共查询到20条相似文献，搜索用时 31 毫秒
1.
Laura Pellegrinelli S. Gambara R. Micheli S. Binda E. Fazzi E. Pariani 《Journal of neurovirology》2018,24(5):656-659
A previously healthy 6-year-old boy was admitted to hospital with hypotonia and hyposthenia of lower limbs. Electromyography and slow motor nerve conduction velocity test identified a lower limb acute motor axonal neuropathy. Brain and spinal cord magnetic resonance imaging demonstrated multifocal cortical gray matter lesions in both cerebral hemispheres consistent with gray matter acute disseminated encephalitis otherwise with viral/Mycoplasma pneumoniae encephalitis, and signs of involvement of anterior nerve roots of the cauda equina consistent with Guillain-Barré syndrome. The patient resulted negative to routinely bacterial and viral investigations but positive to human parechovirus that sequence analyses confirmed as type 6. Intravenous immunoglobulins and methylprednisolone treatment were administered but did not relieve the symptoms of Guillain-Barré syndrome. The disease improved gradually over the next 3-month follow-up with a complete remission of both central and peripheral nervous system symptoms. 相似文献
2.
Mamede de Carvalho Artiom Poliakov Cristiano Tavares Michael Swash 《Clinical neurophysiology》2017,128(11):2200-2204
Objective
We studied motor unit recruitment to test a new method to identify motor unit firing rate (FR) variability.Methods
We studied 68 ALS patients, with and without upper neuron signs (UMN) in lower limbs, 24 patients with primary lateral sclerosis (PLS), 13 patients with spinal cord lesion and 39 normal subjects. All recordings were made from tibialis anterior muscles of normal strength. Subjects performed a very slight contraction in order to activate 2 motor units in each recording. 5–7 motor unit pairs were recorded in each subject. Mean consecutive differences (MCD) were calculated for each pair of potentials. The mean MCD for each muscle was estimated as the mean from the total number of pairs recorded. A p value < 0.01 was accepted as significant.Results
MCD of FR frequency was less in the subjects with spinal cord lesion and PLS. In addition, the FR frequency of the 1st motor unit in a pair of units was markedly reduced in PLS, and in subjects with spinal cord lesions.Conclusion
These results support a lower threshold and reduced FR fluctuation in spinal motor neurons of spastic patients.Significance
This method can be developed for detection of UMN lesions. 相似文献3.
Akihisa Okumura Harushi Mori Pin Fee Chong Ryutaro Kira Hiroyuki Torisu Sawa Yasumoto Hiroyuki Shimizu Tsuguto Fujimoto Keiko Tanaka-Taya 《Brain & development》2019,41(5):443-451
Objecive
To clarify the neuroimaging findings of children with acute flaccid myelitis during an outbreak of EV-D68 infection.Methods
We performed a detailed review of the spinal and cranial MRI results of 54 children with acute flaccid myelitis. We focused on the range of longitudinal lesions, the localization and appearance of lesions within a horizontal section, Gadolinium-enhancement, and changes over time.Results
All children had longitudinal spinal lesions involving central gray matter. Twenty-six children had lesions spanning the entire spine. Six of them had weakness in all limbs, whereas seven had weakness of only one limb. Thirty-eight children had lesions in both gray and white matter and limb weakness tended to be more severe in these children. During the acute period, spinal lesions showed bilateral ill-defined widespread T2 hyperintensity. During the subacute period, lesions were well defined and confined to the anterior horn. The distribution of limb weakness was correlated with the appearance of lesions during the subacute period. Gadolinium enhancement was performed in 37 children, and enhancement was seen in the cauda equina in 29 children. Enhancement was infrequent within 2?days after onset but was seen in almost all children thereafter. Twenty-two children had brainstem lesions continuous with spinal lesions.Conclusion
Extensive longitudinal spinal lesions were characteristic in children with acute flaccid myelitis. Lesions were usually bilateral and widespread during the acute period, whereas localization to the anterior horn could become obvious. Although enhancement of the cauda equina was often observed, its appearance was sometimes delayed. 相似文献4.
