A syndrome of REM and non-REM sleep reduction and lateral gaze paresis after medial tegmental pontine stroke. Computed tomographic scans and anatomical correlations in four patients.

A common pattern of reduction in both rapid eye movement and non-rapid eye movement sleep associated with various lateral gaze paralyses was present in four cases of brain-stem stroke. From computed tomographic scan data, clinical inferences, and, in two cases, neuropathologic sections, the common lesions were localized in the medial pontine tegmentum, ie, the inner part of the gigantocellular and pontis centralis caudalis nuclei. These data in humans were compared with lesions obtained experimentally in cats.

Perhexiline maleate and peripheral neuropathy.

Peripheral neuropathy has been noted as a complication of therapy with perhexiline maleate, a drug widely used in France (and in clinical trials in the United States) for the prophylactic treatment of angina pectoris. In 24 patients with this complication, the marked slowing of motor nerve conduction velocity and the electromyographic changes imply mainly a demyelinating disorder. Improvement was noted with cessation of therapy. In a few cases the presence of active denervation signified a poor prognosis, with only slight improvement. The underlying mechanism causing the neuropathy is not yet fully known, although some evidence indicates that it may be a lipid storage process.

Miller Fisher syndrome: clinical and electrophysiologic evidence of peripheral origin in 10 cases.

Clinical and electrophysiologic data recorded in patients with Fisher syndrome characterized by ophthalmoplegia, ataxia, and areflexia are presented. Cases with limb weakness or pleocytosis in the CSF were excluded, according to Fisher. Ten patients were selected. All had hand and foot numbness. A large amount of protein without cell reaction was found during the third week of illness in serial CSF examinations. EMGs showed very slight limb involvement without spontaneous activity, and in most cases, facial muscles exhibited a denervation pattern. Distal motor nerve conduction velocity on limbs and F wave latencies were normal, whereas the sensory nerve action potentials were altered in all but one case when tested (seven out of eight cases). By means of blink reflex study performed in four patients, no significant pattern of brainstem dysfunction was discovered. The authors discuss the preeminent role of peripheral nerve lesions with regard to the ataxia and ophthalmoplegia.

Peripheral neuropathy in systemic vasculitis: clinical and electrophysiologic study of 22 patients.

Twenty-two patients with evidence of systemic vasculitis and peripheral neuropathy were clinically and electrophysiologically investigated in a retrospective study. Ten had a polyarteritis nodosa, 6 a probable polyarteritis nodosa, and 6 a Churg-Strauss syndrome. Nine patients presented clinically with mononeuropathy or mononeuropathy multiplex considered typical of ischemic involvement of peripheral nerve; nine had more diffuse neuropathy, two of them a symmetric polyneuropathy. EMG examination revealed more diffuse neuropathy than clinically predicted. Findings were of acute or subacute axonal neuropathy.

Cervical magnetic stimulation: a new painless method for bilateral phrenic nerve stimulation in conscious humans.

Assessing diaphragmatic contractility is a common goal in various situations. This assessment is mainly based on static or dynamic maximal voluntary maneuvers and twitch transdiaphragmatic pressures (Pdi) obtained by stimulation of the phrenic nerves (PS). PS eliminates the central components of diaphragmatic activation, but the available techniques of PS remain subject to some limitations. Transcutaneous PS is painful, and needle PS is potentially dangerous. Time-varying magnetic fields can stimulate nervous structures without pain and without adverse effects. In six subjects, we have studied cervical magnetic stimulation (CMS) as a method of PS. We have compared the stimulated Pdi (Pdistim) with the maximal Pdi obtained during static combined expulsive-Mueller maneuver (Pdimax) and with the Pdi generated during a sniff test (Pdisniff). CMS produced twitch Pdi averaging 33.4 +/- 9.7 cmH2O. Pdistim/Pdimax and Pdistim/Pdisniff were 24 +/- 6 and 41 +/- 14%, respectively. These values are comparable to those obtained in other studies with transcutaneous PS. They were highly reproducible in all the subjects. Electromyographic data provided evidence of bilateral maximal stimulation. CMS is a nonspecific method and may stimulate various nervous structures. However, diaphragmatic contraction was elicited by stimulation of the phrenic trunk, since the phrenicodiaphragmatic latencies (less than 7 ms) were in the range of values reported with direct stimulation of the trunk. Cocontraction of neck muscles, including the sternomastoid, was present, but its influence in the CMS-induced Pdi seems minimal. We conclude that magnetic stimulation is an easy, well-tolerated, reproducible safe, and valuable method to assess phrenic conduction and diaphragmatic twitch response.

