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1.
Mult Scler Relat Disord ; 83: 105465, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38308913

RESUMO

In this study, it was aimed to detect ataxia in patients with Multiple Sclerosis (MS) by utilizing static plantar pressure data and capsule networks (CapsNet), one of the deep learning (DL) architectures. CapsNet is also equipped with a robust dynamic routing mechanism that determines the output of the next capsule. MS is a chronic nervous system disease that shows its effect in the central nervous system and manifests itself with attacks. One of the most common and challenging symptoms of MS is known as ataxia. Ataxia causes loss of control of limb muscle tone or gait disorders, leading to loss of balance and coordination. The diagnosis of ataxia in MS is applied employing the standard Expanded Disability Status Scale (EDSS) score. However, due to reasons such as physician misconception, diagnosis differences among physicians, and incorrect patient information, more unbiased solutions are required for the diagnosis. The results included Sensitivity at 96.34 % ± 1.71, Specificity at 98.11 % ± 2.04, Precision at 98.08 % ± 2.16, and Accuracy at 97.13 % ± 0.33. The main motivation of the study is to show that these deep learning methods can successfully detect ataxia in MS patients using static plantar pressure data. The high-performance measurements of sensitivity, specificity, precision and accuracy emphasize that the proposed system can be an effective tool in clinical practice. In addition, it was concluded that the proposed autonomous system would be a support mechanism to assist the physician in the detection of ataxia in patients with MS.


Assuntos
Ataxia Cerebelar , Aprendizado Profundo , Esclerose Múltipla , Humanos , Esclerose Múltipla/complicações , Esclerose Múltipla/diagnóstico , Ataxia/diagnóstico , Ataxia/etiologia , Modalidades de Fisioterapia
4.
Neurol Neuroimmunol Neuroinflamm ; 11(2): e200193, 2024 03.
Artigo em Inglês | MEDLINE | ID: mdl-38181318

RESUMO

A 15-year-old adolescent boy developed subacute ataxia, encephalopathy, ophthalmoplegia, and dysarthria following a sore throat. An MRI examination revealed multifocal enhancing and nonenhancing supratentorial white matter and symmetric brainstem lesions. After 2 additional presentations with worsening symptoms and lesion accumulation, he was ultimately successfully treated with rituximab for his condition.


Assuntos
Encefalopatias , Doenças Desmielinizantes , Oftalmoplegia , Masculino , Adolescente , Humanos , Ataxia/etiologia , Rituximab , Oftalmoplegia/diagnóstico , Oftalmoplegia/etiologia , Doenças Desmielinizantes/complicações
5.
Brain Dev ; 46(2): 103-107, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38000948

RESUMO

OBJECTIVE: To analyze etiologic factors of pediatric acute ataxia and to identify the severity of its underlying causes for urgent medical intervention. METHODS: Clinical data of children diagnosed with acute ataxia between December 2015 and December 2021 from one national medical center were analyzed retrospectively. RESULTS: A total of 99 children (59 boys, 40 girls), median age at disease onset 55 (range: 12-168) months, were enrolled. The median follow period was 46 (range 6-78) months. Eighty-six (86.9 %) children were diagnosed with immune-associated acute ataxia, among which acute post-infectious cerebellar ataxia (APCA) was the most common diagnosis (50.5 %), followed by demyelinating diseases of the central nervous system (18.2 %) and Guillain-Barré syndrome (9.1 %). On cerebrospinal fluid (CSF) examination, 35/73 (47.9 %) patients had pleocytosis (>5 cells/mm3), and 18/73 (24.7 %) had elevated protein levels. Thirty-one patients (31.3 %) had an abnormal cerebral MRI. Children with other immune-associated acute cerebellar ataxia had more extracerebellar symptoms, intracranial MRI lesions, abnormal CSF results, longer hospital stay, higher recurrence rates and incidence of neurological sequelae than children with APCA. CONCLUSION: Immune-associated acute ataxia is the main cause of pediatric acute ataxia, among which APCA is the most common phenotype. However, some immune-associated diseases, especially autoantibody-mediated disease, which has a higher recurrence rate and neurological sequelae account for an increasing proportion of pediatric acute ataxia. When children present with extracerebellar symptoms, abnormal cranial MRI or CSF results, and without prodromal infection, prudent differential diagnosis is recommended.