Chaojun Zheng Yu Zhu Cong Nie Feizhou Lu Dongqing Zhu Robert Weber Jianyuan Jiang 《Clinical neurophysiology》2018,129(7):1383-1389
Objective
To investigate the changes in motor axonal excitability properties in cervical spondylotic amyotrophy (CSA).Methods
Threshold tracking was used to measure the median motor axons in 21 patients with CSA, 10 patients with cervical spondylotic radiculopathy (CSR) and 16 normal controls.Results
Compared with normal controls, patients with distal-type CSA showed increased threshold electrotonus hyperpolarization (TEh [90–100]) and increased superexcitability on the symptomatic side (P?<?0.05), which are suggestive of distal motor axonal hyperpolarization, presumably due to motor axonal regeneration. More importantly, compared with normal controls and CSR cases, both distal- and proximal-type CSA cases showed lower accommodation during depolarising currents (reduced S2 accommodation, decreased TEd [undershoot] and/or lower subexcitability) (P?<?0.05), indicating that slow K+ conductance may be less active in motor axons in patients with CSA.Conclusions
The present study demonstrated changes in motor axonal excitability in patients with CSA compared with both normal controls and patients with CSR.Significance
Less expression of slow K+ conductance may confer greater instability in membrane potential in CSA, thereby presumably contributing to the increased vulnerability of motor axons in patients with CSA. 相似文献5.
Jun-Soon Kim Myoung-jin Jang Seung-Jae Hyun Ki-Jeong Kim Tae-Ahn Jahng Hyun-Jib Kim Sung-Min Kim Kyung Seok Park 《Clinical neurophysiology》2018,129(11):2276-2283
Objective
To identify factors associated with the failure to generate baseline muscle motor evoked potentials (mMEPs) during spinal surgery, and to determine the association between baseline mMEP generation and postoperative outcomes.Methods
A total of 345 patients who underwent spine surgery with intraoperative mMEP monitoring were included, and we retrospectively reviewed their demographic/clinical parameters, and mMEP recording results according to lesion locations.Results
Multivariable logistic regression analysis revealed that preoperative Medical Research Council grade of the weakest muscle <3 was significantly associated with failure of baseline mMEP generation in both cervical and thoracic lesions. In addition, high intramedullary T2 signal intensity on spine MRI for cervical lesions and male sex for thoracic lesions were also significantly associated with baseline mMEP generation failure. Moreover, the failure of baseline mMEP generation was a significantly associated factor for poor functional outcome in patients with thoracic lesions.Conclusion
Sex, radiological abnormality, and preoperative functional status were associated with baseline mMEP generation failure during spine surgery with different patterns according to lesion location. Moreover, baseline mMEP generation failure in thoracic lesion could be associated with risk of postoperative deficits.Significance
The risk of baseline mMEP recording failure could be evaluated based on preoperative clinical parameters. 相似文献6.
José Manuel Matamala James Howells Thanuja Dharmadasa William Huynh Susanna B. Park David Burke Matthew C. Kiernan 《Clinical neurophysiology》2018,129(7):1472-1478
Objective
To evaluate the excitability of sensory axons in patients with amyotrophic lateral sclerosis (ALS).Methods
Comprehensive sensory nerve excitability studies were prospectively performed on 28 sporadic ALS patients, compared to age-matched controls. Sensory nerve action potentials were recorded from digit 2 following median nerve stimulation at the wrist. Disease severity was measured using motor unit number estimation (MUNE), the revised ALS Functional Rating Scale (ALSFRS-R) and the MRC scale.Results
There were no significant differences in standard and extended measures of nerve excitability between ALS patients and controls. These unchanged excitability measures included accommodation to long-lasting hyperpolarization and the threshold changes after two supramaximal stimuli during the recovery cycle. Excitability parameters did not correlate with MUNE, ALSFRS-R, APB MRC scale or disease duration.Conclusions
This cross-sectional study has identified normal axonal membrane properties in myelinated sensory axons of ALS patients. Previously described sensory abnormalities could be the result of axonal fallout, possibly due to a ganglionopathy, or to involvement of central sensory pathways rostral to gracile and cuneate nuclei.Significance
These results demonstrate the absence of generalized dysfunction of the membrane properties of sensory axons in ALS in the face of substantial deficits in motor function. 相似文献7.
