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1.
BMJ Case Rep ; 13(12)2020 Dec 17.
Artigo em Inglês | MEDLINE | ID: mdl-33334756

RESUMO

Acquired cerebellar ataxia is a rare, in many cases immune-modulated and paraneoplastic illness. Acute and slowly progredient processes are possible. An early treatment is important for a good clinical outcome. Here we present the case of female patient in her 60s with an antirecoverin associated cerebellitis without retinopathia and neoplasia. After an immunosuppressive therapy with steroids and rituximab the symptoms improved, and the progression could be stopped.


Assuntos
Autoanticorpos/sangue , Ataxia Cerebelar/diagnóstico , Marcha Atáxica/diagnóstico , Imunossupressores/uso terapêutico , Recoverina/imunologia , Idade de Início , Autoanticorpos/imunologia , Ataxia Cerebelar/sangue , Ataxia Cerebelar/tratamento farmacológico , Ataxia Cerebelar/imunologia , Cerebelo/diagnóstico por imagem , Cerebelo/imunologia , Feminino , Marcha Atáxica/sangue , Marcha Atáxica/tratamento farmacológico , Marcha Atáxica/imunologia , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imagem por Ressonância Magnética , Pessoa de Meia-Idade , Rituximab/uso terapêutico
2.
Annu Int Conf IEEE Eng Med Biol Soc ; 2020: 4571-4574, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-33019011

RESUMO

Cerebellar ataxia (CA) refers to the impaired balance and coordination resulting from injury or degeneration of the cerebellum. Testing balance is one of the simplest means of assessing CA. This study compares instrumented assessment and clinical assessment scales of the balance test called Romberg's test. Inertial Measurement Unit (IMU) data were collected from a sensor attached to their chest of 53 subjects while they performed the test. The corresponding clinical scores were also tabulated. Using this data, 99 features were extracted to quantify acceleration, tremor and displacement of body sway. These features were filtered to identify the subset that better characterize the distinctive behavior of CA subjects. Elastic Net Regression model resulted a greater agreement (0.70 Pearson coefficient) with the clinical SARA scores. The overall results indicated that data from a single IMU sensor is sufficient to accurately assess balance in CA. The significance of this study is that evaluation of balance using Recurrence Quantification Analysis produces a comprehensive framework for the assessment of CA.


Assuntos
Ataxia Cerebelar , Aceleração , Ataxia Cerebelar/diagnóstico , Humanos , Equilíbrio Postural , Tórax , Tremor
3.
Annu Int Conf IEEE Eng Med Biol Soc ; 2020: 5640-5643, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-33019256

RESUMO

Cerebellar Ataxia is a neurological disorder without an approved treatment. Patients will have impaired and uncoordinated motor functionality making them unable to complete their day-to-day activities. Ataxia clinics are established around the world to facilitate research and rehabilitate patients. However, the patients are generally evaluated by human - observation. Therefore, machine learning based data analysis is popular on motion captured via sensors. There are many neurological tests designed to analyse the motor impairments in different domains (such as upper limb, lower limb, gait, balance and speech). Clinicians follow scoring protocols to record the severity of patients for each domain test. This paper delivers a clinical assessment platform combining 12 neurological tests in 5 domains. It captures motion (from BioKin sensors), haptic and audio data (from the tablet or laptop screen). A data analysis system is hosted in a remote server which evaluates data to produce a severity score via different models built for each neurological test. The assessment platform clients and server communicate via a cloud buffer system. The scores input by the clinicians and predicted by the machine learning models are logged in the cloud database. This enables clinicians and doctors to view and compare the history of patient diagnosis. The server system is structured for automated score model upgrades via prompted approval. Thus, the most viable scoring model could be accommodated for each test based on longitudinal studies.


