Doença de Hirayama: relato de caso e atualização
DOI:
https://doi.org/10.34024/rnc.2007.v15.8696Palavras-chave:
Medula espinhal, Células do corno anterior, Doenças neuromuscularesResumo
A Atrofia Muscular Juvenil Distal de Membro Superior ou Doença de Hirayama (DH) atinge as células da ponta anterior da medula nos segmentos de C7-T1 sendo caracterizada por atrofia com fraqueza progressiva da mão e antebraço unilateral na maioria dos indivíduos. Relatamos o caso de um paciente, que há 4 anos iniciou um quadro de fraqueza na mão esquerda associada à dificuldade para realizar a preensão de determinados instrumentos utilizados no trabalho. Após a realização da ressonância magnética funcional verificou-se um comprometimento da medula cervical nos últimos segmentos cervicais e primeiro torácico, caracterizando a doença. Diante de suas características gerais, podemos diferenciá-la de outras Amiotrofias Monomélicas Benignas (AMB). É importante que se realize novas pesquisas visando tornar o diagnóstico mais preciso.
Referências
Ito S, Kuwabara S, Fukutake T, Tokumaru Y, Hattori T. HyperIgEaemia in patients with juvenile muscular atrophy of the distal upper extremity (Hirayama disease). J Neurol Neurosurg Psychiatr 2005;76(1):132-4. DOI: https://doi.org/10.1136/jnnp.2003.031609
Kwon O, Kim M, Lee KW. A Korean case of Juvenile Muscular Atrophy of distal upper extremity (Hirayama Disease) with dynamic Cervical Cord compression. J Korean Med Sci 2004;19:768-71. DOI: https://doi.org/10.3346/jkms.2004.19.5.768
Felice KJ, Whitaker CH, Grunnet ML. Benign Calf Amyotrophy – Clinicopathologic Study of 8 Patients. Arch Neurol 2003;60:1415-20. DOI: https://doi.org/10.1001/archneur.60.10.1415
Chen CJ, Chen CM, Wu CL, Ro LS, Chen ST, Lee TH. Hirayama disease: MR diagnosis. AJNR Am J Neuroradiol 1998; 19:365-8.
Tashiro K, Kikuchi S, Itoyama Y, Tokumaru Y, Sobue G, Mukai E, et al. Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotroph Lateral Scler Other Motor Neuron Disord 2006;7(1):38-45. DOI: https://doi.org/10.1080/14660820500396877
Gourie-Devi M, Nalini A. Long-term follow-up of 44 patients with Brachial Monomelic Amyotrophy. Acta Neurol Scand 2003;107:215-20. DOI: https://doi.org/10.1034/j.1600-0404.2003.02142.x
Gandhi D, Goyal M, Bourque PR, Jain R. Case 68: Hirayama disease. Radiology 2004; 230;692-6. DOI: https://doi.org/10.1148/radiol.2303021089
Chen CJ, Hsu HL, Tseng YC, Lyu RK, Chen CM, Huang YC, et al. Hirayama Flexion Myelopathy: Neutral — Position MR Imaging Findings — Importance of Loss of Attachment. Radiology 2004;231:39-44. DOI: https://doi.org/10.1148/radiol.2311030004
Nascimento OJM, Freitas MRG. Non-progressive Juvenile Spinal Muscular Atrophy of distal upper limb (Hirayama’s disease) — a clinical variant of the benign monomelic amyotrophy. Arq Neuropsiquiatr 2000;58:814-9. DOI: https://doi.org/10.1590/S0004-282X2000000500004
Freitas MRG, Nascimento OJM. Benign Monomelic Amyotrophy — A study of twenty one cases. Arq Neuropsiquiatr 2000;58:808-13. DOI: https://doi.org/10.1590/S0004-282X2000000500003
Jeannet PY, Kuntzer T, Deonna T, Roulet-Perez E. Hirayama disease associated with a severe rhythmic movement disorder involving neck flexions. Neurology 2005;64:1478-9. DOI: https://doi.org/10.1212/01.WNL.0000158678.17161.1B
Fetoni V, Briem E, Carrara F, Mora M, Zeviani M. Monomelic amyotrophy associated with the 7472insC mutation in the mtDNA tRNASer(UCN) gene. Neuromuscul Disord 2004;14:723-6. DOI: https://doi.org/10.1016/j.nmd.2004.07.002
Van den Berg-Vos RM, Visser J, Franssen H, Visser M, De Jong JMB, Kalmijn S. Sporadic lower motor neuron disease with adult onset: classification of subtypes. Brain 2003;126(5):1036-47. DOI: https://doi.org/10.1093/brain/awg117
Hirayama K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease). Intern Med 2000;39:283-90. DOI: https://doi.org/10.2169/internalmedicine.39.283
Hirayama K, Tokumaru Y. Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology 2000;54:1922-6. DOI: https://doi.org/10.1212/WNL.54.10.1922
Okumura H, Homma TT. Juvenile compression myelopathy in the cervical spine. Spine 1994;19:72-6. DOI: https://doi.org/10.1097/00007632-199401000-00016
Willeit J, Kiechl S, Kiechl-Kohlendorfer U, Golaszewski S, Peer S, Poewe W. Juvenile asymmetric segmental spinal muscular atrophy (Hirayama’s disease). Acta Neurol Scand 2001;104:320-2. DOI: https://doi.org/10.1034/j.1600-0404.2001.00074.x
Umphred DA. Fisioterapia neurológica. São Paulo: Manole, 1994.
