalciferol. Hypoalbuminemia was detected in two brothers of the patient; parents and grandparents were consanguineous. Various tests demonstrated that the patient's hypoalbuminemia was due to faulty synthesis. Albumin reactions to several drugs were normal, but those normally transported by albumin have to be administered cautiously because of the low plasma concentrations. The possible relationship between hypovitaminosis D and congenital hypoalbuminemia, an association considered to be fortuitous by Montgomery, remains debatable.