M. Dennis D. Jewell R. Hetherington B. J. Spiegler D. L. MacGregor J. M. Drake R. P. Humphreys F. Gentili 《Child's nervous system》2009,25(11):1447-1453
Objective
The objective of the study was to measure upper limb motor function in young adults with spina bifida meningomyelocele (SBM) and typically developing age peers.Method
Participants were 26 young adults with SBM, with a Verbal or Performance IQ score of at least 70 on the Wechsler scales, and 27 age- and gender-matched controls. Four upper limb motor function tasks were performed under four different visual and cognitive challenge conditions. Motor independence was assessed by questionnaire.Results
Fewer SBM than control participants obtained perfect posture and rebound scores. The SBM group performed less accurately and was more disrupted by cognitive challenge than controls on limb dysmetria tasks. The SBM group was slower than controls on the diadochokinesis task. Adaptive motor independence was related to one upper limb motor task, arm posture, and upper rather than lower spinal lesions were associated with less motor independence.Conclusions
Young adults with SBM have significant limitations in upper limb function and are more disrupted by some challenges while performing upper limb motor tasks. Within the group of young adults with SBM, upper spinal lesions compromise motor independence more than lower spinal lesions.8.
Mariana Pereira Sofia Rita Fernandes Pedro C. Miranda Mamede de Carvalho 《Clinical neurophysiology》2018,129(9):1999-2009
Objective
Trans-spinal direct current stimulation (tsDCS) is a promising technique to modulate spinal circuits. Combining clinical with modelling studies can improve effectiveness of tsDCS protocols. The aim of this study is to measure the effects of lumbar tsDCS on motor spinal responses and observe if these are consistent with the electric field (E-field) predicted from a computational model.Methods
The exploratory study design was double-blind crossover and randomized. tsDCS was delivered for 15?min (anodal, cathodal, sham) at L2 vertebra level (2.5?mA, 90?C/cm2) in 14 healthy subjects. F-wave, H-reflex, cortical silent period, motor evoked potential and sympathetic skin response were analyzed. Statistical methods were applied with Bonferroni correction for multiple comparisons, a p?<?0.05 was set as significant. A human volume conductor model was obtained from available databases. E-field distributions in the spinal grey matter (GM) and white matter (WM) were calculated.Results
No tsDCS effects were observed. E-field magnitude predicted in the lumbosacral spinal GM and WM was <0.15?V/m, insufficient to ensure neuromodulation, which is consistent with the absence of effects.Conclusion
The tsDCS protocol applied did not change motor response to delivered stimulus, thus we observed no effect on motor spinal circuits.Significance
Future tsDCS protocols should be supported by computational models. 相似文献9.
Joachim Bas Emilien Delmont Farzad Fatehi Emmanuelle Salort-Campana Annie Verschueren Jean Pouget Marie-Noëlle Lefebvre Aude-Marie Grapperon Shahram Attarian 《Clinical neurophysiology》2018,129(7):1390-1396
Objective
The aim of this study was to assess the usefulness of motor unit number index (MUNIX) technique in Charcot-Marie-Tooth disease and test the correlation between MUNIX and clinical impairment.Methods
MUNIX technique was performed in the abductor pollicis brevis (APB), the abductor digiti minimi (ADM) and the tibialis anterior (TA) muscles in the nondominant side. A MUNIX sum score was calculated by adding the MUNIX of these 3 muscles. Muscle strength was measured using the MRC (medical research council) scale. Disability was evaluated using several functional scales, including CMT neuropathy score version 2 (CMTNSv2) and overall neuropathy limitation scale (ONLS).Results
A total of 56 CMT patients were enrolled. The MUNIX scores of the ADM, APB and TA muscles correlated with the MRC score of the corresponding muscle (p?<?0.01). The MUNIX sum score correlated with the clinical scales CMTNSv2 (r??=???0.65, p?<?0.01) and ONLS (r??=???0.57, p?<?0.01).Conclusion
MUNIX correlates with muscle strength and clinical measurements of disability in patients with CMT disease.Significance
The MUNIX technique evaluates motor axonal loss and correlates with disability. The MUNIX sum score may be a useful outcome measure of disease progression in CMT. 相似文献10.