Assuntos
Ataxia Cerebelar , Ataxia , Ataxia Cerebelar/diagnóstico , Marcha , Humanos , Fala , Extremidade Superior
4.
Annu Int Conf IEEE Eng Med Biol Soc ; 2020: 816-819, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-33018110

RESUMO

Human observer-based assessments of Cerebellar Ataxia (CA) are subjective and are often inadequate to track mild motor symptoms. This study examines the potential use of a comprehensive sensor-based approach for objective evaluation of CA in five domains (speech, upper limb, lower limb, gait and balance) through the instrumented versions of nine bedside neurological tests. A total of twenty-three participants diagnosed with CA to varying degrees and eleven healthy controls were recruited. Data was collected using wearable inertial sensors and Kinect camera. In our study, an optimal feature subset based on feature importance in the Random Forest classifier model demonstrated an impressive performance accuracy of 97% (F1 score = 95.2%) for CA-control discrimination. Our experimental findings also indicate that the Romberg test contributed most, followed by the peripheral tests, while the Gait test contributed least to the classification. Sensor-based approaches, therefore, have the potential to complement existing clinical assessment techniques, offering advantages in terms of consistency, objectivity and informed clinical decision-making.


Assuntos
Ataxia Cerebelar , Ataxia Cerebelar/diagnóstico , Marcha , Humanos , Reprodutibilidade dos Testes , Fala , Extremidade Superior
5.
Annu Int Conf IEEE Eng Med Biol Soc ; 2020: 859-862, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-33018120

RESUMO

Cerebellar ataxia (CA) results from injury to or disease of the cerebellum. It describes the resulting motor dysfunction, characterised by inaccuracy, incoordination and delay in initiation of movement, tremor, and imbalance. Assessment of ataxia to diagnose and monitor progress is by clinical observance of the performance of standard motor tasks. An accurate instrumented measurement of CA would therefore be of great interest. This study was aimed at assessing upper-limb ataxia during ballistic tracking of a computer-generated target in individuals with CA and controls using motion measures obtained from a Kinect camera and a wearable motioncaptured device. A set of features derived from these motion measurements were used to develop a method for objective quantification of CA. Difference between ataxic and non-ataxic movements can be readily be observed in features from both devices (p= 0.008) and their values associated with a standard clinical scale (rho = 0.80, p < 0.001). The combination of multimodal features improved the ability to distinguish between CA subjects and controls and to measure the severity of upper limb ataxia.


Assuntos
Ataxia Cerebelar , Dispositivos Eletrônicos Vestíveis , Ataxia Cerebelar/diagnóstico , Cerebelo , Humanos , Movimento , Tremor
6.
Brain Nerve ; 72(9): 923-930, 2020 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-32934181

RESUMO

Marie et al. (1922) first proposed a disease entity "late cortical cerebellar atrophy (LCCA)", which is characterized neuropathologically by pure cerebello-olivary degeneration. LCCA was originally described as sporadic, late-onset, pure cerebellar ataxia of unknown etiology; however, it has occasionally been used to denote familial or secondary ataxias, particularly alcoholic cerebellar degeneration. Sporadic ataxia is classified mainly into LCCA or CCA and olivo-ponto-cerebellar atrophy (OPCA) in Japan. OPCA, now multiple system atrophy with predominant cerebellar ataxia, has characteristic brain imaging features and is clearly diagnosed based on the consensus criteria. On the other hand, there is no specific biomarker for LCCA/CCA, and neuropathological examination is required for a definitive diagnosis. Therefore, the clinical diagnosis of LCCA/CCA depends on the exclusion of other diseases manifesting as cerebellar ataxia. However the differential diagnosis for LCCA/CCA is not necessarily made carefully. As a result, the LCCA/CCA category in Japan is a "waste basket," including OPCA, hereditary ataxias, and secondary ataxias, which are unidentified yet. To refine the LCCA/CCA category, we proposed the clinically-defined term "idiopathic cerebellar ataxia (IDCA)" and established its diagnostic criteria. By nationwide screening, we have identified 51 patients with probable IDCA according to the criteria so far. Here we review the clinical characteristics of IDCA patients.


