OBJECTIVE: To investigate whether movement-related cortical potentials (MRCP) provide a physiological correlate that indicates the response to treatment in patients with writer's cramp. METHODS: In 21 patients with writer's cramp, who underwent 4 weeks of limb immobilization followed by re-training for 8 weeks, we recorded MRCPs preceding a self-initiated brisk ...

OBJECTIVE: Erectile dysfunction (ED) following radical prostatectomy is of major concern for both patients and caring physicians. We evaluated the bulbocavernosus reflex latency (BCRL) and amplitude (BCRA) following bilateral nerve-sparing radical retropubic prostatectomy (NS-RRP) to predict the response to sildenafil citrate (SC). METHODS: Patients were recruited in our ED clinic ...

BACKGROUND AND PURPOSE: Sialidosis type 1 (ST-1) is a neurodegenerative disorder with limited long-term follow-up report. This study is to document the chronological profile of ST-1. METHODS: We perform serial analysis of 17 Taiwanese patients with ST-1 focusing on evolution of clinical features, electrophysiological findings, genetic studies, and neuroimage examinations. ...

BACKGROUND AND PURPOSE: Essential tremor (ET) is a slowly progressive disorder characterized by postural and kinetic tremors most commonly affecting the forearms and hands. Several lines of evidence from physiologic and neuroimaging studies point toward a major role of the cerebellum in this disease. Recently, voxel-based morphometry (VBM) has been ...

BACKGROUND AND PURPOSE: The 'hot cross bun' sign (HCBS), typically seen in the patients with multiple system atrophy, refers to a cruciform hyperintensity in the pons on T2-weighted MRI. Little is known about its pathological basis and prevalence in other degenerative cerebellar diseases and healthy population. We investigate the frequency ...

BACKGROUND: We wanted to know whether trapezius motor evoked potentials (MEPs) are helpful in the evaluation of corticospinal (CS) lesions above the fifth cervical segment (C5) and compared trapezius MEP measurements in patients with and without radiological evidence of CS lesions. METHODS: Trapezius MEPs were routinely recorded in all MEP ...

BACKGROUND: dysarthrias are commonly found in patients with spinocerebellar ataxias (SCA). AIM: to characterize perceptual and acoustic features of speech in three patients with spinocerebellar ataxia and to verify the presence of similar features among these patients. METHOD: speech samples of two males with SCA3 and one male with SCA2 ...

Twenty Parkinson's disease (PD) patients (mean age 69.9 years) and 24 normal individuals' (mean age 63.8) both ears were investigated by brainstem auditory evoked potentials (BAEPs) and pure tone audiometry (PTA). There were no statistically significant age differences between the patients and control subjects. PTA results were significantly elevated for ...

TOPIC: Olfactory dysfunction may be assessed in the clinic with psychophysical testing and electrophysiological recording. Chemosensory event-related potentials (CSERPs) constitute an objective method to assess chemosensory function. Olfactory and trigeminal stimuli activate chemoreceptors from the olfactory neuroepithelium and from the nasal mucosa to evoke an electrophysiological response respectively called olfactory ...

INTRODUCTION: It is unclear whether the rate of stimulation for somatosensory evoked potentials (SEPs) can influence the presence or absence of cortical responses to median nerve stimulation in comatose patients. If so, this could affect how SEPs are performed and interpreted for prognostication in coma. Our objective was to determine ...

AIMS: To monitor acute brain injury in the neurological intensive care unit (NICU), we used EEG and somatosensory evoked potentials (SEP) in combination to achieve more accuracy in detecting brain function deterioration. METHODS: Sixty-eight patients (head trauma and intracranial hemorrhage; GCS<9) were monitored with continuous EEG-SEP and intracranial pressure monitoring ...

Studies examined the neurological involvement of ankylosing spondylitis (AS) are limited. This study aimed to assess the frequency of myelopathy, radiculopathy and myopathy in AS correlating them to the clinical, radiological and laboratory parameters. Included were 24 patients with AS. Axial status was assessed using bath ankylosing spondylitis metrology index ...