Assuntos
Ataxia Cerebelar , Masculino , Feminino , Criança , Humanos , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/epidemiologia , Ataxia Cerebelar/etiologia , Estudos Retrospectivos , Ataxia/epidemiologia , Ataxia/etiologia , Hospitais , Imageamento por Ressonância Magnética/efeitos adversos , Doença Aguda
8.
Parkinsonism Relat Disord ; 117: 105861, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37748994

RESUMO

Immune-mediated cerebellar ataxias were initially described as a clinical entity in the 1980s, and since then, an expanding body of evidence has contributed to our understanding of this topic. These ataxias encompass various etiologies, including postinfectious cerebellar ataxia, gluten ataxia, paraneoplastic cerebellar degeneration, opsoclonus-myoclonus-ataxia syndrome and primary autoimmune cerebellar ataxia. The increased permeability of the brain-blood barrier could potentially explain the vulnerability of the cerebellum to autoimmune processes. In this manuscript, our objective is to provide a comprehensive review of the most prevalent diseases within this group, emphasizing clinical indicators, pathogenesis, and current treatment approaches.


Assuntos
Ataxia Cerebelar , Síndrome de Opsoclonia-Mioclonia , Humanos , Ataxia Cerebelar/etiologia , Ataxia Cerebelar/patologia , Ataxia/diagnóstico , Ataxia/etiologia , Cerebelo/patologia , Síndrome de Opsoclonia-Mioclonia/patologia
10.
Pediatr Blood Cancer ; 70(11): e30613, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37561355

RESUMO

BACKGROUND/OBJECTIVE: In children, medulloblastoma (MB) is the most prevalent posterior fossa tumor. The first line of treatment is maximal safe resection. Therefore, symptoms of ataxia are commonly seen. Training the brain on balance and cognitive tasks makes balance more automatic than without cognitive tasks. The goal was to assess the effectiveness of dual-task practice on balance after MB excision in children with ataxia. METHODS: Thirty children with ataxia after MB resection at Children Cancer Hospital Egypt were randomized into two equal groups. Exercises for mobility, balance, and gait training were given to both groups. The research group underwent a specific dual-task program (balance and cognitive). The program ran 3 days per week for 8 weeks. Children were evaluated before and after the treatment regimen using the Scale of Assessment and Rating of Ataxia (SARA), the HUMAC Balance System, Pediatric Balance Scale, and Functional Independent Measurement. All children's legal guardians signed an ethical agreement. RESULTS: A notable improvement in balance was found in the dual group in Pediatric Balance Scale (PBS) (p = .028) and stability test (p = .0001) in favor of the study group. No discernible difference was observed in the Functional Independent Measurement score among the two groups (p = .158), although there was a statistically significant increase in both groups after treatment. CONCLUSION: Dual-task program is more effective than traditional physical therapy alone in improving balance in children with ataxia after MB resection.


Assuntos
Neoplasias Cerebelares , Meduloblastoma , Humanos , Criança , Marcha , Meduloblastoma/cirurgia , Terapia por Exercício , Ataxia/etiologia , Neoplasias Cerebelares/cirurgia
11.
Arch Iran Med ; 26(3): 166-171, 2023 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-37543939

RESUMO

BACKGROUND: COVID-19 infection and its neurological manifestations were seen in children although less common than adults. The aim of this study was to determine the frequency of different types of neurologic findings of hospitalized children with COVID-19. ]. METHODS: This retrospective study was performed on hospitalized pediatric patients aged≤18 years with confirmed SARS-CoV-2 at Children's Medical Center Hospital. Neurological manifestations were defined as the presence of any of the following symptoms: seizure, altered mental status, behavioral/personality change, ataxia, stroke, muscle weakness, smell and taste dysfunctions, and focal neurological disorders. RESULTS: Fifty-four children with COVID-19 were admitted and their mean age was 6.94±4.06 years. Thirty-four of them (63%) were male. The most frequent neurological manifestation was seizure (19 [45%]) followed by muscle weakness (11 [26%]), loss of consciousness (10 [23%]), and focal neurological disorders (10 [23%]). Other neurological manifestations consisted of headache (n=7), movement disorders (n=6), behavioral/personality change (n=5), ataxia (n=3), and stroke (n=3). Twenty-nine percent of our patients had leukocytosis. A neutrophil count above 70% was seen in 31% of participants. Among our patients, 81% had a positive reverse-transcription polymerase chain reaction (RT-PCR) test for SARS-CoV-2. CONCLUSION: During the current pandemic outbreak, hospitalized children with COVID-19 should be evaluated for neurological signs because it is common among them and should not be under-estimated.