Assuntos
Ataxia Cerebelar , Degenerações Espinocerebelares , Atrofia/patologia , Ataxia Cerebelar/diagnóstico , Cerebelo , Humanos , Japão , Degenerações Espinocerebelares/diagnóstico , Degenerações Espinocerebelares/patologia
7.
Brain Nerve ; 72(9): 931-937, 2020 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-32934182

RESUMO

Cerebellar ataxia-predominant multiple system atrophy (MSA-C) and cortical cerebellar atrophy are representative diseases of adult-onset sporadic degenerative ataxia. Both diseases are distinctly different because of α-synuclein pathology. However, it takes approximately 2 years for cerebellar ataxia to progress to concomitant severe autonomic dysfunction in patients with MSA-C. The period of only cerebellar ataxia (mono system atrophy) may extend to more than 10 years. Understanding mono system atrophy is vital for the early diagnosis and drug development for MSA. In this review, we discuss mono system atrophy focusing on the concept and natural history and the possibility of the of early diagnosis and disease-modifying therapy for MSA.


Assuntos
Ataxia Cerebelar , Atrofia de Múltiplos Sistemas , Adulto , Ataxia , Atrofia/patologia , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/tratamento farmacológico , Cerebelo , Humanos , Atrofia de Múltiplos Sistemas/diagnóstico , Atrofia de Múltiplos Sistemas/tratamento farmacológico , Atrofia de Múltiplos Sistemas/patologia
8.
Brain Nerve ; 72(9): 961-967, 2020 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-32934185

RESUMO

Recently, the diagnostic criteria for idiopathic cerebellar ataxia (IDCA) have been proposed in Japan as a diagnosis to replace the clinical concept of cortical cerebellar atrophy, which was originally described as a neuropathological disorder. However, IDCA proposed in Japan may include various diseases such as multiple system atrophy with early stage, rare hereditary ataxias, and autoimmune-mediated cerebellar ataxia. We tackled this significant clinical challenge by detecting anti-cerebellar autoantibodies in patients' sera and identifying their target antigens. We detected anti-cerebellar autoantibodies in the sera of some patients diagnosed with IDCA in Japan. In the future, it will be necessary to confirm the efficacy of immunotherapy for anti-cerebellar autoantibody-positive cases among patients who were thought to be difficult to treat.


Assuntos
Ataxia Cerebelar , Atrofia , Autoanticorpos , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/terapia , Cerebelo , Humanos , Japão
9.
Brain Nerve ; 72(9): 969-972, 2020 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-32934186

RESUMO

Cortical cerebellar atrophy has been defined as a sporadic degenerative cerebellar disorder other than multiple system atrophy, whereas the term "idiopathic cerebellar ataxia (IDCA)" has been recently proposed by a Japanese expert group. There is no diagnostic biomarker for IDCA; therefore, the diagnosis largely depends on exclusion of other cerebellar diseases, such as multiple system atrophy and hereditary spinocerebellar ataxia. Other important differential diagnoses include immune-mediated cerebellar ataxia, such as Hashimoto's encephalopathy, gluten ataxia, anti-glutamic acid decarboxylase-positive cerebellar ataxia, alcoholic cerebellar degeneration, and drug-induced ataxia. Secondary cerebellar disorders are treatable and should be recognized and screened.


Assuntos
Ataxia Cerebelar , Doença de Hashimoto , Degenerações Espinocerebelares , Ataxia , Ataxia Cerebelar/diagnóstico , Diagnóstico Diferencial , Humanos , Degenerações Espinocerebelares/diagnóstico
10.
J Nippon Med Sch ; 87(3): 153-156, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32655091

RESUMO

Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is a clinicoradiological syndrome characterized by transient mild encephalopathy and magnetic resonance imaging (MRI) findings of a reversible lesion in the splenium of the corpus callosum (SCC). Patients with MERS generally present with central nervous system symptoms such as consciousness disturbance, headache, and seizure; adult-onset MERS with cerebellar ataxia is rare. A 53-year-old man was admitted to our hospital with fever of 1 week's duration, headache, neck stiffness, and gait disturbance. Neurological examination revealed bilateral intention tremor (predominantly affecting the right hand) and gait ataxia. Diffusion-weighted brain MRI showed a focal hyperintense lesion in the SCC. Cerebrospinal fluid analysis revealed elevated levels of mononuclear cells and proteins. Brain imaging with 123I-iofetamine single-photon emission computed tomography showed reduced cerebral blood flow in the left thalamus and right cerebellum. Several diseases, including cerebellar stroke and acute cerebellitis, develop as comorbidities in patients with acute cerebellar ataxia. This case suggests that MERS should be suspected in adults with cerebellar ataxia.


