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CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2018; 37(03): 258-262
DOI: 10.1055/s-0038-1670696
Case Report | Relato de Caso
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

Pyramidal and Proprioceptive Dysfunctions as the Main Neurologic Features In Symptomatic Type I Chiari Malformation Patients

Disfunções piramidais e proprioceptivas como principais alterações neurológicas em pacientes sintomáticos portadores de malformaçõo de Chiari tipo I
Bruno Corrêa de Albuquerque Leimig
1   Hospital Estadual Getulio Vargas, Recife, PE, Brazil
,
Claudio Henrique Fernandes Vidal
2   Universidade Federal de Pernambuco, Recife, PE, Brazil
,
Marcelo Moraes Valença
2   Universidade Federal de Pernambuco, Recife, PE, Brazil
,
Joacil Carlos da Silva
2   Universidade Federal de Pernambuco, Recife, PE, Brazil
,
Walter de Freitas Matias Filho
2   Universidade Federal de Pernambuco, Recife, PE, Brazil
› Author Affiliations
Further Information

Publication History

06 April 2018

27 June 2018

Publication Date:
27 September 2018 (online)

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Abstract

Objective Broader access to magnetic resonance imaging (MRI) has increased the diagnosis of tonsillar ectopia, with most of these patients being asymptomatic. The early diagnosis and treatment of type I Chiari malformation (CM I) patients has impact on the prognosis. This study supplements information about the neurologic exam of symptomatic patients with CM I.

Methods The sample was composed of 32 symptomatic patients with CM I diagnosed by a combination of tonsil herniation of more than 5 mm below the magnum foramen (observed in the sagittal T2 MRI) and at least one of the following alterations: intractable occipital headache, ataxia, upper or lower motor neuron impairment, sensitivity deficits (superficial and deep) or lower cranial nerves disorders.

Results Occipital headache was the most frequent symptom (53.12%). During the physical exam, the most common dysfunctions were those from the pyramidal system (96.87%), followed by posterior cord syndrome (87.5%).

Discussion In this study, patients became symptomatic around the fifth decade of life, which is compatible with previous descriptions. Patients with more than 2 years of evolution have worse responses to treatment. Occipital headache, symptoms in the upper limbs, gait and proprioceptive disorders are common findings in patients with CM I.

Conclusion Deep tendinous reflexes and proprioception disorders were the main neurologic features found in symptomatic CM I patients.

Resumo

Objetivo O acesso amplo à ressonância magnética (RM) vem aumentando o diagnóstico de ectopia tonsilar, sendo a maioria dos pacientes com essa condição assintomáticos. O diagnóstico precoce e tratamento dos pacientes portadores da malformação de Chiari tipo I (MC I) tem impacto no prognóstico. O presente estudo adiciona informações sobre o exame neurológico dos pacientes com MC I sintomáticos.

Métodos A amostra foi composta por 32 pacientes sintomáticos com MC I diagnosticados por herniação tonsilar maior que 5 mm abaixo do forame magno (constatada na sequência sagital-T2 da RM) e uma das alterações a seguir: cefaleia occipital intratável, ataxia, disfunção do neurônio motor superior ou inferior, déficit sensitivo (superficial e profundo) ou paresia de nervos cranianos baixos.

Resultados A cefaleia occipital foi o sintoma mais prevalente (51,12%). Durante o exame físico, a disfunção mais comum foi da via piramidal (96,87%), seguida de síndrome cordonal posterior (87,5%).

Discussão No presente estudo, os pacientes se tornaram sintomáticos por cerca da quinta década de vida, compatível com as descrições prévias. Pacientes com mais de 2 anos de evolução obtiveram pior resposta ao tratamento. Cefaleia occipital, sintomas nos membros superiores, distúrbios de marcha e propriocepção são achados frequentes em pacientes com MC I.

Conclusão Reflexos tendinosos profundos e alteração da propriocepção são os os principais sinais no exame neurológico dos pacientes com MC I.

Keywords

arnold-chiari malformation - neurologic examination - pyramidal tracts - proprioception - syringomyelia

Palavras-chave

malformação de Arnold-Chiari - exame neurológico - tratos piramidais - propriocepção - Siringomielia
 

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