Assuntos
COVID-19 , Acidente Vascular Cerebral , Adulto , Humanos , Masculino , Criança , Pré-Escolar , Feminino , COVID-19/epidemiologia , SARS-CoV-2 , Irã (Geográfico)/epidemiologia , Estudos Retrospectivos , Convulsões , Ataxia/etiologia , Hospitais
12.
J Neurol ; 270(10): 4593-4607, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37515734

RESUMO

OBJECTIVE: In the fourth year of the COVID-19 pandemic, mortality rates decreased, but the risk of neuropsychiatric disorders remained the same, with a prevalence of 3.8% of pediatric cases, including movement disorders (MD) and ataxia. METHODS: In this study, we report on a 10-year-old girl with hemichorea after SARS-CoV-2 infection and immunostained murine brain with patient CSF to identify intrathecal antibodies. Additionally, we conducted a scoping review of children with MD and ataxia after SARS-CoV-2 infection. RESULTS: We detected antibodies in the patient's CSF binding unknown antigens in murine basal ganglia. The child received immunosuppression and recovered completely. In a scoping review, we identified further 32 children with de novo MD or ataxia after COVID-19. While in a minority of cases, MD or ataxia were a symptom of known clinical entities (e.g. ADEM, Sydenham's chorea), in most children, the etiology was suspected to be of autoimmune origin without further assigned diagnosis. (i) Children either presented with ataxia (79%), but different from the well-known postinfectious acute cerebellar ataxia (older age, less favorable outcome, or (ii) had hypo-/hyperkinetic MD (21%), which were choreatic in most cases. Besides 14% of spontaneous recovery, immunosuppression was necessary in 79%. Approximately one third of children only partially recovered. CONCLUSIONS: Infection with SARS-CoV-2 can trigger de novo MD in children. Most patients showed COVID-19-associated-ataxia and fewer-chorea. Our data suggest that patients benefit from immunosuppression, especially steroids. Despite treatment, one third of patients recovered only partially, which makes up an increasing cohort with neurological sequelae.


Assuntos
COVID-19 , Ataxia Cerebelar , Coreia , Transtornos dos Movimentos , Feminino , Criança , Humanos , Animais , Camundongos , Ataxia Cerebelar/etiologia , Ataxia Cerebelar/diagnóstico , SARS-CoV-2 , Pandemias , COVID-19/complicações , Transtornos dos Movimentos/etiologia , Ataxia/etiologia , Coreia/etiologia , Anticorpos
14.
Rev Neurol (Paris) ; 179(8): 910-913, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37301658

RESUMO

Sensory neuronopathies name the degeneration of peripheral sensory neurons in dorsal root ganglia. Among the genetic causes, CANVAS could be the most frequent. CANVAS is a clinical entity associating cerebellar ataxia, sensory neuronopathy and vestibular areflexia due to biallelic expansions in RFC1. This study reports the 18 individuals with sensory neuronopathy tested for RFC1 expansion in our center. The clinical picture showed that chronic cough was a frequent sign beginning before the onset of other symptoms. CANVAS is an underestimated cause of late-onset sensory and cerebellar ataxia that needs to be tested for widely now that the molecular cause is known.


Assuntos
Ataxia Cerebelar , Doenças do Sistema Nervoso Periférico , Humanos , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/genética , Ataxia/etiologia , Ataxia/genética , Síndrome , Exame Neurológico
16.
Artigo em Inglês | MEDLINE | ID: mdl-37152624

RESUMO

Background: A 61-year-old male patient presented with cerebellar syndrome, which had progressively worsened for 10 days, followed by a tonic-clonic seizure. Phenomenology Shown: Blood analysis showed severe hypomagnesemia and a brain MRI showed T2 hyperintensity in the cerebellar hemispheres (Figure 1). Therefore, the final diagnosis was cerebellar syndrome and epileptic seizures secondary to severe hypomagnesemia. Educational Value: In cases of subacute onset of ataxia, the possibility of ataxia secondary to hypomagnesemia should be considered, as it can be diagnosed with a basic blood test and there are potentially life-threatening outcomes in the absence of treatment, with a reversible course following early supplementation.


Assuntos
Ataxia Cerebelar , Masculino , Humanos , Pessoa de Meia-Idade , Ataxia Cerebelar/diagnóstico por imagem , Ataxia Cerebelar/etiologia , Ataxia/diagnóstico por imagem , Ataxia/etiologia , Convulsões/diagnóstico por imagem , Convulsões/tratamento farmacológico , Convulsões/etiologia , Imageamento por Ressonância Magnética , Neuroimagem
17.
Parkinsonism Relat Disord ; 111: 105435, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-37187082