Assuntos
Encefalopatias , Ataxia Cerebelar , Encefalite , Doenças da Coluna Vertebral , Adulto , Encéfalo/diagnóstico por imagem , Encefalopatias/diagnóstico , Ataxia Cerebelar/diagnóstico , Corpo Caloso/diagnóstico por imagem , Imagem de Difusão por Ressonância Magnética , Encefalite/diagnóstico , Marcha Atáxica/diagnóstico por imagem , Humanos , Masculino , Meningite Viral/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Doenças Raras , Índice de Gravidade de Doença , Doenças da Coluna Vertebral/diagnóstico , Síndrome
11.
Nervenarzt ; 91(6): 537-540, 2020 Jun.
Artigo em Alemão | MEDLINE | ID: mdl-32367146

RESUMO

This article presents the case of a 74-year-old female patient who first developed a progressive disease with sensory neuropathy, cerebellar ataxia and bilateral vestibulopathy at the age of 60 years. The family history was unremarkable. Magnetic resonance imaging (MRI) showed atrophy of the cerebellum predominantly in the vermis and atrophy of the spinal cord. The patient was given the syndromic diagnosis of cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS). In 2019 the underlying genetic cause of CANVAS was discovered to be an intronic repeat expansion in the RFC1 gene with autosomal recessive inheritance. The patient exhibited the full clinical picture of CANVAS and was tested positive for this repeat expansion on both alleles. The CANVAS is a relatively frequent cause of late-onset hereditary ataxia (estimated prevalence 5­13/100,000). In contrast to the present patient, the full clinical picture is not always present. Therefore, testing for the RFC1 gene expansion is recommended in the work-up of patients with otherwise unexplained late-onset sporadic ataxia. As intronic repeat expansions cannot be identified by next generation sequencing methods, specific testing is necessary.


Assuntos
Vestibulopatia Bilateral , Ataxia Cerebelar , Doenças do Sistema Nervoso Periférico , Idoso , Ataxia , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/genética , Feminino , Humanos , Síndrome
12.
BMC Med Genet ; 21(1): 68, 2020 03 31.
Artigo em Inglês | MEDLINE | ID: mdl-32234020

RESUMO

BACKGROUND: The TWNK gene encodes the twinkle protein, which is a mitochondrial helicase for DNA replication. The dominant TWNK variants cause progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal dominant 3, while the recessive variants cause mitochondrial DNA depletion syndrome 7 and Perrault syndrome 5. Perrault syndrome is characterized by sensorineural hearing loss in both males and females and gonadal dysfunction in females. Patients with Perrault syndrome may present early-onset cerebellar ataxia, whereas middle-age-onset cerebellar ataxia caused by TWNK variants is rare. CASE PRESENTATION: A Japanese female born to consanguineous parents presented hearing loss at age 48, a staggering gait at age 53, and numbness in her distal extremities at age 57. Neurological examination revealed sensorineural hearing loss, cerebellar ataxia, decreased deep tendon reflexes, and sensory disturbance in the distal extremities. Laboratory tests showed no abnormal findings other than a moderate elevation of pyruvate concentration levels. Brain magnetic resonance imaging revealed mild cerebellar atrophy. Using exome sequencing, we identified a homozygous TWNK variant (NM_021830: c.1358G>A, p.R453Q). CONCLUSIONS: TWNK variants could cause middle-age-onset cerebellar ataxia. Screening for TWNK variants should be considered in cases of cerebellar ataxia associated with deafness and/or peripheral neuropathy, even if the onset is not early.


