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[A patient with restless legs syndrome/periodic limb movement successfully treated by wearing a lumbar corset].

We report a 77-year-old woman with restless legs syndrome(RLS) and periodic limb movement(PLM). From 62 years of age, she was awakened by tingling and involuntary movement in her legs during sleep. There symptoms disappeared when she stood up and walked. She was treated with clonazepam (2.5 mg/day) and valproate (400 mg/day) at 77 years of age, and the symptoms clearly ameliorated. However, she developed mild truncal ataxia and was referred to our hospital. On admission, neurological examination revealed Babinski and Chaddock signs bilaterally with depressed tendon reflexes in the lower limbs, mild truncal ataxia and horizontal gaze nystagmus. She did not present with involuntary movement of the legs while taking the anti-epileptic drugs. Cessation of these drugs alleviated the truncal ataxia and nystagmus, but reexacerbated abnormal sensation with involuntary movement in her legs during sleep. The involuntary movements in her legs were slower than myoclonus and resembled a Babinski reflex (duration about 1 second), and they appeared periodically (around every 30 seconds) in I-II sleep stages. Neither brain MRI nor EEG detected any abnormality. Cervical MRI revealed focal compression of the spinal cord by osteophytes at C5-C6 (more severe on the left side). Motor evoked potentials with transcranial magnetic stimulation revealed a mild delay in the central conduction time (CCT), which was more prolonged on the left side. She was thus diagnosed as having RLS/PLM with involvement of the bilateral pyramidal tracts. Although nerve conduction studies did not detect any abnormality in the peripheral nerves, RLS/PLM of the left leg was induced by electric stimulation of the left tibial nerve. Because she did not want medication any more, we treated her with a lumbar corset, hoping that wearing a lumbar corset might induce contraction of the truncal muscles that would mimic standing and walking or might produce additional sensory input that would induce a 'sensory trick'. Consequently, her abnormal sensation and involuntary movement during sleep as well as the stimulation of the tibial nerve disappeared. Wearing a lumbar corset may inhibit the excitability of the spinal cord that generates RLS/PLM, though the level of sensory input by the corset was higher than the input level of abnormal sensation in her legs. A lumbar corset may be a useful alternative choice for patients with RLS/PLM, who cannot tolerate either anti-epileptic or dopaminergic drugs.

Aged↗

[Anesthetic management of a patient with hereditary spastic paraplegia].

We experienced the anesthetic management of a 39 year-old-male with hereditary spastic paraplegia (HSP) associated with pain due to pes cavus. He underwent orthomorphia ostectomy and tenodesis. Preoperative neurological examination revealed that he had slight dementia, symptoms of the pyramidal tract, lower limb bathyhypesthesia, and neurogenic bladder in addition to spastic paraplegia, and he was diagnosed as having combined type of HSP. Spinocerebellar degeneration is often accompanied with this type of HSP. To avoid the use of muscle relaxants and narcotics, we tried to induce anesthesia with inhalation of nitrous oxide (N2O), oxygen and sevoflurane (sevo). A laryngeal mask (LM) was inserted at the expiratory concentration of sevo 3.5%. Anesthesia was maintained by N2O, oxygen and sevo (2-3%) under spontaneous respiration. The patient recovered from anesthesia after the end of surgery, and the LM was removed smoothly. The patient's respiratory condition was stable, and no problems were found in the postoperative period.

Adult↗

[Acute onset of tuberculous meningoencephalitis presenting with symmetric linear lesions in the bilateral thalamus: a case report].

