Anterior horn damage in brachial multisegmental amyotrophy with superficial siderosis and dural tear: an autopsy case report

• Published in: BMC neurology Vol. 23; no. 1; p. 129
• Main Authors: Takahashi, Yusuke, Kodaira, Minori, Yamada, Mitsunori, Uehara, Kai, Ito, Kiyoshi, Kaneko, Tomoki, Ohashi, Hiroki, Yazaki, Masahide, Sekijima, Yoshiki
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 29.03.2023; BioMed Central; BMC
• Subjects: Age; Analysis; Anterior horn; Ataxia; Atrophy; Atrophy, Muscular; Autopsies; Autopsy; Brachial multisegmental amyotrophy; Care and treatment; Case Report; Case reports; Central nervous system; Central nervous system diseases; Compression; Denervation; Diagnosis; Dural tear; Electromyography; Gray Matter; Humans; Magnetic resonance imaging; Male; Middle Aged; Pathophysiology; Patients; Siderosis; Siderosis - complications; Siderosis - diagnostic imaging; Snake-eyes appearance; Spinal cord; Spinal Cord Compression - complications; Spinal Cord Compression - diagnostic imaging; Stenosis; Substantia grisea; Superficial siderosis; Thorax; Japan; Snake-eyes appearance; Dural tear; Superficial siderosis; Anterior horn; Brachial multisegmental amyotrophy
• ISSN: 1471-2377; 1471-2377
• DOI: 10.1186/s12883-023-03180-z

Patients with superficial siderosis (SS) rarely show brachial multisegmental amyotrophy with ventral intraspinal fluid collection accompanied with dural tear. We describe spinal cord pathology of a 58-year-old man who developed brachial multisegmental amyotrophy with ventral intraspinal fluid collection from the cervical to lumbar spinal levels accompanied with SS, dural tear, and snake-eyes appearance on magnetic resonance imaging (MRI). Radiological and pathological analyses detected diffuse and prominent superficial deposition of hemosiderin in the central nervous system. Snake-eyes appearance on MRI expanded from the C3 to C7 spinal levels without apparent cervical canal stenosis. Pathologically, severe neuronal loss at both anterior horns and intermediate zone was expanded from the upper cervical (C3) to middle thoracic (Th5) spinal gray matter, and these findings were similar to compressive myelopathy. Extensive damage of the anterior horns in our patient may be due to dynamic compression induced by ventral intraspinal fluid collection.