Peripheral neuropathies during treatment with almitrine: report of 46 cases.

Almitrine bismesylate is thought to cause sensory peripheral neuropathy. Forty-six patients are reported who received almitrine bismesylate alone for chronic respiratory failure or in combination with raubasine for various cerebrovascular diseases. Polyneuropathy appeared between 9 and 25 months after the onset of treatment. Sensory signs and symptoms were confined to the distal parts of the lower limbs and involved large and small fibres. Histological and electrophysiological findings indicated axonal degeneration. Respiratory failure could have caused the polyneuropathy in some cases but many had no chest disease. Patients began to improve between 3 and 6 months after withdrawal of the drug. Recovery was usually complete after 12 months.

Peroneal muscular atrophy. Part 1. Clinical and electrophysiological study.

144 patients with the clinical syndrome of peroneal muscular atrophy or Charcot-Marie-Tooth disease were studied. Thirteen were recognized as the spinal form of the disease since all had normal motor nerve conduction velocity and sensory nerve action potential. The remaining patients could be classified according to their values of motor conduction velocity for the median nerve. Two main groups were therefore identified: 55 patients whose nerve conduction was below 30 m/s belonged to group I, which corresponds to the previously reported hypertrophic form or hereditary motor sensory neuropathy (HMSN) type I. Sixty-four patients whose nerve conduction was above 40 m/s belonged to group II which corresponds in the majority of cases to the neuronal form or HMSN type II. Twelve patients could not be classified since the motor nerve conduction velocity for median nerve ranged between 30 and 40 m/s. These could belong to either of the two main groups or might form an intermediate group, the existence of which is discussed. Clinical genetic and electrophysiological features of the two main groups are discussed and compared.

[Recurrent peripheral nerve palsies and concomitant chronic neuropathy. Neuromusclar electrophysiologic study and neuropathologic examination: apropos of 5 cases]. / Paralysies tronculaires récidivantes et neuropathie chronique concomitante. Etude électrique neuromusculaire et examen neuropathologique: a propos de 5 observations

The authors report 5 personal cases (3 in the same family) of patients suffering from recurrent peripheral nerve palsies usually induced by pressure and review the relevant literature. There is evidence in such cases of a mild, chronic, diffuse, subclinical neuropathy which can be recognized by electrophysiological examination. Examination of peripheral nerve biopsy material in such cases shows evidence of both axonal and Schwann cell abnormalities with evidence of neurogenic atrophy of skeletal muscles. This chronic subclinical neuropathy which is often familial can be distinguished from other chronic primary neuropathies with or without myelin sheath proliferation and hypertrophy.

[Sleep and dreams in Korsakoff's amnesia due to alcoholism]. / Sommeil et rêves dans l'amnésie de Korsakoff d'origine alcoolique.

Sleep and dreams in 15 chronic alcoholic patients with amnesia were compared with sleep and dreams of 15 age- and sex-adjusted normal subjects. The patients were subjected to psychological tests in order to determine their I.Q. and their memory disturbances. All subjects had two nights of polygraphic recordings; the first tested the natural sleep organization. During the second night, they were awakened 7 min after the onset of each REM sleep episode, and, at least once, 20 min after the onset of a stage II episode, in order to record on a tape their dream reports according to a standardized protocol. The sleep patterns of the amnesic patients did not show any significant alteration. However, after wakening during the night, patients exhibited a higher tendency to return to REMS than controls. There was still some dream activity in those patients, although noticeably less frequently, and their dream activity had a very poor verbal expression. However, there was no change with respect to the spatio-temporal organization, sensorial perceptions, motor activity and verbalizations during their dreams.

[Sleep abnormalities and evoked potentials (VEP-BAER-SEP) in progressive supranuclear palsy]. / Etude des anomalies du sommeil et des potentiels évoqués (PEV-PEAp-PES) dans la paralysie supranucléaire progressive (PSP).