A 18-year-old woman was admitted to our hospital because of high fever and headache. Nuchal stiffness was present, and a CSF examination showed lymphocyte-domonant pleocytosis and a decreased level of glucose. Although antibiotics, aciclovir and an antimycotic drug were administered, disturbance of consciousness, involuntary movements, and pyramidal tract signs appeared. Soon after the medications were changed to antituberculous medicines, the meningoencephalitis started to subside, and was finally cured. Judging from the clinical findings, the CSF findings, the effectiveness of antituberculous medicines, an elevated ADA level in CSF, and positive conversion in tuberculin tests, the final diagnosis was made as tuberculous meningoencephalitis. At the severest stage of the disease, a brain MRI showed symmetric, linear lesions without the effect of Gd-enhancement in the bilateral thalamus, which thereafter disappeared along with the healing of the illness. From all these things, we conclude that thalamic and other parenchymal lesions should be kept in mind in case of acute tuberculous meningoencephalitis.

Acute Disease↗

[Diaphragmatic motor evoked potential in amyotrophic lateral sclerosis patients].

OBJECTIVE: To investigate the features and application of diaphragmatic motor evoked potential (DMEP) in amyotrophic lateral sclerosis (ALS) patients. METHODS: To record the latency and amplitude of diaphragmatic compound muscle action potential (DCMAP) from 37 ALS patients and 31 control subjects with surface electrodes after transcranial and transcervical magnetic stimulation, central motor conduction time (CMCT) was calculated. The percentage of forced vital capacity (%FVC) was performed in 22 ALS patients simultaneously. RESULTS: Cortical and cervical latencies of the ALS patients were longer than those of the controls, and cortical and cervical amplitudes of the ALS patients were lower than those of the controls. CMCT of the ALS patients were longer than that of the controls. Cortical latency, cortical amplitude and CMCT were all correlated with the dysfunction of the pyramidal tract. Cortical latency and CMCT were also correlated with the clinical respiratory dysfunction, cervical latency was correlated with %FVC. CONCLUSIONS: CMCT, Cortical and cervical latencies are sensitive index in DMEP parameters reflecting respiratory dysfunction in ALS, CMCT reflects cortical spinal tract function of ALS patients associated with respiration. It would be helpful to detect the neurological basis of the respiratory disturbance in ALS combining CMCT with cervical latency.

Adolescent↗

[Autosomal dominant cortical cerebellar atrophy (ADCCA) linked to chromosome 16q].

Clinical features of and genetic approach identifying the gene for autosomal dominant cortical cerebellar atrophy linked to chromosome 16q (16q-ADCCA) are presented. The clinical characteristics of our 6 families with 16q-ADCCA were slowly progressive pure cerebellar syndrome with late age of onset (average +/- SD years: 55.9 +/- 10.0), which contrasts with more earlier onset, prominent sensory neuropathy and pyramidal tract signs seen in spinocerebellar ataxia type 4 (SCA4) linked to the same chromosomal region. To identify the gene responsible for 16q-ADCCA, we refined the previously mapped region and found that all of our 6 families share haplotype for markers lying in about 3-cM region in 16q31-q22, indicating that the founder effect is present in this disease. We next constructed a BAC contig spanning this 3-cM region. The maximum size of this contig was estimated 5 megabase (Mb). By searching public genome databases, we first examined for the presence for expansion of tandem repeat sequences in our patients. We found more than 50 tandem repeats including 8 CAG repeats. However, we have not detected any expansion in our patients so far. We are currently examining remaining repeat sequences for expansion and also several genes that appear potential candidates from their expression and functional profiles.

Cerebellar Ataxia↗

[Electrophysiologic effects of stimulating the vestibular nuclei on the sensomotor cortex of cats].

The evoked potentials in the anterior suprasylvian and posterior sigmoid gyri to the bulbar vestibular nuclei stimulation were recorded in relaxed anesthetized cats. Neuronal discharges of 70 units in the forelimb cortical zone in the posterior sigmoid gyrus were also recorded. 26 neurons responded to the vestibular nuclei stimulation, 6 of them were identified as cortico-rubral neurons. All of them were activated by the radial-nerve muscle branch stimulation. There was no neurons characterized by the muscle-vestibular convergence from the pyramidal tract neurons. The muscle-vestibular afferent system projection to the cortico-rubral neurons is supposed to play a major role in the tonic muscle coordination of the forelimbs.

