학술논문

A case of paraneoplastic neurological syndrome with dizziness and bilateral progressive sensorineural hearing loss / 浮動性めまいと両側進行性感音難聴を呈した傍腫瘍性神経症候群の1例
Document Type
Journal Article
Source
Equilibrium Research. 2021, 80(1):41
Subject
anti-Ma1 antibody
anti-Ma2 (Ta) antibody
dizziness
paraneoplastic neurological syndrome
sensorineural hearing loss
Language
Japanese
ISSN
0385-5716
1882-577X
Abstract
Paraneoplastic neurological syndrome (PNS) is a rare syndrome representing the remote effect of a cancer, that is often associated with the presence of specific serum antibodies. PNS can affect the central, peripheral, and autonomic nervous systems at various levels. Clinically, it is characterized by subacute progression of neurological dysfunction and substantial refractoriness to therapies. Herein, we report a case of PNS with dizziness and bilateral progressive sensorineural hearing loss. A 65-year-old woman complaining of numbness in the left upper extremity, abnormal sensation in the limbs, and dizziness and light-headedness during walking visited our otolaryngology department. At the first visit, she showed pure rotatory nystagmus, and in the stepping test, she almost fell over to the right within a few steps. Thereafter, the right sensorineural hearing loss was recognized, and the nystagmus changed to a left-beating horizontal-torsional nystagmus; the hearing loss progressed even after oral steroid treatment. At week 14 after the first visit, the muscle weakness in her limbs worsened and the patient became unable to move or walk herself, and sensorineural hearing loss developed on the left side. She was admitted to the department of neurology for closer examination. Finally, the diagnosis of PNS was made based on the detection of a squamous cell carcinoma of the lung and positive test result for anti-Ma1 antibody and anti-Ma2 (Ta) antibody. After the steroid pulse therapy and chemotherapy, the tumor shrank, but the neurological symptoms persisted, with only slight improvement. The patient died ten and a half months after her first visit to our department.