[Meningoradiculitis after injection of an antirabies vaccine. A vaccine from human diploid cell culture]. / Méningo-radiculite secondaire à une vaccination antirabique. A partir d'un vaccin préparé sur culture de cellules diploïdes humaines.

We report the case of a 45-year old farmer who developed meningoradiculitis after preventive anti-rabies vaccination with a vaccine obtained from human diploid cell culture. Two weeks after the second injection of vaccine, the patient complained of sensory symptoms in the right half of his body. These symptoms spontaneously regressed. The literature is reviewed and the physiopathological hypotheses are discussed.

[Mitochondrial encephalopathy affecting only the central nervous system]. / Encéphalopathie mitochondriale avec atteinte exclusive du système nerveux central.

A 32 year-old diabetic woman presented with an acute coma followed by epileptic seizures, aphasia and constructive apraxia. No ischemic lesion was demonstrated by CT scan and carotid angiograms. The other investigations showed sensorineural hearing loss, retinal degeneration, calcifications of the basal ganglia and lactic acidosis. The follow-up was marked by pseudo-dementia with personality disorders, memory deficits, behavioural changes, migrainous and epileptic features. Although there was no sign of muscular deficiency, a muscular biopsy showed characteristic ragged-red fibers and mitochondrial abnormalities at electron microscopy. The muscular biopsy enables us to classify this case as a mitochondrial encephalopathy similar to the MELAS syndrome. The stroke-like episodes are probably caused by a specific angiopathy involving the mitochondria of brain vessels.

Type 2 fiber predominance in muscle cramp and exertional myalgia.

In patients complaining of muscle cramps and exertional myalgia, we found a significant decrease of type 1 muscle fiber proportion in comparison with a control group. The possible mechanism of this change is discussed.

[Familial dementia of the Neumann type (subcortical gliosis)]. / Démence familiale de type Neumann (gliose sous corticale).

The case of a 70 year-old patient with familial dementia is reported. A cerebral biopsy and neurotransmitters dosages in CSF and brain tissue ruled out a primary dementia as Pick's or Alzheimer's disease. Our case may be related to Neumann's cases of subcortical gliosis.

Unclassified familial myopathy resembling Steinert disease, without myotonia.

A familial myopathy with predominantly proximal muscle atrophy is described. Although several clinical features such as sternomastoid and brachioradialis muscle involvement suggested Steinert disease, myotonia was not demonstrated by clinical examination or EMG. Histological data were consistent with myotonic dystrophy.

Cerebral amyloid angiopathy as a cause of multiple intracerebral hemorrhages.

Clinical and pathologic findings in six autopsies and five biopsies of cerebral amyloid angiopathy associated with cerebromeningeal hemorrhages are presented. One patient had experienced a previous meningeal hemorrhage. Only two had chronic hypertension; the multiple fresh hematomas found in all the autopsied brains always spared the basal ganglia and brainstem, as did vascular lesions, which were mostly cortical and meningeal. Extensive lesions of Alzheimer's disease were found in the autopsied cases with dementia. The most significant feature for clinical diagnosis of hemorrhagic cerebral amyloid angiopathy is the presence of multiple hemorrhages in unusual locations in the absence of hypertension.

[Adrenomyeloneuropathy. 4 cases developing with the features of familial spasmodic paraplegia]. / Adrénomyéloneuropathie. Quatre cas évoluant sous les traits d'une paraplégie spasmodique familiale.

Four cases of adrenomyelopathy, the adult form of adrenoleukodystrophy, three of which were familial, were reported. The neurological disease consisted of progressive spasmodic paraplegia, sphincter disorders, peripheral neuropathy, occasional deep sensibility impairment and psychiatric disturbances in one case. Adrenal insufficiency was confirmed after the appearance of the neurological signs in two cases; in another case it had been diagnosed in childhood. Gonadal insufficiency was present in all cases and clinically apparent in two patients. Detailed genetic studies were carried out in two patients of the same family. Histocompatibility tests showed A2 and B15 antigens in three out of four cases. The diagnosis was confirmed in three cases by measurement of serum long chain fatty acid with increased C26 levels and C26/C22 ratios. Peripheral nerve biopsy in 1 patient showed characteristic inclusion bodies in the Schwann cells. The authors discuss the relationship between ALD and familial spasmodic paraplegia, the mode of genetic transmission of the disease and the diagnostic value of neuromuscular biopsy and measurement of serum long chain fatty acid levels.

[Adrenomyeloneuropathy: clinical, genetic and morphologic features. Apropos of 5 cases]. / Adrénomyéloneuropathie: particularités cliniques, génétiques et morphologiques. A propos de cinq cas.