RESUMO

BACKGROUND: Symmetric biphasic pulses have been shown to acutely increase the therapeutic window of ventralis intermedius deep brain stimulation (Vim-DBS) for essential tremor (ET) compared to cathodic pulses. Acute supratherapeutic stimulation can induce ataxic side effects in Vim-DBS. OBJECTIVE: To investigate the effect on tremor, ataxia and dysarthria of 3 h of biphasic stimulation in patients with DBS for ET. METHODS: A randomized, doubled-blind, cross-over design was used to compare standard cathodic pulses with symmetric biphasic pulses (anode-first) during a 3-h period per pulse shape. During each 3-h period, all stimulation parameters were identical, except for the pulse shape. Tremor (Fahn-Tolosa-Marin Tremor Rating Scale), ataxia (International Cooperative Ataxia Rating Scale) and speech (acoustic and perceptual measures) were assessed hourly during the 3-h periods. RESULTS: Twelve ET patients were included. During the 3-h stimulation period, tremor control was equivalent between the two pulse shapes. Biphasic pulses elicited significantly less ataxia than cathodic pulses (p = 0.006). Diadochokinesis rate of speech was better for the biphasic pulse (p = 0.048), but other measures for dysarthria were not significantly different between the pulses. CONCLUSION: Symmetric biphasic pulses induce less ataxia than conventional pulses after 3 h of stimulation DBS in ET patients.


Assuntos
Estimulação Encefálica Profunda , Tremor Essencial , Humanos , Ataxia/etiologia , Estimulação Encefálica Profunda/efeitos adversos , Disartria/etiologia , Eletrodos , Tremor Essencial/terapia , Tremor Essencial/etiologia , Resultado do Tratamento , Tremor/etiologia , Núcleos Ventrais do Tálamo/cirurgia
18.
J Equine Vet Sci ; 126: 104493, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-37072073

RESUMO

Cervical vertebral stenotic myelopathy (CVSM), also known as equine wobbler syndrome or cervical ataxia, is a devastating neurological syndrome resulting from compression of the spinal cord at the cervical region. This report describes a novel surgical technique for treatment of 16-month-old Arabian filly with CVSM. The filly showed grade 4 ataxia, hypermetria, weakness of the hind limbs, stumbling during walking, and abnormal gait. Case history, clinical signs and myelography revealed spinal cord compression between the C3 and C4 and C4-C5. The filly underwent a novel surgical interference for decompression and stabilization of the point of stenosis using specially designed titanium plate and intervertebral spacer. Evidence of arthrodesis with absence of complications was confirmed by periodic radiography over eight months of postoperative care. The new technique applied in this cervical surgery was efficient for the decompression and stabilization of the vertebrae, allowing arthrodesis development and remission of the clinical signs. The obtained results encourage further assessment of this novel procedure in horses clinically affected by CVSM.


Assuntos
Compressão da Medula Espinal , Doenças da Medula Espinal , Estenose Espinal , Animais , Cavalos , Feminino , Doenças da Medula Espinal/complicações , Doenças da Medula Espinal/veterinária , Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Estenose Espinal/diagnóstico por imagem , Estenose Espinal/cirurgia , Estenose Espinal/complicações , Estenose Espinal/veterinária , Compressão da Medula Espinal/diagnóstico por imagem , Compressão da Medula Espinal/etiologia , Compressão da Medula Espinal/cirurgia , Compressão da Medula Espinal/veterinária , Ataxia/etiologia , Ataxia/veterinária
20.
Rinsho Shinkeigaku ; 63(4): 201-208, 2023 Apr 25.
Artigo em Japonês | MEDLINE | ID: mdl-36990780

RESUMO

Ataxia is not only due to cerebellar lesions, but also due to non-cerebellar lesions such as those in the brain, spinal cord, dorsal root (DR), peripheral nerve. In this article, optic ataxia is excluded and 'vestibular ataxia' is briefly referred. Non-cerebellar ataxias are generically called sensory ataxia or posterior column ataxia. However, since non-cerebellar lesions, e.g. frontal lobe lesions, may develop "cerebellar-like ataxia" (Hirayama, 2010). At the same time, non-posterior column lesions, e.g. parietal lobe lesion, can show "posterior column-like ataxia". From these viewpoints, I here describe various non-cerebellar ataxia in some disorders such as tabes dorsalis and sensory neuropathies and emphasize a role of a peripheral sensory input to the cerebellum via the DR ganglia and spinocerebellar tract for sensory ataxia because there is the International Consensus (2016) that the ataxia in Miller Fisher syndrome is suggested cerebellar-like clinicophysiologically.


Assuntos
Ataxia Cerebelar , Degenerações Espinocerebelares , Humanos , Ataxia Cerebelar/etiologia , Degenerações Espinocerebelares/patologia , Ataxia/etiologia , Cerebelo/patologia , Raízes Nervosas Espinhais/patologia
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