Benign monomelic amyotrophy: a study of twenty-one cases Amiotrofia monomélica benigna: estudo de 21 casos
A consecutive series of 21 patients with single limb atrophy (monomelic amyotrophy) is reported. Sixteen had lower limb atrophy and five had upper limb involvement. The median age of the onset was 20 years. Characteristic features were sporadic occurrence, wasting confined to one limb, insidious ons...
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Thieme Revinter Publicações
2000-09-01
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| Series: | Arquivos de Neuro-Psiquiatria |
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| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2000000500003 |
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| author | MARCOS R.G. DE FREITAS OSVALDO J. M. NASCIMENTO |
| author_facet | MARCOS R.G. DE FREITAS OSVALDO J. M. NASCIMENTO |
| author_sort | MARCOS R.G. DE FREITAS |
| collection | DOAJ |
| description | A consecutive series of 21 patients with single limb atrophy (monomelic amyotrophy) is reported. Sixteen had lower limb atrophy and five had upper limb involvement. The median age of the onset was 20 years. Characteristic features were sporadic occurrence, wasting confined to one limb, insidious onset with slow progression, stabilizing in 1 to 4 years, and absence of pyramidal signs. All the patients with upper limb involvement were male, however in our cases with lower limb amyotrophy there were no male preponderance. We observed wasting of the entire length of the lower limbs in six patients. There were nine cases with amyotrophy restricted to the leg and one with amyotrophy only in the thigh. In the upper limb in four cases the involvement was distal and in one patient the atrophy was proximal. The electromyographic features were suggestive of anterior horn disease not only in the affected limb but also, in some cases, in clinically uninvolved limb. Cervical or lumbar MRI was normal. MRI of the lower limb disclosed increased signal intensity in the gastrocnemius and soleus muscles in one patient suggesting denervation.<br>Relatamos uma série consecutiva de 21 pacientes com amiotrofia de um só membro, denominada de amiotrofia monomélica. Em 16 casos a atrofia era no membro inferior e em 5 localizava-se no membro superior. Todos eram jovens e a idade média do início foi 20 anos. Os dados mais característicos da doença foram ausência de história familiar, comprometimento de um só membro, início e progressão lenta estabilizando em até 4 anos e ausência de sinais piramidais. Nossos enfermos com amiotrofia de membro superior eram todos do sexo masculino, entretanto naqueles com amiotrofia do membro inferior havia igualdade de sexos. Dos pacientes com atrofia de membro inferior, em 9 a atrofia era restrita a perna, em 6 era em todo o membro e somente um apresentava amiotrofia localizada só na coxa. Naqueles com comprometimento do membro superior a atrofia era distal em 4 e proximal em 1. Os achados na eletromiografia foram compatíveis com acometimento do II neurônio motor. Todos foram submetidos a RM da coluna cervical ou lombar que se mostrou normal. Em um caso realizamos RM da perna acometida que evidenciou sinais hiperintensos nos músculos gastrocnemius e soleus, o que sugere desnervação. |
| format | Article |
| id | doaj-art-0bda59cd24eb4fbd9d6f88faed0edca3 |
| institution | OA Journals |
| issn | 0004-282X 1678-4227 |
| language | English |
| publishDate | 2000-09-01 |
| publisher | Thieme Revinter Publicações |
| record_format | Article |
| series | Arquivos de Neuro-Psiquiatria |
| spelling | doaj-art-0bda59cd24eb4fbd9d6f88faed0edca32025-08-20T01:59:30ZengThieme Revinter PublicaçõesArquivos de Neuro-Psiquiatria0004-282X1678-42272000-09-01583B80881310.1590/S0004-282X2000000500003Benign monomelic amyotrophy: a study of twenty-one cases Amiotrofia monomélica benigna: estudo de 21 casosMARCOS R.G. DE FREITASOSVALDO J. M. NASCIMENTOA consecutive series of 21 patients with single limb atrophy (monomelic amyotrophy) is reported. Sixteen had lower limb atrophy and five had upper limb involvement. The median age of the onset was 20 years. Characteristic features were sporadic occurrence, wasting confined to one limb, insidious onset with slow progression, stabilizing in 1 to 4 years, and absence of pyramidal signs. All the patients with upper limb involvement were male, however in our cases with lower limb amyotrophy there were no male preponderance. We observed wasting of the entire length of the lower limbs in six patients. There were nine cases with amyotrophy restricted to the leg and one with amyotrophy only in the thigh. In the upper limb in four cases the involvement was distal and in one patient the atrophy was proximal. The electromyographic features were suggestive of anterior horn disease not only in the affected limb but also, in some cases, in clinically uninvolved limb. Cervical or lumbar MRI was normal. MRI of the lower limb disclosed increased signal intensity in the gastrocnemius and soleus muscles in one patient suggesting denervation.<br>Relatamos uma série consecutiva de 21 pacientes com amiotrofia de um só membro, denominada de amiotrofia monomélica. Em 16 casos a atrofia era no membro inferior e em 5 localizava-se no membro superior. Todos eram jovens e a idade média do início foi 20 anos. Os dados mais característicos da doença foram ausência de história familiar, comprometimento de um só membro, início e progressão lenta estabilizando em até 4 anos e ausência de sinais piramidais. Nossos enfermos com amiotrofia de membro superior eram todos do sexo masculino, entretanto naqueles com amiotrofia do membro inferior havia igualdade de sexos. Dos pacientes com atrofia de membro inferior, em 9 a atrofia era restrita a perna, em 6 era em todo o membro e somente um apresentava amiotrofia localizada só na coxa. Naqueles com comprometimento do membro superior a atrofia era distal em 4 e proximal em 1. Os achados na eletromiografia foram compatíveis com acometimento do II neurônio motor. Todos foram submetidos a RM da coluna cervical ou lombar que se mostrou normal. Em um caso realizamos RM da perna acometida que evidenciou sinais hiperintensos nos músculos gastrocnemius e soleus, o que sugere desnervação.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2000000500003neurônio motor inferioramiotrofia monomélicaamiotrofia espinhallower motor neuronmonomelic amyotrophyspinal muscular atrophy |
| spellingShingle | MARCOS R.G. DE FREITAS OSVALDO J. M. NASCIMENTO Benign monomelic amyotrophy: a study of twenty-one cases Amiotrofia monomélica benigna: estudo de 21 casos Arquivos de Neuro-Psiquiatria neurônio motor inferior amiotrofia monomélica amiotrofia espinhal lower motor neuron monomelic amyotrophy spinal muscular atrophy |
| title | Benign monomelic amyotrophy: a study of twenty-one cases Amiotrofia monomélica benigna: estudo de 21 casos |
| title_full | Benign monomelic amyotrophy: a study of twenty-one cases Amiotrofia monomélica benigna: estudo de 21 casos |
| title_fullStr | Benign monomelic amyotrophy: a study of twenty-one cases Amiotrofia monomélica benigna: estudo de 21 casos |
| title_full_unstemmed | Benign monomelic amyotrophy: a study of twenty-one cases Amiotrofia monomélica benigna: estudo de 21 casos |
| title_short | Benign monomelic amyotrophy: a study of twenty-one cases Amiotrofia monomélica benigna: estudo de 21 casos |
| title_sort | benign monomelic amyotrophy a study of twenty one cases amiotrofia monomelica benigna estudo de 21 casos |
| topic | neurônio motor inferior amiotrofia monomélica amiotrofia espinhal lower motor neuron monomelic amyotrophy spinal muscular atrophy |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2000000500003 |
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