Few physiological studies have been performed in PSP. We studied: sleep abnormalities in 36 h polygraphic recordings; changes of PEV after pattern-reversal stimulation, of BAER and of short latency SEP after stimulation of the median nerve. The population was for the 1st group: 18 patients with full typical symptomatology, for the 2nd group: 7 patients with likely diagnose of PSP and for the 3rd group: 10 normal subjects as control sample. All patients of the 1st group had sleep abnormalities: decrease of total sleep time; decrease of the percentage of REM sleep; morphological abnormalities (specially horizontal ocular square wave jerks). Detail is given of the repartition of such abnormalities in the two groups of patients. There is no correlation between sleep abnormalities and the natural history of the disease. The PEV and BAER, abnormalities were present in 50% of the cases. The PES were always normal. The help that can be provided by electrophysiological studies in the diagnose of PSP is discussed (particularly in group 2).

Effect of age on auditory evoked responses (AER) and augmenting-reducing.

Auditory evoked responses (AER) were obtained from Cz and Fz in 30 adults (14 male, 16 female) from 20-80 years old. Sound bursts (1000 Hz-200 msec) of four different intensities were used. Peak to trough amplitudes of P1N1 and N1P2 and latencies of P1, N1 and P2 peaks were measured with increasing stimulus intensity and slopes of amplitude - intensity and latency - intensity curves were analysed for assessment of an age effect. The main result is that the increase in P1N1 amplitude with increasing stimulus intensity is more pronounced in older persons. Previous studies have established a negative correlation between the augmenting-reducing responses and HVA levels in the CSF (with lower amounts of HVA in the CSF of "augmenters"). Decreased dopamine metabolism in old subjects could account for our results, so further studies should focus on patients with pathological dopamine deficiencies.

Auditory evoked responses (AER) and augmenting-reducing phenomenon in patients with progressive supranuclear palsy (PSP).

Variations in amplitude and latency of P1, N1 and P2 waves of AER induced by increasing the stimulus intensity (augmenting-reducing) were measured in PSP patients and compared to those observed in normal subjects. The studied population included 17 patients (10 male, 7 female, mean age 66 +/- 8 yr) with a typical PSP symptomatology and 17 normal subjects (10 male, 7 female, mean age 66 +/- 9 yr). All subjects from both the groups showed a normal auditory threshold (less than 30 db SPL or a moderately increased threshold never exceeding 10 db SPL). Nine patients had normal BAER; 4 patients showed an abnormal III wave; 3 patients showed an abnormal V wave. One patient had a poorly individualized BAER. Latencies and amplitudes of P1, N1 and P2 waves derived from Cz and Fz (linked ear reference) were studied with 50, 60, 70 and 80 db intensities and for each patient slopes of amplitude-stimulus intensity and latency-stimulus intensity curves were studied. Although patients showed decreased AER amplitudes, the augmenting-reducing phenomenon was not different from controls regarding either latency or amplitude changes with increasing stimulus intensity. Previous studies had established a negative correlation between the augmenting-reducing responses and HVA levels in the cerebrospinal fluid (CSF). Similarity of augmenting-reducing mechanisms in PSP and normal subjects favors the hypothesis of unimpaired mesocortical and mesolimbic dopaminergic pathways in PSP. This hypothesis is also supported by postmortem studies using biochemical markers.

[Efficacy of uvulopalatopharyngoplasty (UPPP) and modifications in sleep structure in the sleep apnea syndrome (SAS)]. / Efficacité de l'uvulopalatopharyngoplastie (UPPP) et modifications de la structure du sommeil dans le syndrome d'apnée du sommeil (SAS).