Assuntos
Ataxia Cerebelar/genética , DNA Helicases/genética , Proteínas Mitocondriais/genética , Ataxia Cerebelar/complicações , Ataxia Cerebelar/diagnóstico , Consanguinidade , Feminino , Marcha Atáxica/complicações , Marcha Atáxica/diagnóstico , Marcha Atáxica/genética , Disgenesia Gonadal 46 XX/diagnóstico , Disgenesia Gonadal 46 XX/genética , Perda Auditiva/complicações , Perda Auditiva/diagnóstico , Perda Auditiva/genética , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/genética , Homozigoto , Humanos , Japão , Transtornos de Início Tardio/diagnóstico , Transtornos de Início Tardio/genética , Pessoa de Meia-Idade , Mutação , Linhagem
13.
J Clin Neurosci ; 76: 238-240, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32307300

RESUMO

The hot cross bun (HCB) sign describes cruciform-shaped T2-weighted hyperintensities of pontocerebellar fibers within the pons and is a typical, but not specific imaging hallmark of the cerebellar variant of multiple system atrophy (MSA-C). We report a case of a 51-year-old woman who was first diagnosed with MSA-C based on progressive cerebellar ataxia, the HCB sign and T2-weighted hyperintensities in middle cerebellar peduncles on MRI. However, further diagnostic work-up revealed positive anti-amphiphysin antibodies in blood and cerebrospinal fluid and subsequently breast cancer. This report of a paraneoplastic rhombencephalitis which initially mimicked MSA-C imaging features stresses the importance of considering immune-mediated rhombencephalitis as differential diagnosis in cases of progressive cerebellar ataxia and the HCB sign on T2-weighted MRI, especially in the absence of pontocerebellar atrophy.


Assuntos
Atrofia de Múltiplos Sistemas/diagnóstico , Neoplasias da Mama , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/diagnóstico por imagem , Doenças Cerebelares/diagnóstico por imagem , Cerebelo/diagnóstico por imagem , Diagnóstico Diferencial , Feminino , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Pedúnculo Cerebelar Médio/diagnóstico por imagem , Atrofia de Múltiplos Sistemas/diagnóstico por imagem , Degenerações Espinocerebelares/diagnóstico por imagem
14.
Rev. cuba. med. trop ; 72(1): e476, ene.-abr. 2020. tab, graf
Artigo em Espanhol | LILACS, CUMED | ID: biblio-1126703

RESUMO

Introducción: La ataxia constituye una alteración en la coordinación de los movimientos, resultado de una disfunción del cerebelo, sus conexiones, así como alteraciones en la médula espinal, nervios periféricos o una combinación de estas condiciones. Las ataxias se clasifican en hereditarias, esporádicas y en adquiridas o secundarias, en las cuales los virus neurotrópicos constituyen los principales causantes. Objetivo: Actualizar los conocimientos relacionados con las ataxias causadas por virus neurotrópicos y los mecanismos neurodegenerativos que pudieran tener relación con la ataxia. Métodos: Se realizó una revisión bibliográfica incluyendo artículos publicados en las principales bases de datos bibliográficas (Web of Sciences, Scopus, SciELO). Se utilizaron las palabras claves: ataxia, virus neurotrópicos, ataxias cerebelosas, ataxias infecciosas, en inglés y español. Análisis e integración de la información: Los virus más conocidos que provocan ataxias infecciosas son el virus de inmunodeficiencia humana, virus del herpes simple, virus del herpes humano tipo 6, virus de la varicela zoster, virus Epstein-Barr, virus del Nilo Occidental, y enterovirus 71, aunque existen otros virus que causan esta afectación. Los mecanismos neuropatogénicos sugeridos son la invasión directa del virus y procesos inmunopatogénicos desencadenados por la infección. Estos virus pueden causar ataxia cerebelosa aguda, ataxia aguda posinfecciosa, síndrome opsoclono-mioclono-atáxico y ataxia por encefalomielitis aguda diseminada. Aunque la mayoría de los reportes de casos informan la evolución satisfactoria de los pacientes, algunos refieren complicaciones neurológicas e incluso la muerte. Conclusiones: Actualmente existe la necesidad de profundizar en el estudio de este tipo de ataxia para favorecer su diagnóstico y tratamiento(AU)


