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• Vol.61 No.2
• CLINICA NEUROL, 61: 115−119, 2021
• Vol.61 No.2 contents

Case Report

A case of facial-onset sensory and motor neuronopathy (FOSMN) with cerebellar ataxia and abnormal decrement in repetitive nerve stimulation test

Daisuke Yamamoto, M.D., Ph.D.¹⁾, Minoru Yamada, M.D.¹⁾, Bo Cao, M.D.¹⁾, Syuuichirou Suzuki, M.D., Ph.D.¹⁾, Shin Hisahara, M.D., Ph.D.¹⁾ and Shun Shimohama, M.D., Ph.D.¹⁾

¹⁾Department of Neurology, Sapporo Medical University School of Medicine

A 59-year-old woman presented with a 7-year history of facial numbness on the left side, and gradual worsening of symptoms. Over several years, facial muscle weakness, dysarthria, tongue atrophy and fasciculation had progressed. Then, she developed cerebellar ataxia affecting the left extremities, in addition to earlier symptoms. Brain MRI revealed cerebellar atrophy, and 99mTc-SPECT depicted cerebellar hypoperfusion. A repetitive nerve stimulation test (RNS) indicated abnormal decrement in the nasalis and trapezius muscles on the left side. Facial-onset sensory and motor neuronopathy (FOSMN) was diagnosed. Administration of intravenous immunoglobulin resulted in improvement of some symptoms. Although cerebellar ataxia is not a common symptom of FOSMN, a case showing TDP-43-positive glial cytoplasmic inclusions in cerebellar white matter has been reported. Therefore, it is possible that FOSMN may cause cerebellum impairment in some patients. Furthermore, RNS positive rate in the trapezius muscle is known to be high in amyotrophic lateral sclerosis (ALS) patients. It is speculated that RNS of the affected muscles in FOSMN may show abnormal decrement by the same mechanisms as ALS.
Full Text of this Article in Japanese PDF (4778K) 　　　　 Full Text of this Article in English HTML

(CLINICA NEUROL, 61: 115−119, 2021)
key words: facial-onset sensory and motor neuronopathy, cerebellar ataxia, cerebellar atrophy, repetitive nerve stimulation, abnormal decrement

(Received: 30-Jun-20)