Thirty-four patients (32 male, 2 female; mean age 53 +/- 7 years) with confirmed sleep apnea syndrome (SAS) were studied before and after uvulopalatopharyngoplasty (UPPP). Clinical symptoms were tiredness, excessive daytime sleepiness and snoring. All patients were overweight. Patients underwent a thorough physical and oropharyngeal examination and polysomnography before and 3 months after surgery. On the basis of post-operative results, patients are divided into 3 groups: --group 1: 16 cured patients: apnea index (A.I./h) 38 +/- 17 before and 4.4 +/- 4 apneas/h sleep after surgery. Improved nocturnal hypoxemia: mean minimum oxyhemoglobin saturation (SAO2) before and after UPPP in NREM sleep 83 +/- 4% v. 90 +/- 4% in REM sleep 76 +/- 11% v. 85 +/- 7%. Uninterrupted sleep is restored; --group 2: 8 improved patients: A.I./h of 64 +/- 11 before and 20 +/- 6 after UPPP: improved nocturnal hypoxemia: mean minimum SAO2 in NREM sleep 74 +/- 10% before and 86 +/- 6% after UPPP: in REM sleep 59 +/- 9% before and 79 +/- 6% after UPPP, lower amount and percentage of fragmented sleep; --group 3: 10 non-improved patients: A.I./h unchanged 55 +/- 22% before and 50 +/- 20% after UPPP. Persistent nocturnal hypoxemia: mean minimum SAO2 in NREM sleep 76 +/- 13 before and 81 +/- 12% after UPPP: in REM sleep 63 +/- 16% before and 65 +/- 24% after UPPP. Sleep remains fragmented. In this last group patients are more overweight and all suffer from severe SAS with greater nocturnal oxyhemoglobin desaturation. Surgical treatment by UPPP is shown to be effective for 70% of our patients. Better results are obtained when SAS is less severe and overweight less important.

[Progressive muscular atrophy localized in the hand. Monomelic form of motor neuron disease?]. / Atrophie musculaire progressive localisée à la main. Forme monomélique de maladie du motoneurone?

Ten new cases of distal amyotrophy localized in an upper limb are reported. In all cases, patients were young males. The onset of the neurological impairment was always progressive during one to three years. The disease remained stable with a follow-up study of at least three years. Electrophysiological and neuroradiologic results were in keeping with an involvement of the anterior horn cell, in agreement with similar studies found in the literature. The relatively benign evolution of this syndrome, in regard to the usual prognosis of the other forms of motor neuron disease, especially amyotrophic lateral sclerosis, has to be outlined.

[Eaton-Lambert myasthenic syndrome: clinical, electrophysiologic, histological and ultrastructural study]. / Syndrome myasthénique de Lambert-Eaton. Etude clinique, électrophysiologique, histologique et ultrastructurale.

In this case, showing the classical features of the Eaton-Lambert myasthenic syndrome, the neuromuscular symptoms appeared 21 months before the bronchial carcinoma was diagnosed. The electrophysiological study demonstrated, beside the marked potentiation under repetitive nerve stimulation at 30 c/sec., some modifications of distal latency, specially after administration of Edrophonium chloride. The muscle biopsy showed changes secondary to a peripheral motor nerve involvement; electronmicroscopic study of the motor end-plates revealed a highly developped subneural apparatus. Long-run treatment by guanidine hydrochloride determined haematologic and renal toxic manifestations. Prednisolone was for a time active on the myasthenic syndrome.

[Heterochronia of nerve fibers in man. Diagnostic value: initial results]. / L'Hétérochronisme des fibres nerveuses chez l'homme. Son intérêt diagnostique : premiers résultats

A classical physiological observation not usually apparent with methods used for detecting nerve potential in man, heterochronia of the nerve fibres is often found in extraordinary circumstances with considerably higher than normal frequence : mainly in muscular disorders, in particular myotonic dystrophy ; more incidentally in disorders of muscle tone. The authors draw attention to certain sources of error which could lead to wrong diagnosis of heterochronia and emphasize that these are only the initial results of research which needs to be pursued further.

[Study of the relation between disorders of nocturnal respiratory rhythm and hypersomnia]. / Etude de la relation entre troubles du rythme respiratoire nocturne et hypersomnie.

Sleep and respiration were recorded during two consecutive nights in 15 normal subjects, 18 patients suffering from narcolepsy and 9 with non-cataplectic hypersomnia. The normal subjects showed episodes of central apnea, the degree of which increased with age and periodic respiration. In both categories of hypersomniacs, the patients were divided into three groups according to the severity of disturbances in nocturnal respiratory rhythm:--group 1: identical to the normal subjects,--group 2: moderate qualitative or quantitative abnormalities,--group 3: presence of a large number of episodes of obstructive apnea. On the basis of these results, it was concluded that disturbances in nocturnal respiratory rhythm are not specific for any particular type of hypersomnia, nor were they the main cause of hypersomnia when present to a large extent, but that obstructive apnea could be an aggravating factor.

