André Kohler

A Controlled Study of Early Neurologic Abnormalities in Men with Asymptomatic Human Immunodeficiency Virus Infection

BACKGROUND Although neurologic complications are frequent in the acquired immunodeficiency syndrome, their incidence and progression in early human immunodeficiency virus (HIV) infection remain controversial. The goal of this study was to assess neurologic manifestations in asymptomatic carriers of HIV. METHODS We studied 29 HIV-seropositive homosexual men and 33 seronegative homosexual controls by means of neurologic and neuropsychological examinations, magnetic resonance imaging (MRI), and electrophysiologic tests (electroencephalography, multimodal evoked-potential tests, and otoneurologic tests). After six to nine months, the tests were repeated in 27 seropositive men and 30 controls. The investigators were blind to the serologic status of the subjects. RESULTS The seropositive subjects had a mean CD4+ lymphocyte count of 635 X 10(6) per liter. Neurologic and neuropsychological examination, MRI, and measurements of pattern visual evoked potentials did not show significant differences between the two groups. The latencies of the median-nerve somatosensory evoked potentials were slightly prolonged in the seropositive men. The initial electroencephalogram was judged abnormal in 8 of 27 of the seropositive subjects (30 percent) as compared with none of the controls, with a slowing of fundamental activity, anterior spread [corrected] of alpha rhythm, subnormal reactivity, and unusual anterior theta activities. These findings were confirmed by computerized spectral analysis. The second electroencephalogram was abnormal in 10 of 25 of the seropositive men (40 percent). The otoneurologic evaluation identified abnormalities in the central auditory or vestibulo-ocular pathways in 34 percent of the seropositive men (10 of 29), as compared with 6 percent of the controls (2 of 33), on the first examination and in 44 percent (12 of 27) and 7 percent (2 of 30), respectively, on the second examination. Altogether, electrophysiologic abnormalities were found in 67 percent of the seropositive men (18 of 27) and 10 percent of the controls (3 of 30) (P less than 0.00005). CONCLUSIONS In persons with asymptomatic HIV infection, electrophysiologic tests may be the most sensitive indicators of subclinical neurologic impairment. Electrophysiologic abnormalities are far more common in asymptomatic carriers of HIV than in controls and tend to progress over time.

Needle muscle biopsy in the investigation of neuromuscular disorders.

We have evaluated needle muscle biopsies in 220 patients with various neuromuscular disorders, using a method developed previously at Tufts University. The method uses a 14‐gauge needle propelled by an automatic device. An average of 3.5 samplings were taken per patient. Muscle samples were used for histological and molecular genetic analysis, and for the isolation of muscle satellite cells for in vitro cultures. The biopsy is well tolerated by the patients who never declined multiple samplings. Complications were few and minor, with no sequelae. In most cases the small size of the muscle specimen (ca. 15 mg per sampling) was sufficient to perform the various procedures and to yield a diagnosis. Specimens were considered insufficient for histological results in 9 patients (4%), due to technical artifacts or insufficient material. We now routinely use this method, which has several advantages over the surgical technique for most muscle biopsies.

Cerebrospinal fluid in acute peripheral facial palsy.

Abstract Cerebrospinal fluid (CSF) is rarely analyzed in peripheral facial palsy, and reports in the literature are scarce. We report the CSF findings in 265 patients with acute isolated peripheral facial palsy. The CSF findings were abnormal in 11% of 230 patients with idiopathic peripheral facial palsy, in 60% of 17 patients with Ramsay Hunt syndrome (pleocytosis), in 25% of 8 patients with Lyme disease, in all of 8 patients with HIV infection, and in 2 other patients (sarcoidosis and herpes simplex). We conclude from this large series that the CSF is usually normal in idiopathic peripheral facial palsy. If the CSF is abnormal, a specific cause should be sought.

Fisher's syndrome associated with Campylobacter jejuni infection

A patient developed typical features of Fishers syndrome after an infection with Campylobacter jejuni as ascertained by positive serology both in the blood and spinal fluid. This may be the first case of this association described.

Proximal myotonic myopathy: clinical, electrophysiological and pathological findings in a family.

Proximal myotonic myopathy (PROMM) is an autosomal dominant muscle disorder characterized by proximal weakness, myotonia, muscle pain and cataract. It resembles Steinert myotonic dystrophy (MD), but weakness is proximal, without facial muscle involvement, and the chromosome 19 CTG trinucleotide repeat expansion characteristic of MD is not present. We describe a further family with PROMM. Affected members complained of weakness of lower limbs or of myotonia. EMG revealed diffuse myotonic discharges. Muscle histology showed dystrophic abnormalities. The PROMM phenotype varies, even in the same pedigree, and may mimic MD or limb-girdle muscle dystrophy. EMG is particularly useful, since it may disclose myotonic discharges even in the absence of overt myotonia. Thus far it is not known whether PROMM is a single entity, or if it represents a heterogeneous group of disorders. This question will probably soon be settled through genetic analysis.

Conduction Block in Vasculitic Neuropathy

We describe a 28-year-old man who developed a mononeuropathy with a conduction block of the median nerve in the forearm, during the acute stage of a polyarteritis nodosa. Sequential studies show progressive disappearance of the conduction block (together with the clinical symptoms), without signs of axonotmesis.

Contraction response to muscle percussion is increased in peripheral nerve conduction block

We studied the contraction of skeletal muscle upon direct percussion in 119 patients presenting with palsies related to focal peripheral nerve disorders.We compared the response of the paralyzed muscles with the response obtained on the contralateral normal side. The cause of the palsy was subsequently assessed by electrophysiological testing which disclosed: conduction block (CB), axonal, or mixed lesions (when both CB and denervation existed in similar proportions). Most of the time, the muscle response to percussion was increased in patients with CB (37/47), decreased in axonal lesions (44/53), and symmetric in mixed lesions (12/19). We conclude that distinction between CB and denervation is often possible on the basis of this simple bedside test. NEUROLOGY 1996;47: 1243-1246

Conduction Block of the Ulnar Nerve in Cervical Dystonia

Introduction Association between dystonia and peripheral neuropathy has been frequently reported. On the one hand, focal dystonias secondary to peripheral lesions have been documented [1, 2]. On the other hand, dystonic posture may induce peripheral neuropathy, such as when cervical dystonia causes a compression of cervical nerves [3]. We report the observation of a patient presenting with such an association, but with a different mechanism, rarely reported [4].