Animals↗

[Infantile neuro-axonal dystrophy: anatomo clinical study of one case (author's transl)].

A case of Seitelberger's infantile neuroaxonal dystrophy (a rare familial neurologic disease of childhood) is described. The clinical picture is characterized by a progressive deterioration of psychomotor functions leading to flaccid paraplegia with hypotonia of axial muscles, complete involution of language, and total loss of communication with the external world; death due to recurrent unassociated disease occurred at the age of 4 years. Histology showed numerous axonal spheroids mainly in the gray matter of the C.N.S. and plurisystemic degenerations of the motor and sensory systems, of the cerebellum, of the basal ganglia, and of specific sensory system such as the optic and (reported here for the first time) of the olfactory and acoustic systems. In particular, the main histopathological findings included: 1) a characteristic distribution of axonal swellings prevailing in the posterior horn of the spinal cord and in the dorsolateral portions of the medulla oblongata, mainly at the level of the sensory nuclei; 2) demyelinization of the pyramidal tracts and of the ascendings pathways of the sensory system with fibrillar gliosis and myelin breakdown products in some areas (internal capsule, pes pedunculi, VPL thalamic nuclei); 3) severe cerebellar atrophy with almost complete loss of granule and Purkinje cells and marked fibrillary gliosis; 4) presence of enormous amount of sudanophilic lipids in the striatum and pallidum; 5) optic, acoustic and olfactory system degeneration with demyelinization and gliosis at all levels examined and, in particular, sudanophilic lipid deposition in the optic radiations, trigone, and olfactory striae. The discussion emphasized the dying-back type of evolution of the degenerative process insofar as a) the spheroids represent a peculiar alteration of presynaptic endings (as demonstrated by electron microscopy) prevailing at the first sensory neuron, and b) in all systems involved, the degeneration is most marked at distal levels. The striato-pallidal lipophanerosis suggests that the sudanophilic lipids are, here as in other systems, parenchymal degeneration products. On the other hand, there are still many unresolved problems in this rare and complex disease, such as a) the predilection of the lesions for the sensory systems which in our case involved all three special senses; b) the extreme cerebellar atrophy; and c) the etiopathogenetic substrate of the process. All biochemical and histochemical studies have not yielded any results up to the present.

Age Factors↗

The "bedsheet" Babinski.

BACKGROUND: Babinski's reflex (dorsiflexion of the hellus and fanning of the toes in response to plantar stimulation) is a classic sign in neurology. Although typically elicited by stroking the plantar surface of the foot with a firm object, other means of obtaining the response have been described. METHODS: We assessed the frequency of the Babinski response after plantar stimulation by exposing the feet by removal of the bedsheet or socks in 10 patients with neurologic disease and 10 control subjects. These results were compared with those obtained by traditional means of stimulation. RESULTS: We found a high correlation between the two responses (sensitivity of 80% and specificity of 90%). CONCLUSION: Although this study is small and requires a larger study to verify its accuracy, it appears that simple observation of the toe response at the time that the feet are exposed frequently provides evidence of pyramidal tract dysfunction.

Adult↗

[A case of slowly progressive unilateral spastic hemiparasis, hand clumsiness, limb ataxia and constructional apraxia].

A 65-year-old woman was seen in our clinic because of a 4-year history of slowly progressive left hand clumsiness and left limping. Neurologically, she had left-side spastic hemiparasis including her tongue, hand clumsiness, limb ataxia, constructional apraxia, and memory impairment. Dementia, parkinsonism and lower motor neuron sign were not confirmed. MRI study showed diffuse cerebral atrophy and shrinkage of the right cerebral peduncle and pontine base. SPECT (99mTc-ECD) study revealed hypoperfusion of the right fronto-parietal cortex. This is a rare case presented with slowly progressive left-side spastic hemiparasis, mimicking Mills' syndrome. In addition this case had other cortical signs, such as ipsilateral hand clumsiness, limb ataxia, constructional apraxia. Neuroimaging study suspects that the right front-parietal cortex is the primary lesion. Etiologically atypical motor neuron disease with adjacent cortical involvement is suspected rather than corticobasal degeneration with severe unilateral pyramidal tract degeneration.