The aim of the present study was to determine the sensitivity and the profile of motor evoked potentials (MEP) in patients with clinically isolated syndrome (CIS) suggestive of multiple sclerosis (MS). We measured the central motor conduction time (CMCT), amplitude ratio (AR), and surface ratio (SR) in tibialis anterior and ...

Ataxia with oculo-motor apraxia type 2 (AOA2) is a recently described autosomal recessive cerebellar ataxia (ARCA) caused by mutations in the senataxin gene (SETX). We analysed the phenotypic spectrum of 19 AOA2 patients with mutations in SETX, which seems to be the third most frequent form of ARCA in Algeria ...

We report an unusual case of sporadic adult onset cerebellar ataxia with hypogonadism. A 40-year-old unmarried man presented with progressive ataxia and dysarthria along with complaints of non-development of secondary sexual characteristics and erectile dysfunction. There were complaints of intermittent diarrhea. Clinical examination revealed a pan-cerebellar syndrome with features of ...

We report an unusual case of sporadic adult onset cerebellar ataxia with hypogonadism. A 40-year-old unmarried man presented with progressive ataxia and dysarthria along with complaints of non-development of secondary sexual characteristics and erectile dysfunction. There were complaints of intermittent diarrhea. Clinical examination revealed a pan-cerebellar syndrome with features of ...

Viral hepatitis is a major health problem worldwide, the principal cause of cirrhosis and hepatocarcinoma. Once cirrhosis occurs, the consequences of liver dysfunction and portal hypertension become evident and, sometimes, life threatening for patients. Among the various complications of liver cirrhosis, the alteration of haemostatic balance is often a hard ...

The early recognition of comatose patients with a hopeless prognosis--regardless of how aggressively they are managed--is of utmost importance. Median somatosensory evoked potentials (SSEP) supplement and enhance neurological examination findings in anoxic-ischemic coma and are useful as an early guide in predicting outcome. The key finding is that bilateral absence ...

Recent studies have suggested that there may be functional and structural changes in the cerebellum of patients with adult onset primary focal dystonia. The aim of this study was to establish whether there is any neurophysiological indicator of abnormal cerebellar function, using the classic eyeblink conditioning paradigm. This paradigm at ...

OBJECTIVE: The aim of the present study is to investigate cortical excitability in patients with DYT 11 positive Myoclonus-Dystonia (M-D), using transcranial magnetic stimulation (TMS). METHODS: Silent period, motor evoked potential (MEP) recruitment curve, short interval intracortical inhibition (SICI), intracortical facilitation (ICF) and short interval intracortical facilitation (SICF), with short ...

BACKGROUND: Both balance control dysfunction and dysfunction of the central nervous system have been proposed as being causative factors in adolescent idiopathic scoliosis (AIS), yet the relationship between these factors has not been investigated in detail. An intergroup comparative study was conducted to investigate the effect of abnormal somatosensory function ...

Previous studies implicated potential value of mismatch negativity (MMN) in predicting recovery of consciousness in patients with disorders of consciousness (DOC). We have adopted a novel MMN evoked by subject's own name (SON), a self-referential stimulus thought to be powerful in evoking residual brain activity, and examined the correlation between ...

Three unrelated Japanese patients who presented with ataxia and mild mental retardation were examined in this study. Early development was normal in two patients and slightly delayed in one. All could walk independently, but were unstable due to cerebellar ataxia. They had mild intellectual retardation and displayed slow, progressive, and ...

This study evaluates the clinical spectrum of central post-stroke pain (CPSP) and correlates it with magnetic resonance imaging (MRI) and somatosensory-evoked potential (SEP) changes. Thirty-one consecutive CPSP patients whose median age was 51 years were evaluated and subjected to quantitative sensory testing and median and tibial SEPs. Cranial MRI abnormalities ...

To investigate whether visual evoked potentials (VEPs) are valuable for distinguishing between brucellosis with or without neurological involvement. A total of 23 patients who were admitted to the Department of Infectious Diseases and Microbiology, and Neurology, Ministry of Health Ankara Education and Research Hospital, Ankara, Turkey between December 2004 and ...