The authors describe five cases of adrenomyeloneuropathy, an adult form of adrenoleukodystrophy. Three of the cases were related. The clinical picture comprises progressive spasmodic paraplegia, sphincter disorders, peripheral neuropathy, sometimes hollow foot and posterior columns disorders. In one case psychic disorders were observed. Biochemical analyses confirmed or revealed adrenal insufficiency in four cases and gonadal insufficiency in three cases. A detailed genealogical study was made in two patients belonging to the same family. Histocompatibility tests revealed the presence of A2 and B15 antigens in three cases. Diagnosis was confirmed in three cases by plasma determination of long-chain fatty acids, with an increase in the level of C26 acids and the C26/C22 ratio; biopsy of the peripheral nerve in one patient revealed inclusions characteristic of Schwann's cells. Symptomatological links between ALD and familial spasmodic paraplegia, the mode of genetic transmission of the disease, the contribution of neuromuscular biopsy to the diagnosis and the therapeutic prospects of a diet low in long-chain fatty acids are discussed.

[Changes in the motor units in patients with traumatic paraplegia]. / Modifications des unités motrices chez des malades atteints de paraplégie traumatique.

Neuromuscular biopsies were obtained in 10 patients with traumatic spinal cord lesions at various levels and in one patient with cerebral lesions due to a long lasting coma. In all cases, there was a rapid onset of both fibers types atrophy, with numerous transitional type III and intermediate fibers. Only the two patients with cervical lesions had an increased terminal innervation ratio suggesting denervation. Our observations do not support the hypothesis of a transneuronal degeneration. These changes cannot be related to disuse only as the atrophy does not preferentially involve type II fibers. The underlying neural mechanism of spasticity in muscle of patients with cortico-spinal lesions could explain the change of the histochemical pattern of the muscle fibers.

Adult form of basal cell naevus syndrome: a family study.

A 32-year-old patient had marked reduction of visual acuity due to falciform folds of the retina and retinal detachment, and severe neurological abnormality: bilateral pyramidal involvement, fasciculation in all limbs and gait ataxia. Skull radiographs showed internal frontal hyperostosis; CT scan showed calcification of the falx cerebri, and multiple arachnoid cysts were shown by myelography. A naevoid lesion had previously been removed from the left forearm. There was a history of ophthalmological symptoms in the mother and the daughter of the propositus. His son has "café au lait" spot on the abdomen and dentigerous cysts. The diagnosis of an adult form of basal cell naevus syndrome with an autosomal dominant mode of inheritance is discussed.

[Terminal innervation in 3 cases of leprous neuropathy (author's transl)]. / Etude de l'innervation terminale dans 3 cas de lèpre nerveuse.

Three cases of leprosy are reported. In all patients, the diagnosis was made by finding Hansen bacilli in skin lesions, nasal secretions and ears, and the immunological typing of leprosy was established. Conduction velocity of motor and sensory nerves was measured and muscle biopsies were obtained. Histochemical typing of muscle fibers and measurement of the terminal innervation ratio of motor axon vitally stained with methylene blue were performed. A subclinical involvement of peripheral motor nerves was demonstrated by these investigations.

Charcot-Marie-Tooth disease associated with retinal pigment dystrophy and protanopia. Neurological, ophthalmological and genetic study of a family.

Neurological, ophthalmological and genetic investigations were performed on a family, a member of which presented with a rare association of tapeto-retinal degeneration, protanopia and Charcot-Marie-Tooth disease (CMT), and asked for genetic counseling. The neurological enquiry was completed by measurement of motor nerve conduction velocity in several completed by measurement of motor nerve conduction velocity in several members of the family. The propositus was submitted to a muscle biopsy. The ophthalmological examination included ophthalmoscopy, fluorescein angiography, electroretinogram and electrooculogram. The propositus, a woman aged 40, had typical CMT disease and her father also had a mild form of it. She had protanopia as had her father, her son and her nephew. In addition she had large macular pigmented changes, described as retinal dystrophy, "flavus flavimaculatus." Her mother had only senile pigmented modification of the fundus and her three daughters had mild macular pigmented changes, like "salt and pepper." Two genes are probably involved: one for protanopia with X linked recessive inheritance, the other responsible of CMT and tapeto-retinal degeneration, with an autosomal dominant inheritance, giving a 50% risk of recurrence.

[Familial periodic paralysis with hypokaliemia, hyperaldosteronism and extracellular vacuolization (author's transl)]. / Paralysie périodique familiale avec hypokaliémie, hyperaldostéronisme et vacuolisation extra-cellulaire.

The observation of a 20 years old patient with a periodic paralysis is reported. During a crisis induced by 20 U of insulin and 150 g of glucose, transient hyperaldosteronism was found and the muscular biopsy performed in the left biceps brachii, disclosed vacuoles with a double membrane. The significance of these findings is discussed.

[Diffuse poliencephalitis and visceral neoplasm (author's transl)]. / Polioencéphalite diffuse et néoplasme viscéral.

A case of diffuse polioencephalitis associated with non-differentiated bronchial carcinoma is reported. Clinically the patient presented mental distrubance and an extra-pyramidal syndrome. Anatomopathological investigation revealed inflammation of the cerebral hemispheres and the brain stem. Similar involvement of others regions of the central nervous system has been reported in the literature, probably indicating various locations of a single nosological entity. A viral origin is probable but not yet established.