Introduction: Ataxia is an alteration in the coordination of movements caused by a dysfunction of the cerebellum and its connections, as well as alterations in the spinal cord, the peripheral nerves, or a combination of these factors. Ataxias are classified into hereditary, sporadic and acquired or secondary, in which neurotropic viruses are the main causative agents. Objective: Update knowledge about ataxias caused by neurotropic viruses and the neurodegenerative mechanisms which could bear a relationship to ataxia. Methods: A review was conducted of papers published in the main bibliographic databases (Web of Sciences, Scopus, SciELO), using the search terms ataxia, neurotropic virus, cerebellar ataxias, infectious ataxias, in English and in Spanish. Discussion: The best known viruses causing infectious ataxias are the human immunodeficiency virus, herpes simplex virus, human herpesvirus 6, varicella zoster virus, Epstein-Barr virus, Western Nile virus and enterovirus 71, though other viruses may also cause this condition. The neuropathogenic mechanisms suggested are direct invasion of the virus and immunopathogenic processes triggered by the infection. These viruses may cause acute cerebellar ataxia, acute postinfectious ataxia, opsoclonus-myoclonus-ataxia syndrome and ataxia due to acute encephalomyelitis disseminata. Though most case reports describe a satisfactory evolution of patients, some refer to neurological complications and even death. Conclusions: There is a current need to carry out further research about this type of ataxia to improve its diagnosis and treatment(AU)


Assuntos
Humanos , Masculino , Feminino , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/epidemiologia , Fatores de Virulência
15.
BMJ Case Rep ; 13(3)2020 Mar 17.
Artigo em Inglês | MEDLINE | ID: mdl-32188617

RESUMO

Human herpesvirus 6 (HHV-6) infection is the cause of roseola infantum in children. The reactivation of HHV-6 is associated with multiple clinical syndromes including encephalitis and myelitis, especially in haematopoietic stem cell transplant recipients. However, the virus can cause encephalitis in other immunosuppressed as well as immunocompetent individuals. We report a case of a 70-year-old woman who was immunocompromised secondary to treatment of rheumatoid arthritis with leflunomide and methotrexate. The patient presented with acute ataxia, diplopia and dysarthria. MRI brain showed an enhancing lesion in the midbrain. The diagnosis of HHV-6 encephalitis was made after HHV-6 A DNA was detected in both serum and cerebrospinal fluid. Treatment consisted of a 3-week course of intravenous ganciclovir along with physiotherapy. At a 3-month follow-up, repeat MRI brain showed a decrease in size and oedema of the lesion and the patient's neurological function was improved.


Assuntos
Ataxia Cerebelar/diagnóstico , Encefalite Viral/diagnóstico , Herpesvirus Humano 6/isolamento & purificação , Hospedeiro Imunocomprometido , Infecções por Roseolovirus/diagnóstico , Idoso , Antivirais/uso terapêutico , Ataxia Cerebelar/complicações , Ataxia Cerebelar/diagnóstico por imagem , Ataxia Cerebelar/tratamento farmacológico , Diagnóstico Diferencial , Encefalite Viral/complicações , Encefalite Viral/diagnóstico por imagem , Encefalite Viral/tratamento farmacológico , Feminino , Ganciclovir/uso terapêutico , Humanos , Imagem por Ressonância Magnética , Infecções por Roseolovirus/complicações , Infecções por Roseolovirus/diagnóstico por imagem , Infecções por Roseolovirus/tratamento farmacológico
16.
Medicine (Baltimore) ; 99(9): e19344, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32118770