Though there are few studies addressing brainstem auditory evoked potentials (BAEP) in patients with chronic obstructive pulmonary disease (COPD), subclinical BAEP abnormalities in stable COPD patients have not been studied. The present study aimed to evaluate the BAEP abnormalities in this study group. MATERIALS AND METHODS: In the present study, ...

Behcet's disease, a multisystemic vascular inflammatory disorder of unknown origin, is relatively rare and central nervous system involvement is seen in 5% of affected individuals. This form of the disease, called as neurobehcet's disease (NB), can be misdiagnosed as multiple sclerosis (MS), a demyelinating disorder of central nervous system, so ...

Evidence suggests that sensory loss may occur in a proportion of patients affected by poliomyelitis. We hypothesize that sensory problems may be a lasting sequela in some polio survivors. Sensory pathways in polio survivors were evaluated clinically and electrophysiologically using sensory evoked potentials (SEPs). Patients with sensory deficits or abnormal ...

OBJECTIVE: There are mutual interactions between the olfactory and trigeminal systems. The purpose of this study was (1) to assess trigeminal sensitivity using chemosensory event-related potentials (CSERPs) in patients with olfactory dysfunction and (2) to evaluate whether trigeminal ERPs were different with regard to the presence or absence of olfactory ...

BACKGROUND: The principal features of the cerebellar infarcts are ataxia, failure of coordination, gait instability, and articulation and eye movement disabilities. Language disabilities are also seen with cerebellar lesions, but there are difficulties in diagnosis. This study was planned to evaluate the effects of cerebellar lesions on language functions and ...

OBJECTIVE: To assess patterns of novelty P3 elicited by the subject's own name (SON) in comatose patients and to compare SON novelty P3 prognostic value with that of mismatch negativity (MMN). METHODS: A passive oddball paradigm, previously validated in healthy subjects, including duration deviants and SON presented as a novel ...

OBJECTIVE: To evaluate the usefulness of functional measures in patients with spinocerebellar ataxia (SCA). METHODS: We assessed three functional measures-8 m walking time (8MW), 9-hole peg test (9HPT), and PATA repetition rate-in 412 patients with autosomal dominant SCA (genotypes 1, 2, 3, and 6) in a multicenter trial. RESULTS: While ...

In the present study, eye movements are recorded in two patient groups with an autosomal dominantly inherited cerebellar disorder, i.e. spinocerebellar ataxia type 6 (SCA6) and familial cortical myoclonic tremor with epilepsy (FCMTE). In SCA6 and FCMTE patients striking similarities with the extensive Purkinje cell changes in the cerebellar cortex ...

Falls pose an important problem to neurologists caring for patients with cerebellar disorders. Normal human gait is characterized by prominent up-and-down linear head movements (vertical translations). Thus, we asked whether patients with cerebellar gait ataxia showed abnormal responses of otolithic vestibuloocular reflexes to this motion. Compared with healthy subjects, all ...

BACKGROUND: Ataxia with oculomotor apraxia type 2 (AOA2) is an autosomal recessive disease caused by SETX mutations in 9q34 resulting in cerebellar ataxia in association with peripheral neuropathy, cerebellar atrophy on imaging, an elevated alpha-fetoprotein (AFP) serum level, and occasional oculomotor apraxia. OBJECTIVE: To describe the clinical and molecular findings ...

The authors describe a large Iranian family with autosomal dominant cerebellar ataxia, which included 14 patients in four generations. We examined seven patients who had expanded CAG repeats in the CACNA1A gene with repeat instability (24 and 25 repeats). Although all patients showed cerebellar ataxia, each patient exhibited peripheral neuropathy ...

Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease that results from the expansion of an unstable trinucleotide CAG repeat encoding for a polyglutamine tract. In normal individuals, alleles contain between 14 and 31 CAG repeats, whereas the pathological alleles have more than 35 CAG repeats. The clinical ...

INTRODUCTION: Copper deficiency is an increasingly recognised cause of neurological impairment. This retrospective review highlights clinical and electrodiagnostic findings in patients diagnosed at our institution with copper deficiency. METHODS: Clinical, radiographic and electrodiagnostic findings were reviewed in patients with evidence of copper deficiency. Patients with other potential causes of myelopathy ...