Aged↗

Craniocervical tuberculosis: protocol of surgical management.

OBJECTIVE: Craniovertebral junction tuberculosis (CVJ-TB) is rare and occurs in only 0.3 to 1% of patients with tuberculous spondylitis. In the available literature, the treatment options offered for this entity have ranged from a purely conservative approach to radical surgery without well-defined guidelines. In this study, we attempt to establish the most effective strategy for the management of this condition. METHODS: Twenty-five patients with CVJ-TB were treated during the past 8 years. Severe neck pain, restricted neck movement, and myelopathy were the predominant symptoms. The patients were graded according to their disability as follows: Grade I (n = 7), only neck pain with no pyramidal tract involvement; Grade II (n = 8), independent with minor disability; Grade III (n = 1), partially dependent on others for assistance with activities of daily living; and Grade IV (n = 9), completely dependent on others for assistance with all activities of daily living. Nine patients in Grade IV also had severe respiratory compromise. In all patients, lateral radiographs of the CVJ in flexion and extension were used to determine the presence of atlantoaxial dislocation (AAD). Bony destruction, paraspinal abscess, and thecal compression were seen on intrathecal contrast computed tomographic scans (n = 9) and magnetic resonance imaging studies (n = 22). Under the cover of antituberculous therapy (ATT) administered for 18 months, the patients were placed under a management protocol that took into account the patient's preoperative grade, the presence of mobile or fixed AAD, bony destruction and retropharyngeal abscess formation at the CVJ, and the clinicoradiological response to ATT within 3 months. Thus, 14 patients were kept on conservative management, with their neck movements stabilized with an external orthosis; 4 patients underwent a single-stage transoral decompression and posterior fusion procedure; and 7 patients underwent direct posterior fusion. RESULTS: In a follow-up period that ranged from 6 months to 7 years (mean, 2.5 yr), the patients in Grades I and II maintained their neurological status. The single patient in Grade III improved to Grade II. Seven of the nine patients in Grade IV returned to normal, and one improved to Grade II. Neck pain improved in all patients. The only death in the series occurred as a result of aspiration pneumonitis leading to septicemia in a child in Grade IV with poor respiratory status and multilevel tuberculous involvement who had undergone transoral decompression and posterior fusion for fixed AAD. CONCLUSION: This study discusses the clinicoradiological presentation as well as the management of CVJ-TB, in which ATT is administered for 18 months. In the patients with minor deficits (Grades I and II), conservative neck stabilization is adopted; in the patients with severe deficits (Grades III and IV) due to significant cervicomedullary compression caused by fixed AAD or bone destruction and granulation, anterior decompression and posterior fusion are performed. Patients with persistent reducible AAD undergo direct posterior fusion. A significant improvement is possible even in poor-grade patients with judicious use of the surgical options and ATT.

Adolescent↗

[A case of portal-systemic shunt encephalopathy due to congenital portal vein hypoplasia presenting with abnormal cerebral white matter lesions on the MRI].

A 49-year-old woman, without any past history of liver diseases and blood transfusion, was admitted to our service because of somnolence, and flapping tremor. Neurologically, she was drowsy and disoriented. She had bilateral pyramidal tract signs and flapping tremor. Although the laboratory examination showed marked hyperammonemia (217 micrograms/dl), neither abdominal CT nor liver biopsy showed any evidence of liver cirrhosis. An abdominal angiography showed portal vein hypoplasia associated with the portal-systemic shunt. A T2-weighted MRI showed the high intensity areas in the bilateral deep cerebral white matter, and the posterior limbs of the bilateral internal capsules. This is a rare case of portal-systemic shunt encephalopathy due to congenital portal vein hypoplasia presenting with abnormal cerebral white matter lesions on the MRI.

