Flail arm syndrome mimic caused by hemosiderin deposition in the anterior horn
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LETTER TO THE EDITOR
Flail arm syndrome mimic caused by hemosiderin deposition in the anterior horn Kazushi Deguchi1 · Naomi Honjo2 · Tadayuki Takata1 · Tetsuo Touge3 · Tsutomu Masaki1 Received: 31 July 2020 / Accepted: 26 August 2020 © Belgian Neurological Society 2020
Keywords Flail arm syndrome · Superficial siderosis · Hemosiderin · Anterior horn cells Ventral longitudinal intraspinal fluid collection (VLIFC) with [1] or without [2] superficial siderosis (SS) may cause bibrachial or multisegmental amyotrophy. Amyotrophy localized in the proximal part of both upper limbs mimics flail arm syndrome (FAS), which is a variant of amyotrophic lateral sclerosis (ALS). Although lower motor neuron deficits suggest an anterior horn cell pathology, the involvement of direct anterior horn cell damage remains unclear [3]. We present a case of FAS mimic due to VLIFC with SS. This case suggests that anterior horn cells impaired due to hemosiderin deposition might play a role in the development of FAS mimic. A 56-year-old man developed difficulty in raising his left upper limb. One year later, he was referred to our hospital. Neurological examination showed muscle weakness and atrophy of the bilateral deltoid and biceps with fasciculation in the left biceps. The patient’s tendon reflexes were preserved and his plantar responses were flexor. Electromyography revealed chronic denervation changes in the cervical myotomes but not in the brainstem, thoracic and lumbosacral myotomes. The weakness in the patient’s deltoid and biceps worsened very slowly, suggesting FAS. At age 67, he presented with repeated short-lasting visual deficits. T2*-weighted MRI (T2*WI) of the brain showed a rim of marked hypointensity surrounding the auditory nerve, cerebellum and midbrain (Fig. 1). Sagittal MRI of the spinal * Kazushi Deguchi [email protected]‑u.ac.jp 1
Department of Gastroenterology and Neurology, Kagawa University Faculty of Medicine, 1750‑1 Ikenobe, Miki‑cho, Kita‑gun, Kagawa 761‑0793, Japan
2
Department of Neuroradiology, Osaka Neurosurgical Hospital, Kagawa, Japan
3
Department of Health Sciences, Kagawa University Faculty of Medicine, Kagawa, Japan
cord showed a VLIFC from C2 through T12, obscured at C5/6 by a degenerative bulging disc with osteophytes. Axial T2*WI demonstrated hypointensity in accordance with the anterior horn from C4 to C5. Mild ventral cord flattening and posterior displacement of the cord were also observed (Fig. 2). At the time of imaging, the affected region was localized in the bilateral deltoid and biceps, and auditory disturbance, cerebellar ataxia and pyramidal signs were inapparent. Although the patient’s history of bilateral chronic subdural hematoma after a head injury at age 40 suggested a cerebrospinal fluid (CSF) leak from a dural tear [4], dynamic CT myelography and digital subtraction myelography were not performed because of complications related to a thoracic and abdominal dissecting aortic aneurysm. This patient was diagnosed with bibrachial amyotrophy due to VLIFC with SS. Al
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