Thomas Schüller Theo O.J. Gruendler René Huster Juan Carlos Baldermann Daniel Huys Markus Ullsperger Jens Kuhn 《Clinical neurophysiology》2018,129(9):1866-1872
Objective
Whether motor inhibition capabilities are impaired in Tourette’s syndrome (TS) remains inconclusive. The ability to suppress tics has been proposed to ensure normal motor control in uncomplicated, adult patients. The aim of the present study was to characterize cortical processes of motor inhibition and performance monitoring using event-related potentials (ERPs) elicited by a visual stop signal task.Methods
15 TS patients and 15 matched healthy controls performed a stop signal task while multi-channel EEG were recorded.Results
The behavioral results revealed no significant differences in inhibitory capabilities between groups. The latency of the P3 was discriminative of inhibition success, with shorter latencies for successful inhibition in both groups. P3 amplitude was not altered by inhibition success, but significantly attenuated for TS patients. Furthermore, the amplitude of the error-related negativity (ERN) was elevated while the error positivity (PE) was diminished for TS patients.Conclusion
In the stop signal task performance is not altered in adult TS patients but ERPs related to motor inhibition and performance monitoring are altered suggesting potential compensatory mechanisms.Significance
The results support the hypothesis of compensatory cortical mechanisms to ensure sufficient motor performance. 相似文献11.
Sara Yagüe Misericordia Veciana Carlos Casasnovas Montserrat Ruiz Jordi Pedro Josep Valls-Solé Aurora Pujol 《Clinical neurophysiology》2018,129(3):507-515
Objective
Patients with adrenomyeloneuropathy may have dysfunctions of visual, auditory, motor and somatosensory pathways. We thought on examining the nociceptive pathways by means of laser evoked potentials (LEPs), to obtain additional information on the pathophysiology of this condition.Methods
In 13 adrenomyeloneuropathic patients we examined LEPs to leg, arm and face stimulation. Normative data were obtained from 10 healthy subjects examined in the same experimental conditions. We also examined brainstem auditory evoked potentials (BAEPs), pattern reversal full-field visual evoked potentials (VEPs), motor evoked potentials (MEPs) and somatosensory evoked potentials (SEPs).Results
Upper and lower limb MEPs and SEPs, as well as BAEPs, were abnormal in all patients, while VEPs were abnormal in 3 of them (23.1%). LEPs revealed abnormalities to stimulation of the face in 4 patients (30.7%), the forearm in 4 patients (30.7%) and the leg in 10 patients (76.9%).Conclusions
The pathologic process of adrenomyeloneuropathy is characterized by a preferential involvement of auditory, motor and somatosensory tracts and less severely of the visual and nociceptive pathways. This non-inflammatory distal axonopathy preferably damages large myelinated spinal tracts but there is also partial involvement of small myelinated fibres.Significance
LEPs studies can provide relevant information about afferent pain pathways involvement in adrenomyeloneuropathic patients. 相似文献12.