RESUMO

INTRODUCTION: The inferior cerebellar peduncle (ICP) is a major neural tract in the cerebellum and is involved in coordination of movement and proprioceptive; therefore, ICP injury can be accompanied by poor coordination of movement, including ataxia. In this study, using diffusion tensor tractography (DTT), we investigated the relationship between ataxia and ICP injury in patients with cerebral infarct. METHODS: We recruited 14 stroke patients with ataxia after the onset of stroke and 12 normal subjects. The Score of Assessment and Rating of Ataxia (SARA) was used to evaluate ataxia. The values of fractional anisotropy (FA), apparent diffusion coefficient, and fiber number (FN) of the ICP were measured for the diffusion tensor imaging parameters. RESULTS: Significant differences were observed in the FA and FN values of the ICP in the affected hemisphere between the patient and control groups (P < .05). In addition, the FN value of the ICP in the affected hemisphere showed a negative correlation with SARA (r = -0.538, P < .05). However, parameters of the ICP in the unaffected hemisphere or the FN value in the unaffected hemisphere showed no correlation with SARA (P > .05). CONCLUSION: We found that the ataxia severity was closely related to the severity of ICP injury in patients with cerebral infarct. Our results suggest that evaluation of the ICP using DTT would be useful for patients with ataxia after cerebral infarct.


Assuntos
Ataxia Cerebelar/complicações , Infarto Cerebral/etiologia , Pedúnculo Cerebral/lesões , Adulto , Idoso , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/epidemiologia , Infarto Cerebral/epidemiologia , Infarto Cerebral/fisiopatologia , Pedúnculo Cerebral/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
17.
J Clin Neurosci ; 71: 135-140, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31501004

RESUMO

Cerebellar ataxia and Parkinson's disease are neurodegenerative disorders clinically characterized by motor disabilities including gait disturbance. This study aimed to investigate the usefulness of an infrared depth sensor device to quantitatively evaluate gait disturbances in patients with movement disorders. 25 ataxic, 25 Parkinson's disease, and 25 control subjects were enrolled and evaluated their walk. Stride length, feet interval, gait rhythm, and a ratio of the actual walking route length to the linear distance between the start and goal points (A/L ratio) were assessed and compared. Outcome correlations with clinical scales were also analyzed. The average stride length was shorter in ataxic subjects or Parkinson's disease subjects than in control subjects. The average feet interval was larger in ataxic subjects than in control subjects. The stride length coefficient of variation (CV), gait rhythm CV, and average and standard deviations of the A/L ratio were larger in ataxic or Parkinson's disease subjects than in control subjects. Ataxic subjects exhibited significant positive correlations between the CV of stride length or average feet interval and scale for the assessment and rating of ataxia scores or international cooperative ataxia rating scale scores. Parkinson's disease subjects exhibited a significant correlation between the average stride length, CV of stride length, or standard deviation of A/L ratio and unified Parkinson's disease rating scale score. The device used in this study differentiated the characteristics of gait disturbance in each movement disorder and quantitatively evaluated ataxia or Parkinson's disease severity, indicating its potential clinical utility across applications.


Assuntos
Ataxia Cerebelar/diagnóstico , Análise da Marcha/instrumentação , Doença de Parkinson/diagnóstico , Idoso , Feminino , Análise da Marcha/métodos , Humanos , Raios Infravermelhos , Pessoa de Meia-Idade
18.
Int J Neurosci ; 130(3): 276-278, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31554442

RESUMO

Purpose: Arbovirosis, viral infection transmitted by arthropods, is a widespread health problem. In Italy, as well for all Mediterranean basin, from late spring to the end of summer, Toscana Virus (TOSV), a sandfly borne virus, accounts for the majority of aseptic meningitis/meningoencephalitis cases. TOSV meningitis/meningoencephalitis has usually a self-extinguishing benign course. Our aim is to report a case of a young healthy women diagnosed with Toscana Virus meningoencephalitis with a complicated clinical course.Materials and methods/results: Case report of a 33-years old woman, admitted to the Infectious Diseases Unit at Careggi General Hospital (Florence-Italy), with a diagnosis of Toscana Virus meningoencephalitis. Seventy-two hours after the admission, she developed typical symptoms, as impaired legs coordination, slurred speech, stumbling and dysmetria, of acute cerebellar ataxia (ACA). Urgent neurological assessment was provided performing an electroencephalography study followed by a brain and brainstem magnetic resonance imaging. In the meanwhile, bilateral nystagmus arised. Through neurologist consultation ACA clinical diagnosis was then made and intravenous steroid therapy was administered with prompt symptoms resolution. The patient was finally discharged at day 10 since the ACA onset in good clinical conditions.Conclusions: To raise awareness among physicians about possible neurological complications during Toscana Virus meningoencephalitis.


