Subscribe to RSS
![](/products/assets/desktop/img/oa-logo.png)
DOI: 10.1055/s-0038-1670696
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 IPublication History
06 April 2018
27 June 2018
Publication Date:
27 September 2018 (online)
![](https://www.thieme-connect.de/media/10.1055-s-00028832/201803/lookinside/thumbnails/10-1055-s-0038-1670696_1800029-1.jpg)
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.
-
References
- 1 Alden TD, Ojemann JG, Park TS. Surgical treatment of Chiari I malformation: indications and approaches. Neurosurg Focus 2001; 11 (01) E2
- 2 Lazareff JA, Galarza M, Gravori T, Spinks TJ. Tonsillectomy without craniectomy for the management of infantile Chiari I malformation. J Neurosurg 2002; 97 (05) 1018-1022
- 3 Bejjani GK. Definition of the adult Chiari malformation: a brief historical overview. Neurosurg Focus 2001; 11 (01) E1
- 4 Bejjani GK, Cockerham KP, Rothfus WE, Maroon JC, Maddock M. Treatment of failed Adult Chiari: Malformation decompression with CSF drainage: observations in six patients. Acta Neurochir (Wien) 2001; Feb; 145 (02) 107-116 ; discussion 116
- 5 Wan MJ, Nomura H, Tator CH. Conversion to symptomatic Chiari I malformation after minor head or neck trauma. Neurosurgery 2008; 63 (04) 748-753 , discussion 753
- 6 Dyste GN, Menezes AH, VanGilder JC. Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg 1989; 71 (02) 159-168
- 7 Oakes W, Gaskill S. Chiari Malformation, hydromyelia, and syringomyelia. In Wilkins R, Rengachary S. (eds): Neurosurgery. New York: McGraw-Hill; 1985: 2102-2124
- 8 Sahuquillo J, Rubio E, Poca MA, Rovira A, Rodriguez-Baeza A, Cervera C. Posterior fossa reconstruction: a surgical technique for the treatment of Chiari I malformation and Chiari I/syringomyelia complex--preliminary results and magnetic resonance imaging quantitative assessment of hindbrain migration. Neurosurgery 1994; 35 (05) 874-884 , discussion 884–885
- 9 Alzate JC, Kothbauer KF, Jallo GI, Epstein FJ. Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 cases. Neurosurg Focus 2001; 11 (01) E3
- 10 Richard G. Everson, MD, Langston T. Holly, MD, and Ulrich Batzdorf, MD.Chiari 1 Malformation in the Adult: Part I. Contemporary Neurosurgery 2017; 39: 4
- 11 Caetano de Barros M, Farias W, Ataíde L, Lins S. Basilar impression and Arnold-Chiari malformation. A study of 66 cases. J Neurol Neurosurg Psychiatry 1968; 31 (06) 596-605
- 12 Silva J. Impressão basilar: Malformações Occipitocervicais [Basilar impression: Occipitocervical Malformations]. Recife: Editora Universitária-UFPE; 2003. . Portuguese
- 13 McGirt MJ, Nimjee SM, Floyd J, Bulsara KR, George TM. Correlation of cerebrospinal fluid flow dynamics and headache in Chiari I malformation. Neurosurgery 2005; 56 (04) 716-721 , discussion 716–721
- 14 Matsumoto T, Symon L. Surgical management of syringomyelia--current results. Surg Neurol 1989; 32 (04) 258-265
- 15 Saez RJ, Onofrio BM, Yanagihara T. Experience with Arnold-Chiari malformation, 1960 to 1970. J Neurosurg 1976; 45 (04) 416-422
- 16 Mauer UM, Gottschalk A, Mueller C, Weselek L, Kunz U, Schulz C. Standard and cardiac-gated phase-contrast magnetic resonance imaging in the clinical course of patients with Chiari malformation Type I. Neurosurg Focus 2011; 31 (03) E5
- 17 Gilman S. Joint position sense and vibration sense: anatomical organisation and assessment. J Neurol Neurosurg Psychiatry 2002; 73 (05) 473-477
- 18 Calne DB, Pallis CA. Vibratory sense: a critical review. Brain 1966; 89 (04) 723-746
- 19 Netsky MG. Syringomyelia; a clinicopathologic study. AMA Arch Neurol Psychiatry 1953; 70 (06) 741-777
- 20 Doppman JL, Girton M, Popovsky MA. Acute occlusion of the posterior spinal vein. Experimental study in monkeys. J Neurosurg 1979; 51 (02) 201-205
- 21 Henderson FC, Crockard HA, Stevens JM. Spinal cord oedema due to venous stasis. Neuroradiology 1993; 35 (04) 312-315
- 22 Taylor AR, Byrnes DP. Foramen magnum and high cervical cord compression. Brain 1974; 97 (03) 473-480
- 23 Schmidt BJ. Subacute delayed ascending myelopathy after low spine injury: case report and evidence of a vascular mechanism. Spinal Cord 2006; 44 (05) 322-325
- 24 Levine DN. The pathogenesis of syringomyelia associated with lesions at the foramen magnum: a critical review of existing theories and proposal of a new hypothesis. J Neurol Sci 2004; 220 (1-2): 3-21
- 25 Tseng J-S, Lee Y-C, Pan H-C, Chang M-H. Motor neuron disease-like syndrome secondary to trapped fourth ventricle and obstruction of cerebrospinal fluid pathway. Clin Neurol Neurosurg 2007; 109 (04) 383-387
- 26 Ravaglia S, Bogdanov EI, Pichiecchio A, Bergamaschi R, Moglia A, Mikhaylov IM. Pathogenetic role of myelitis for syringomyelia. Clin Neurol Neurosurg 2007; 109 (06) 541-546
- 27 Silva J, Silva E, Nóbrega P. Pyramidal liberation syndrome as clinical expression of basilar impression: report of 2 operated cases. Arq bras neurocir 1990; 9:161–7
- 28 Kahn EA. The role of the dentate ligaments in spinal cord compression and the syndrome of lateral sclerosis. J Neurosurg 1947; 4 (03) 191-199
- 29 Canelas HM, Tenuto RA, Zaclis J, Cruz OR. [Occipito-cervical deformities; 20 new cases]. Arq Neuropsiquiatr 1956; 14 (01) 1-27
- 30 Layzer RB. Myeloneuropathy after prolonged exposure to nitrous oxide. Lancet 1978; 2 (8102): 1227-1230
- 31 Prabhakar NR, Pieramici SF, Premkumar DR, Kumar GK, Kalaria RN. Activation of nitric oxide synthase gene expression by hypoxia in central and peripheral neurons. Brain Res Mol Brain Res 1996; 43 (1-2): 341-346