Female↗

[A case of motor neuron disease with dementia and apraxia of the upper limbs].

We report a 61-year-old, right-handed woman with motor neuron disease, dementia, and apraxia of the upper limbs. The patient developed clumsiness of the right hand and dysarthria two years and a half prior to admission. Neurological examination showed limb-kinetic apraxia and ideomotor apraxia, predominantly on the right side, in addition to dementia and anarthria. There was mild muscle wasting in the neck and hands. A muscle biopsy from the biceps muscle of arm as well as needle EMG revealed neurogenic changes compatible with motor neuron disease. Brain MRI indicated pyramidal tract degeneration. Three-dimensional brain perfusion imaging generated from SPECT demonstrated an asymmetric decrease in cerebral blood flow in the fronto-temporo-parietal regions, predominantly on the left side. This case suggests that asymmetric limb apraxia can be associated with motor neuron disease.

Apraxia, Ideomotor↗

[Medical treatment of spasticity].

Spasticity is one of the clinical signs observed after a lesion of the pyramidal tract. Clinical manifestations are polymorphous and depend on the location of the lesion on the pre-motoneuron. Functional consequences are also variable. Only negative effects such as painful spasms, stiffness, distortions, are to be treated. Three different categories of drugs are available: GABA-like (baclofen, benzodiazepine), central alpha 2 agonists (tizanidine, clonidine) and peripheral anti-spastics (dantrolene). Baclofen remains the most commonly used anti-spastic. The preferential indication is spasticity from spinal cord disease, especially when the aetiology is multiple sclerosis. Efficacy of benzodiazepines (diazepam, tetrazepam, clonazepam) is comparable with baclofen; however, side effects (drowsiness) are more frequent. Benzodiazepines are indicated when spasticity is associated with anxiety. Tizanidine is an efficient and well tolerated antispastic. In France, prescription requires a temporary authorization of use. Dantrolen has a peripheral mechanism of action and can be prescribed in the different forms of spasticity. There are other compounds with anti-spastic properties (gabapentine, cyproheptadine, piracetam). Their advantage is rather limited when used alone. Generally, they are administrated in combinaison with usual anti-spastic drugs.

Adrenergic alpha-Agonists↗

[Pathogenesis of main objective neurological symptoms in Chiari malformation type I].

To study the influence of Chiari malformation (CM) size, patient's age, hydrocephaly and intracranial hypertension on the disease manifestations, 263 patients with CM type I without concomitant syringomyelia have been examined. All the patients underwent clinical neurological examination, craniography and magnetic resonance imaging of the brain and spinal cord. Five main groups of objective neurological signs were identified: oculomotor (17.1%); cochlear vestibulocerebellar (84.8%); bulbar (35.4%); conduction motor (25.9%); segmental motor sensory disturbances (9.5%). The data obtained suggest that (1) a complex of pathogenic factors determines clinical manifestations of the disease in the given patient with CM type I; (2) CM size determines significantly the incidence and extent of the main objective neurological signs; (3) with age the seventy of cochlear and vestibulocerebellar disturbances increases; (4) hydrocephaly and intracranial hypertension deteriorate cochlear, vestibulocerebellar and bulbar disturbances as well as pyramidal-tract signs.

Adolescent↗

Mini-infarct encephalopathy associated with uncommon microvessel convolute formation presenting with presenile dementia.