[Detection and peripheral or central localization of sensory pathway involvement of the lower limbs by somatosensory evoked potentials]. / Détection et localisation périphérique ou centrale des atteintes des voies sensitives par les potentiels évoqués somesthésiques des membres inférieurs.

In the presence of more or less atypical sensory or sensorimotor symptoms the questions that arise most frequently concern the authenticity of the disorders and the precise level of the lesion. In this study, somatosensory evoked potentials (SEPs) to stimulation of the tibial nerve at the ankle were recorded at different levels in 35 healthy subjects and 32 patients with sensory disorders. Recording electrodes were placed at the popliteal fossa (peripheral sensory nerve conduction velocity), at the T12-L1 level (medullary potential: N21) and at the vertex (P40 wave). The spine to cortex time interval was measured. A systematic study of evoked responses to median nerve stimulation was performed. The 32 patients were divided into 4 groups: Group I (3 cases) had slowed sensory conduction velocity (SCV), similar delay in N21 latency and normal N21-P40: peripheral neuropathy. Group II (4 cases) had normal SCV, delayed N21 latency and normal N21-P40: radicular or conus medullaris injury. Group III (19 cases) had normal SCV, normal N21 latency and lengthened N21-P40 interval. A study of responses to median nerve stimulation made it possible to discriminate between spinal and cortical or subcortical impairment. Group IV (6 cases) had abnormalities from any two of the three groups defined above. In 24 out of 32 patients (75 p. 100), further investigations (myelography, MRI, EMG) confirmed the localization determined by evoked responses. In the other 8 patients (25 p. 100) whose clinical picture suggested a medullary or radicular impairment, SEPs alone clearly revealed an injury. SEPs can distinctly show a spinal impairment and determine the choice of further investigations.

[The peripheral nervous system and HIV infection. 13 cases]. / Système nerveux périphérique et infection par le V.I.H. 13 cas.

Disorders of the peripheral nervous system occur at all stages of HIV infection. From 13 patients referred for a peripheral neuropathy, 9 were known to be HIV seropositive and 4 were found to be seropositive. All were Caucasian males aged 27 to 58. Ten were homosexual, 2 were drug-addicts. Patients fell into several groups: the first group was made of 5 patients, 4 asymptomatic and 1 AIDS-related-complex (ARC), with an inflammatory demyelinating polyneuropathy, acute in 1 case and subacute in 4; the CSF showed pleiocytosis in all cases. Motor conduction nerve velocities were markedly reduced and motor distal latencies prolonged. Three patients recovered spontaneously, 1 responded to corticosteroids, 1 to plasmapheresis. In the second group, 4 patients, 1 asymptomatic and 3 ARC, had a distal symmetrical polyneuropathy; CSF was normal in 2 cases. Electrophysiological studies and nerve biopsies indicated a mixed axonal-demyelinating polyneuropathy. Three patients recovered spontaneously, 1 is unchanged. Among both groups, an infiltration of mononuclear cells was found on nerve biopsies in 4 cases. The third group was made of 3 patients with AIDS who presented with a painful sensory polyneuropathy involving the distal lower limbs. Electrophysiological and pathological study of the nerve indicated axonal degeneration. No cell infiltration was found. The last patient with AIDS had a progressive meningoradiculopathy. These 4 patients died within 6 months after the onset of the neuropathy. These findings are close to those previously reported, and confirm the wide spectrum of disorders of the peripheral nervous system associated to HIV infection.

[Sleep and dreams in patients with parietal and frontal lobe lesions]. / Sommeil et rêve chez des patients atteints de lésions partiétales et frontales.

Polygraphic recordings, psychological tests, and analyses of dreams during paradoxical sleep were conducted in 9 patients with parietal lobe, in 7 with frontal lobe lesions and in a control group. No significant differences in sleep organization were observed in the parietal group, but there was a considerable reduction in oneiric activity and alterations in results of some psychological tests. Several correlations based on statistical data are discussed. Oneiric activity was disorganized to a much lesser degree in patients with frontal lesions.