Assuntos
Ataxia Cerebelar/diagnóstico , Encefalite por Arbovirus/diagnóstico , Meningite Viral/diagnóstico , Meningoencefalite/diagnóstico , Vírus da Febre do Flebótomo Napolitano/patogenicidade , Doença Aguda , Adulto , Ataxia Cerebelar/etiologia , Encefalite por Arbovirus/complicações , Encefalite por Arbovirus/virologia , Feminino , Humanos , Meningite Viral/complicações , Meningite Viral/virologia , Meningoencefalite/complicações , Meningoencefalite/virologia , Doenças Raras
19.
J Neurol ; 267(3): 625-639, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31713101

RESUMO

BACKGROUND: Cerebellar ataxia is characterized by difficulty in the planning of movement and lack of anticipatory postural adjustments, which can result in deficits of balance. Being able to have quantitative measurements in clinical practice, to detect any improvements on balance resulting from new rehabilitation treatments or experimental drugs is very important. AIM: The purpose of this study was to develop an application (APP) able to assess static and dynamic balance in patients with cerebellar ataxias (CA). The APP that works by a wearable device (smartphone) placed at the breastbone level and immobilized by an elastic band, measures the body sway by means of a triaxial accelerometer. METHODS: We investigated 40 CA patients and 80 healthy subjects. All patients were clinically evaluated using the "Berg Balance Scale" (BBS) and the "Scale for the Assessment and Rating of Ataxia" (SARA). Balance impairment was quantitatively assessed using a validated static balance evaluating systems, i.e., Techno-body Pro-Kin footboard. All participants underwent static and dynamic balance assessments using the new APP. RESULTS: We observed a strong correlation between the APP measurements and the score obtained with the BBS, SARA, and Pro-Kin footboard. The intra-rater reliability and the test-retest reliability of the APP measurements, estimated by intraclass correlation coefficient, were excellent. The standard error of measurement and the minimal detectable change were small. No learning effect was observed. CONCLUSIONS: We can state that the APP is an easy, reliable, and valid evaluating system to quantify the trunk sway in a static position and during the gait.


Assuntos
Acelerometria/instrumentação , Ataxia Cerebelar/diagnóstico , Aplicativos Móveis , Equilíbrio Postural/fisiologia , Smartphone , Dispositivos Eletrônicos Vestíveis , Adulto , Idoso , Avaliação da Deficiência , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem
20.
Sensors (Basel) ; 19(24)2019 Dec 17.
Artigo em Inglês | MEDLINE | ID: mdl-31861099

RESUMO

BACKGROUND: Patients suffering from cerebellar ataxia have extremely variable gait kinematic features. We investigated whether and how wearable inertial sensors can describe the gait kinematic features among ataxic patients. METHODS: We enrolled 17 patients and 16 matched control subjects. We acquired data by means of an inertial sensor attached to an ergonomic belt around pelvis, which was connected to a portable computer via Bluetooth. Recordings of all the patients were obtained during overground walking. From the accelerometric data, we obtained the harmonic ratio (HR), i.e., a measure of the acceleration patterns, smoothness and rhythm, and the step length coefficient of variation (CV), which evaluates the variability of the gait cycle. RESULTS: Compared to controls, patients had a lower HR, meaning a less harmonic and rhythmic acceleration pattern of the trunk, and a higher step length CV, indicating a more variable step length. Both HR and step length CV showed a high effect size in distinguishing patients and controls (p < 0.001 and p = 0.011, respectively). A positive correlation was found between the step length CV and both the number of falls (R = 0.672; p = 0.003) and the clinical severity (ICARS: R = 0.494; p = 0.044; SARA: R = 0.680; p = 0.003). CONCLUSION: These findings demonstrate that the use of inertial sensors is effective in evaluating gait and balance impairment among ataxic patients.


Assuntos
Acelerometria/instrumentação , Acidentes por Quedas , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/fisiopatologia , Equilíbrio Postural , Estudos de Casos e Controles , Feminino , Marcha , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de Risco
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