J Korean Neurol Assoc.  2018 Aug;36(3):210-214. 10.17340/jkna.2018.3.15.

Unilateral Hypoglossal Neuropathy in Multifocal Acquired Demyelinating Sensory and Motor Neuropathy: Differential Diagnosis of Motor Neuron Disease

• Yoo SK
• Kim MO
• Kim CH
• Kim YS
• Kim SK
• Kang H
• Choi NC
• Kwon OY
• Lim BH
• Park KH

• KMID: 2436081
• DOI: http://doi.org/10.17340/jkna.2018.3.15

Abstract

Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) is a peripheral neuropathy characterized by multifocal weakness and associated sensory impairment. MADSAM is associated with multifocal persistent conduction block and other signs of demyelination. The incidence of cranial nerve involvement in MADSAM was recently reported to be approximately 15%. However, reports of hypoglossal neuropathy occurring in MADSAM are rare. Unilateral hypoglossal neuropathy in MADSAM is usually misdiagnosed as motor neuron disease. We report a patient with MADSAM presenting with tongue hemiatrophy.

Keyword

Peripheral demyelinating neuropathy; Motor neuron disease; Hypoglossal nerve diseases

MeSH Terms

Cranial Nerves
Demyelinating Diseases
Diagnosis, Differential*
Humans
Hypoglossal Nerve Diseases*
Incidence
Motor Neuron Disease*
Motor Neurons*
Peripheral Nervous System Diseases
Tongue