Ingrid J.T. Herraets H. Stephan Goedee Johan A. Telleman Jan-Thies H. van Asseldonk Leo H. Visser W. Ludo van der Pol Leonard H. van den Berg 《Clinical neurophysiology》2018,129(1):232-237
Objective
Wartenberg’s migrant sensory neuritis (WMSN) is a rare, patchy, pure sensory neuropathy of unknown etiology. High-resolution ultrasonography (HRUS) is an emerging diagnostic technique for neuropathies, but it has not been applied in WMSN. In this study we aimed to determine HRUS abnormalities in WMSN.Methods
We performed a case-control study of 8 newly diagnosed patients with WMSN and 22 treatment-naive disease controls (16 patients with pure sensory axonal neuropathy and 6 with pure sensory chronic inflammatory demyelinating polyneuropathy (CIDP) or Lewis-Sumner syndrome (LSS)). All patients underwent routine diagnostic evaluations and a predefined HRUS protocol.Results
We found multifocal nerve enlargement in all 8 WMSN patients. The median nerve in the upper arm and the sural nerve were significantly larger in WMSN than in axonal controls (p?=?0.01 and p?=?0.04). In CIDP/LSS, sonographic enlargement was more extensive. Furthermore we found brachial plexus involvement in 3 of 8 (38%) WMSN patients.Conclusion
HRUS showed enlargement of multiple nerves in all WMSN patients even if clinical testing and NCS were normal.Significance
The feature of multifocal nerve enlargement may be of additional value in establishing the diagnosis of WMSN and may support the suggestion of an auto-immune etiology. 相似文献13.
Vykuntaraju K. Gowda Tamilarasan Udhayabanu Perumal Varalakshmi Varunvenkat M. Srinivasan Balasubramaniem Ashokkumar 《Brain & development》2018,40(7):582-586
Background
Fazio-Londe syndrome also called progressive bulbar palsy of childhood is a very rare motor neuron disease of pediatric age group characterized by progressive paralysis of lower cranial nerves.Objective
To describe Fazio-Londe syndrome in sibling with different phenotype.Methods
A 6?years old female child presented with inability to close eyes, difficulty in swallowing, respiratory muscle weakness and voice change since 5?yr of age. Examination showed lower motor neuron facial nerve palsy, absent gag reflex, tongue atrophy, fasciculation, limb wasting and exaggerated deep tendon reflexes. An 11?year old boy, elder sibling of the above child presented with similar complaints at 10?years of age, other than later onset and lack of respiratory problem. Genetic testing in both cases confirmed the diagnosis of Fazio-Londe Syndrome.Conclusion
In any child who presents with progressive bulbar palsy with lower motor neuron facial palsy a diagnosis of Fazio-Londe Syndrome should be considered and family members should also be screened. 相似文献14.
Kentaro Nakashima Yuhki Koga Yasunari Sakai Hidetoshi Takada Katsumi Harimaya Saiji Ohga Tomoaki Taguchi Yoshinao Oda Hiroshi Honda Shouichi Ohga 《Brain & development》2018,40(10):952-955
Background
Langerhans cell histiocytosis (LCH) is a clonal disease with focal or disseminated lesions that may compress the surrounding tissues, including the spinal cord. Because few reports have described the spinal symptoms as the first manifestation of pediatric LCH, the long-term neurological outcomes remain unclear.Case report and literature review
We report a 21-month-old boy who presented with sudden-onset paraplegia. Imaging analyses revealed that osteolytic lesions and epidural tumors compressing the spinal cord at the T7-9 vertebrae. Twelve days after he developed leg weakness, emergency radiotherapy was started after a tumor biopsy. During the course of radiotherapy, paralysis steadily ameliorated. After we excluded infections and determined the pathological diagnosis of LCH, multi-drug chemotherapy was started. Apparent improvement in his complete paraplegia was observed after a total 15?Gy of radiotherapy and subsequent chemotherapy, leaving no neurological sequelae at 4?years of age. Through a literature search of studies published from 1980 to 2017, we found that children with LCH showed a generally favorable recovery from neurological dysfunction after the acute phase of spinal symptoms.Conclusion
This report underscores the utility of emergency radiotherapy for the neurological recovery of spinal LCH in infants. Our long-term observation further denotes the value of this treatment in terms of the intact survival with preserved motor functions and physical growth. 相似文献15.