Human parvovirus B19 generally causes erythema infectiosum in childhood, but it can be associated with unusual findings, particularly in immunocompromised patients. This is a report about an immunocompetent 4-year-old female child affected with acute encephalitis by parvovirus B19, documented by polymerase chain reaction performed on cerebrospinal fluid, who was treated ...

OBJECTIVES: We investigated whether visual field defects can be objectively evaluated using multifocal visual evoked potentials (mfVEPs) in patients with intracranial disease complicated by mental disorders. PATIENTS AND METHODS: First, to determine the normal pattern of mfVEPs, recordings were performed using a VERIS Junior Science recording apparatus (Mayo, Aichi, Japan) ...

OBJECTIVE: To assess the impact of the disease stage and therapy on motor cortical excitability in Parkinson's disease (PD). METHODS: Twenty newly diagnosed and medication-free, early stage patients, 20 late stage patients under antiparkinsonian therapy and 20 normal healthy controls were included. Motor threshold (MT), amplitudes of motor evoked potential ...

To determine the adaptive capability of saccadic eye movements, and its association with enduring saccadic dysmetria in cerebellar and lateral medullary infarction (LMI), we investigated saccadic accuracy and adaptation in 15 patients with cerebellar or lateral medullary infarction, compared with those of 7 patients with diffuse cerebellar atrophy and 11 ...

BACKGROUND: Rectoanal inhibitory reflex is not always evident in patients with chagasic megacolon. This may be due to insufficient volumes of air used during insufflation for the manometric examination. AIMS: To identify the volume of air necessary to induce rectoanal inhibitory reflex in patients with chagasic megacolon and to observe ...

OBJECTIVE: To assess the pain-relieving effects of motor cortex electrical stimulation (MCS) within the central sulcus and the predictive factors retrospectively. METHODS: Thirty-four patients with intractable neuropathic pain underwent MCS; 19 patients had cerebral lesions, and 15 had non-cerebral lesions. In selected 12 patients, test electrodes were implanted within the ...

A reliable objective test is still needed to confirm the diagnosis of neurogenic thoracic outlet syndrome (NTOS). Over the past 20 years, it has been suggested that responses to medial antebrachial cutaneous nerve (MAC) and C8 nerve root stimulation could be used for this purpose. Herein, we explore this thesis. ...

OBJECTIVE: To evaluate cognitive function in B12 deficiency neurological syndromes and response to B12 therapy. METHODS: Patients were diagnosed on the basis of low serum B12 or megaloblastic bone marrow or both. Detailed neurological examination was performed and mental status was evaluated by the Mini Mental State Examination (MMSE). Hemoglobin, ...

This report presents studies of the neurophysiological correlates of the characteristics of cognitive disorders in patients with first psychotic episodes of endogenous psychosis at juvenile age. Three groups of patients were studied: those with a predominance of catatonic symptomatology (22 patients), those with a predominance of hallucinatory-delusional symptomatology (22 patients), ...

OBJECTIVE: To assess corticospinal tract involvement in patients with amyotrophic lateral sclerosis (ALS) by correlating diffusion tensor imaging (DTI) measures with intra- and extracranial central motor conduction time (CMCT) and clinical features of the patients. METHODS: We investigated 31 patients with ALS and 31 normal volunteers by DTI and measured ...

Patients with progressive supranuclear palsy (PSP) fall frequently, beginning early in the course of their disease. Abnormal vestibulospinal reflexes are suspected, but the angular vestibulo-ocular reflex, which is mediated by the labyrinthine semicircular canals, survives late into the course of the disease. To test the hypothesis that otolithic-mediated reflexes are ...

Auditory brainstem responses (ABR), ipsilateral and contralateral acoustic reflexes and the masking level difference for speech (MLD) were studied in 29 patients with adrenomyeloneuropathy (AMN). Abnormalities were seen for all ABR components with Waves V and III affected to the greatest degree. For male patients with AMN, the I-III, III-V ...