A female patient started to suffer from transient ischemic attacks when she was 47 years of age, followed by increasing predominantly left-side spastic tetraparesis, generalized seizures and progressive dementia over a period of 11 years. She died when she was 58 years of age. On gross examination the brain showed enlarged ventricles and arteriosclerotic changes of large extracerebral vessels of the circulus arteriosus. Microscopic examination of the atrophic brain showed innumerable incomplete microinfarcts in the white and gray matter throughout all parts of the brain. In the white matter these lesions were characterized by small foci of demyelination and loss of oligodendrocytes while occasionally some scavenger cells were seen. Axons seemed to be unaffected or displayed irregular axonal regeneratory growth. Any inflammatory reaction failed. In the cerebral cortex and subcortical nuclei the lesions showed loss of neurons and decrease in synaptophysin expression. Intracerebral arteries showed fibrosis or fibrohyalinosis of the entire intracerebral small-vessel network. In addition, numerous uncommon clusters of angioma-like telangiectatic vessels were observed. Medium-sized ischemic infarcts were found in the right putamen and adjacent internal capsule region, left-side dorsolateral brain stem and cerebellar hemisphere as well as a left-side pyramidal tract degeneration. Contralateral pseudohypertrophy of the inferior olivary nucleus was seen. The clinical and the neuropathologic observations made in this patient are compatible with small vessel disease characterized by a multicentric special and not yet described type of incomplete mini-infarcts in cerebral cortex and white matter accompanied by some larger ischemic infarcts of the common type in brain stem and cerebellum.

Alzheimer Disease↗

[Pathological aspects of post-stroke muscle tone disorder: clinical-neuro-visual study].

We studied how different patterns of muscle tone disorders depend upon lesion site using CT. 111 patients not earlier 3 months after hemispheric stroke were studied. We showed the degree of spasticity to be determined by depth of hemispheric damage in relation to posterior limb of capsula intema. It also correlates with the degree of corticoreticular pathway damage. Even or irregular damage to this pathway which accompany pyramidal tract stipulates different patterns of spasticity, distribution and its interaction with structure of hemiparesis. Degree and structure of post-stroke muscle tone disorders after 3 months from the onset of stroke is depend on hemispheric damage but do not depend on the duration of recreative or residual period of the disease. It can be applied in medical expertise that pattern and prominence of post-stroke muscle tone disorders 3 months after stroke onset depend on lesion location, but not on the length of recovery period or period of residual changes.

Brain↗

[Anatomical and clinical correlations in the cerebellar eredodegeneration].

Spinocerebellar hereditary degeneration makes up a heterogeneous group of diseases headed by Strumpell-Lorrain syndrome and Friedreich's disease. They are a heterogeneous group characterized by spasticity and paraplegia and related to demyelinization of the pyramidal tract and of the posterior cordons. During a 4-year period, we studied 14 patients (42-61 years old) suffering cerebellar eredodegeneration (hereditary ataxia). The aim of our work was to correlate anatomopathological findings with clinical signs. The important role played by the cerebellum in vesicosphincterial coordination was shown; in particular severe alteration of the ponto-cerebellar bundles could be cause of the abnormal behaviour of the detrusor.

Adult↗

[Current neurophysiological tests and revised JSCN technical standards for clinical EEG].

The purpose of this lecture is to review the development of current neurophysiology and the revised standard of society for clinical EEG. 1. The improvement of neurophysiological tests. 1) EEG and evoked potential: EEG and evoked potential testing includes the routine EEG recording, EEG monitoring in surgical operation, all night sleep polygraph for the diagnosis of sleep apnea syndrome and many kinds of brain evoked potentials. Especially, the P300 component in the ERP(event-related evoked potential) is useful for the testing of essential brain functions. 2) EMG and evoked EMG: These tests are applied for the diagnosis of neurogenic, myogenic and neuromuscular junction disorder, and also the single fiber EMG using micro needle electrode is useful for the diagnosis of myasthenia gravis. Motor and sensory nerve conduction velocity are calculated from the latency of evoked EMGs. Furthermore, the distribution of these conduction velocities in many nerve fibers is measured by the collision technique. 3) Other tests: Near-infrared spectroscopy for the testing of brain functions has made rapid progress, and the transcranial magnetic stimulation method has come to be used for evaluation of functional diseases in the pyramidal tract, cerebellum and the spinal cord. 2. The revised JSCN technical standards for clinical EEG. The revised recording conditions of ECI(electro cerebral inactivity: flat EEG) in brain death are the focus of this lecture.

Brain↗