Antoine Gueguen Claude Jardel Marc Polivka S. Veronica Tan Françoise Gray Catherine Vignal Anne Lombès Olivier Gout Hugh Bostock 《Clinical neurophysiology》2017,128(7):1258-1263
Objective
To explore potential spreading to peripheral nerves of the mitochondrial dysfunction in chronic progressive external ophthalmoplegia (CPEO) by assessing axonal excitability.Methods
CPEO patients (n = 13) with large size deletion of mitochondrial DNA and matching healthy controls (n = 22) were included in a case-control study. Muscle strength was quantified using MRC sum-score and used to define two groups of patients: CPEO-weak and CPEO-normal (normal strength). Nerve excitability properties of median motor axons were assessed with the TROND protocol and changes interpreted with the aid of a model.Results
Alterations of nerve excitability strongly correlated with scores of muscle strength. CPEO-weak displayed abnormal nerve excitability compared to CPEO-normal and healthy controls, with increased superexcitability and responses to hyperpolarizing current. Modeling indicated that the CPEO-weak recordings were best explained by an increase in the ‘Barrett-Barrett’ conductance across the myelin sheath.Conclusion
CPEO patients with skeletal weakness presented sub-clinical nerve excitability changes, which were not consistent with axonal membrane depolarization, but suggested Schwann cell involvement.Significance
This study provides new insights into the spreading of large size deletion of mitochondrial DNA to Schwann cells in CPEO patients. 相似文献16.
G. Martino Y. Ivanenko M. Serrao A. Ranavolo F. Draicchio M. Rinaldi C. Casali F. Lacquaniti 《Clinical neurophysiology》2018,129(3):516-525
Objective
A comprehensive treatment of Hereditary Spastic Paraplegia (HSP) should consider the specific pathophysiological changes in the spinal cord. Here we reported a detailed characterization of the spinal motoneuronal output in HSP during locomotion.Methods
We recorded kinematics and electromyographic (EMG) activity of 12 leg muscles in 29 patients with pure forms of HSP and compared them with 30 controls while walking at matched speeds. We assessed the spinal locomotor output by evaluating EMG patterns and by mapping them onto the rostrocaudal location of the spinal motoneuron pools.Results
The activity profiles of muscles innervated from the sacral segments were significantly wider in patients. Similarly, spinal maps revealed a tendency for spreading the main loci of activation, involving initially the sacral segments and, at more severe stages, the lumbar segments.Conclusions
The degeneration of the corticospinal tract in HSP is associated with a widening of spinal locomotor output spreading from caudal to rostral segments.Significance
The findings highlight pathophysiologically relevant differential changes in the spinal locomotor output in HSP related to the specific innervation of muscles in the spinal cord, and might be helpful for developing future therapeutic strategies and identifying physiological markers of the disease. 相似文献17.
Christophoros Astaras Rita de Micheli Bianca Moura Thomas Hundsberger Andreas F. Hottinger 《Current neurology and neuroscience reports》2018,18(1):3
Purpose of Review
Immune checkpoint inhibitors represent a major step forward in the field of oncologic immunotherapy these last years and have significantly increased survival of cancer patients in an ever-growing number of indications. These agents block specific immune checkpoint molecules (programmed cell death protein 1 and its ligand as well as cytotoxic T-lymphocyte-associated antigen 4) that normally downregulate the immune response. These new agents show a specific range of adverse effects induced by abnormal immunologic activation.Recent Findings
Many different neurologic adverse events have been described, including encephalitis, myelopathy, aseptic meningitis, meningoradiculitis, Guillain-Barré-like syndrome, peripheral neuropathy (including mononeuropathy, mononeuritis multiplex, and polyneuropathy) as well as myasthenic syndrome.Summary
Immune checkpoint inhibitors have shown promising results in cancer but can possibly induce autoimmune disorders. Although rare, neurological adverse events require prompt recognition and treatment to avoid substantial morbidity.18.
