Article (Scientific journals)
CNS Superficial Siderosis Mimicking a Motor Neuron Disease.
Castro-Gomez, Sergio; Binder, Julius; Schievelkamp, Arndt-Hendrik et al.
2022In Brain Sciences, 12 (11)
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Keywords :
ALS-mimics; case report; hemosiderin accumulation; motoneuron disease; spinal MRI; superficial siderosis
Abstract :
[en] Superficial siderosis of the central nervous system (SS-CNS) is a rare condition characterized by a hemosiderin accumulation along the subpial surfaces and arises from an intermittent chronic bleeding in the subarachnoid space usually as a result of a chronic subarachnoid hemorrhage by trauma, vascular malformations, CNS tumors, or cerebral amyloid angiopathy (CAA). We present a 61-year-old male with a 12-year history of limb weakness, muscle wasting, cramps, clumsiness, progressive unsteady gait, and fine motor impairments. His medical history included the resection of a left parietal meningioma and a myxopapillary ependymoma near the conus terminalis (L3/4) at the age of 51 years. The clinical examination revealed a motor neuron syndrome with a clear bilateral wasting of the hand muscles, a diffuse atrophy of the shoulder and calf muscles, and a weakness of the arms, fingers, hips, and feet. Deep tendon reflexes were symmetrically briskly hyperactive. Standing and walking were only possible with a support. Magnetic resonance imaging of the entire neuroaxis showed progressive severe cerebral, brainstem, and spinal superficial siderosis in form of extensive hypointensities on T2-weighted gradient-echo images and susceptibility-weighted sequences. Despite a successful neurosurgical removal of the tumors and delaed medical treatment with an iron chelator for one year, we observed no clinical recovery or stability in our patient, making this case unique, and suggesting an irreversible neurodegenerative process. This case reinforces the need of including SS-CNS in the list of amyotrophic lateral sclerosis (ALS)-mimics and demonstrates the fundamental use of a complete neuraxial MRI investigation on evaluating possible ALS cases.
Disciplines :
Neurology
Author, co-author :
Castro-Gomez, Sergio
Binder, Julius
Schievelkamp, Arndt-Hendrik
Heneka, Michael  ;  University of Luxembourg > Luxembourg Centre for Systems Biomedicine (LCSB)
External co-authors :
yes
Language :
English
Title :
CNS Superficial Siderosis Mimicking a Motor Neuron Disease.
Publication date :
2022
Journal title :
Brain Sciences
ISSN :
2076-3425
Publisher :
MDPI AG, Basel, Switzerland
Volume :
12
Issue :
11
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBilu :
since 25 January 2023

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