MRI findings of benign monomelic amyotrophy of lower limb

Hamano, Tadanori; Mutoh, Tatsuro; Hirayama, Masaaki; Ito, Keita; Kimura, Masaichi; Aita, Takashi; Kiyosawa, Kazuhiro; Ohtaki, Tetsuo; Kuriyama, Masaru

doi:10.1016/s0022-510x(99)00086-6

Cited by 20 publications

(18 citation statements)

References 6 publications

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“…This image suggests lack of muscular tissue due to fibroadipose substitution 18 . Our findings were similar with the recently described by Hamano et al 19 . As the combination of clinical and electromyographic examinations could not detect mild involvement in deep skeletal muscles, MRI muscle examination is very useful for detecting such affected muscles.…”

Section: Fig 3 Case 21 -Mri Of Calf Note the Selective Asymmetricasupporting

confidence: 94%

“…Computed tomography (CT) and MRI of skeletal muscles involved can offer useful additional information on muscle involvement in MA 2,7,18,19 . The CT scanning of muscles showed fibroadipous substitution of posterior muscles in the affected leg, but sometimes there was a selective involvement of the gastrocnemius medialis muscle in the clinically unaffected limb 2 .…”

Section: Fig 3 Case 21 -Mri Of Calf Note the Selective Asymmetricamentioning

confidence: 99%

“…Only one patient had an attenuation of the gastrocnemius medialis muscle in the clinically unaffected leg. Hamano et al 19 reported 2 patients with BMA of the distal lower limb, in which they performed a MRI of the lower limb. In both the T1-and T2-weight MRI showed increased signal intensities in the semimembranous, semitendinous, vastus intermedius muscles, as well as the soleus and medial gastrocnemius muscles on the affected limb on the right side.…”

Section: Fig 3 Case 21 -Mri Of Calf Note the Selective Asymmetricamentioning

confidence: 99%

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Benign monomelic amyotrophy: a study of twenty-one cases

Freitas

Nascimento

2000

Arq. Neuro-Psiquiatr.

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-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.KEY WORDS: lower motor neuron, monomelic amyotrophy, spinal muscular atrophy. Amiotrofia monomélica benigna: estudo de 21 casosRESUMO -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. PALAVRAS-CHAVE: neurônio motor inferior, amiotrofia monomélica, amiotrofia espinhal.

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Section: Fig 3 Case 21 -Mri Of Calf Note the Selective Asymmetricasupporting

confidence: 94%

Section: Fig 3 Case 21 -Mri Of Calf Note the Selective Asymmetricamentioning

confidence: 99%

Section: Fig 3 Case 21 -Mri Of Calf Note the Selective Asymmetricamentioning

confidence: 99%

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Benign monomelic amyotrophy: a study of twenty-one cases

Freitas

Nascimento

2000

Arq. Neuro-Psiquiatr.

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“…In the conduction velocity studies that we performed in this case, we found that the left median and ulnar temporal disperse responses and CMAP amplitude were diminished and that there were no F responses. This change in conduction velocity and amplitudes can be interpreted as a consequence of denervation in affected muscles (13). EMG states typical neurogenic symptoms conforming to the anterior horn cell involvement in atrophic muscles.…”

Section: Discussionmentioning

confidence: 85%

“…However, atypical cases showing decrease in CMAP amplitudes and slowing down in conduction velocity have also been reported. In the study conducted by Hamano et al (13), it was noted that there is a decrease in the conduction velocity and CMAP amplitude in the affected limb. In the conduction velocity studies that we performed in this case, we found that the left median and ulnar temporal disperse responses and CMAP amplitude were diminished and that there were no F responses.…”

Section: Discussionmentioning

confidence: 93%

Hirayama Disease

Karadeniz¹,

Boyraz²,

Öznam³

et al. 2015

Turk J Phys Med Rehab

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Hirayama disease (HD) is a rare benign lower motor neuron disorder in the young with a male preponderance and usually affecting one upper extremity. It is characterized by the insidious onset and progressive weakness and wasting of a distal extremity. Generally, this disease is considered as a benign and nonprogressive motor neuron disease that stabilizes within five years of onset. We describe a 20-year-old male patient who experienced left distal upper extremity amyotrophy with no sensorial abnormality.

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Benign monomelic amyotrophy of lower limb in a cohort of chinese patients

et al. 2021

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Background Benign monomelic amyotrophy of lower limb (BMALL) is a neurogenic syndrome representing an unclear field. Further studies might be helpful to elucidate uncertainties regarding causation, outcome, and the risk of progression to amyotrophic lateral sclerosis (ALS). Methods According to the inclusion and exclusion criteria, 37 patients with BMALL were retrospectively collected in three neuromuscular centers from January 2012 to October 2018. The detailed medical data were summarized. Multiple laboratory tests were examined. Routine electrophysiological examinations, muscle MRI of lower limbs, and muscle biopsy were conducted. Results The cohort included 24 male and 13 female cases with median age of onset 47 years. Muscle MRI revealed that the distribution of involved muscles matched with the extent of fat infiltration, so the pattern muscle atrophy can be divided into the following four types: six patients with thigh atrophy (type I), 14 patients with leg atrophy (type II); 10 patients with disproportionate atrophy in both thigh and leg (type III); and seven patients with well‐proportionate atrophy in both thigh and leg (type IV). Electrophysiological findings showed neurogenic pattern, spontaneous activity, and abnormal H reflex, which suggested a disorder of spinal anterior horn cell in the patients with types I‐III. However, no electrophysiological abnormalities were found in the patients with type IV. Muscle pathology varied from almost normal pattern to advanced neurogenic pattern in nine biopsied patients. Follow‐up showed that two patients with type II developed to ALS four years later, and all patients with type IV were in stable condition without any complaints. Conclusion Muscle MRI was useful to exactly localize the distribution of involved muscles in BMALL patients. The distribution of atrophic muscles can be roughly divided into four types based on the MRI features. The classification of distributing types might be as an indicator for the prognosis of BMALL.

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MRI findings of benign monomelic amyotrophy of lower limb

Cited by 20 publications

References 6 publications

Benign monomelic amyotrophy: a study of twenty-one cases

Benign monomelic amyotrophy: a study of twenty-one cases

Hirayama Disease

Benign monomelic amyotrophy of lower limb in a cohort of chinese patients

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