Brain metabolism in mitochondrial encephalomyopathy: a PET study.

Regional brain glucose metabolism has been studied in a case of mitochondrial encephalomyopathy with ragged red fibers, using positron emission tomography with fluorodeoxyglucose as the tracer. A marked decrease in glucose utilization was found in all gray structures, with a preponderance of metabolic alterations in posterior cortical regions and thalamus and a relative sparing of anterior cortical areas and basal nuclei.

[Wegener's disease. Unusual initial symptomatology: acute pain in the lower limbs]. / Maladie de Wegener. Symptomatologie initiale inhabituelle: douleur aiguë des membres inférieurs.

The authors report a case of Wegener's granulomatosis unusual for its initial signs. The first evidence of disease consisted in pains in the lower limbs, probably caused by inflammatory processes involving the fascia. Biochemical, radiological and histological examinations allowed the diagnosis to be made.

[Multiple cranial nerve involvement associated with tentorial pachymeningitis of granulomatous type]. / Atteinte multiple des paires crâniennes associée à une pachyméningite tentorielle à caractère granulomateux.

Otitis media in a 43 year-old male patient was followed by signs of chronic meningitis with persistent headaches, moderate pleiomorphic lymphocytosis of CSF without hypoglycorrhachia and unilateral impairment of cranial nerves XI to II. These signs were accompanied by widespread inflammation with general deterioration of the patient's condition. After a time the tentorium of cerebellum showed thickening and significant contrast uptake in conjunction with necrotizing granulomatous inflammation, indicating tentorial pachymeningitis. Swift and complete remission was obtained with a corticoid-cyclophosphamide combination. After reviewing the other possible causes of chronic meningitis, the authors proposed a diagnosis of Wegener's disease of auricular origin in view of the compatibility of all the data. A few cases of cranial pachymeningitis of indeterminate origin are described in the literature. Only this patient was treated with a corticoid-cyclophosphamide combination. The remarkable efficacy of this double therapy confirms the diagnostic hypothesis of Wegener's granulomatosis.

[Wartenberg's disseminated sensory neuropathy]. / La neuropathie sensitive disséminée de Wartenberg.

We studied ten cases of disseminated sensory neuropathy. Both sexes were similarly involved. Except in one case, the first symptoms appeared between ages 20 and 50. The clinical disorders, their evolution and similar cases previously reported suggest and autonomous benign disease. The deficits resulted from a selective involvement of superficial cutaneous nervous fibers and appeared in different cutaneous areas. Some territories were more frequently affected (nervus peroneus, sural, radialis, cutaneous femoralis lateralis). Tinel's sign was often present. Patients sometimes described a sensation of electric discharge when elongating nerve trunks. In half of the cases the attacks of dysesthesia or of sensory loss followed one another within less than one year. The deficits were fully reversible in a third of the cases after a mean total evolution of 4 to 8 years. Blood and cerebrospinal fluid were always normal. Electromyography and motor conduction velocities were normal. Sensory conduction velocities were uncommonly decreased. On the other hand, the aspect of somesthetic potentials which were evoked from cutaneous areas involved by the disease were modified in most cases. Pathological examination of nerve biopsies which were performed in 5 cases showed non specific axo-myelinic degeneration in 3 cases and a severe sclerosis of the perineurium in one case. The etiopathogenesis of the disease is discussed.

[Internuclear ophthalmoplegia and non-paralytic pontine exotropia]. / Ophtalmoplégie internucléaire et exotropie pontine non paralytique.

The authors discuss internuclear ophthalmoplegia associated with non-paralytic pontine exotropia of the contralateral eye. Impairment of the posterior oculogyric tract in its protuberantial segment, resulting in oculographic signs of alteration in the contralateral continuation. Seems to be responsible for the exotropia.

[Anterior choroid artery syndrome with neuropsychological and oculomotor disorders]. / Syndrome de l'artère choroïdienne antérieure avec troubles neuropsychologiques et oculomoteurs.

A patient with infarct in the region of the right anterior choroidal artery presented neuropsychological signs corresponding to a minor hemisphere syndrome and disorders of horizontal oculomotricity similar to those obtained in unilateral parietal-occipital lesions. An anatomic interpretation of the latter finding is attempted.