Miguel Oliveira Santos Michael Swash Mamede de Carvalho 《Clinical neurophysiology》2017,128(4):643-646
Objective
To investigate the origin of ectopic activity in neuromyotonia (NMT).Methods
We studied two patients. In addition to routine studies, we tested synchronicity of spontaneous discharges in different motor units in simultaneous recordings made with two needle electrodes in the first dorsal interosseus muscle. Time-locked fasciculations in these double recordings would represent abnormal ectopic activity initiated in a nerve trunk with ephaptic stimulation of a nearby axon. In patient 1, this research protocol was applied once, 15 years after regular intravenous immunoglobulin (IvIg) treatment. Patient 2 was investigated before and 1 year after IvIg.Results
Both patients improved after IVIg, mirrored by a striking decrease in the amount of spontaneous activity on electromyography. Moreover, our technique did not detect synchronous spontaneous activity (time-locked fasciculations) on the second assessment, although this was predominant before treatment in patient 2.Conclusions
In NMT, abnormal discharges originate both in distal axonal branches and in more proximal segments. It appears that IvIg is more effective in blocking antibody activity in proximal axonal segments, perhaps related to factors such as blood-nerve barrier, temperature or differing ion channel distributions.Significance
Treatment effects can shed light on the origin of abnormal activity in NMT. 相似文献19.
Marisa Blanquet Jens A. Petersen Antonella Palla Dorothe Veraguth Konrad P. Weber Dominik Straumann Alexander A. Tarnutzer Hans H. Jung 《Clinical neurophysiology》2018,129(4):863-873
Objective
We aimed to quantify peripheral-vestibular deficits that may contribute to imbalanced stance/gait in patients with inflammatory neuropathies.Methods
Twenty-one patients (58?±?15?y [mean age?±?1SD]; chronic-inflammatory-demyelinating-polyneuropathy?=?10, Guillain-Barré Syndrome?=?5, Anti-MAG peripheral neuropathy?=?2, multifocal-motor-neuropathy?=?4) were compared with 26 healthy controls. All subjects received video-head-impulse testing (vHIT), caloric irrigation and cervical/ocular vestibular-evoked myogenic-potentials (VEMPs). The Yardley vertigo-symptom-scale (VSS) was used to rate vertigo/dizziness. Postural stability was assessed using the functional gait-assessment (FGA). Pure-tone audiograms (n?=?18), otoacoustic emissions (n?=?12) and auditory brainstem responses were obtained (n?=?12).Results
Semicircular-canal hypofunction was noted in 9/21 (43%) patients (vHIT?=?6; caloric irrigation?=?5), whereas otolith function was impaired in 12/21 (57%) (oVEMPs?=?8; cVEMPs?=?5), resulting in vestibular impairment of at least one sensor in 13/21 (62%). On average, 2.4?±?1.1 vestibular end organs (each side: anterior/posterior/horizontal canal, utriculus, sacculus; total?=?10) were affected. The VSS-scores were higher in patients (16.8?±?8.6 vs. 9.5?±?6.2, p?=?0.002) but did not correlate with the number of affected organs. Auditory neuropathy was found in 1/12 (8%) patients.Conclusion
Impairment of one or more vestibular end organs was frequent, but usually mild, possibly contributing to imbalance of stance/gait in inflammatory neuropathies.Significance
While our data does not support routine vestibular testing in inflammatory neuropathies, this may be considered in selected cases. 相似文献20.
Luis Velázquez-Pérez Roberto Rodríguez-Labrada Reidenis Torres-Vega Ricardo Ortega-Sánchez Jacqueline Medrano-Montero Rigoberto González-Piña Yaimeé Vázquez-Mojena Georg Auburger Ulf Ziemann 《Clinical neurophysiology》2018,129(5):895-900