Case Report: A Comprehensive Neuropsychological Assessment of a Case of Superficial Siderosis

Superficial siderosis (SS) is a neurodegenerative condition due to the long-term effects of hemosiderin deposition on the surface of the brain, cerebellum, brainstem, and spinal cord. SS symptoms include sensorineural hearing loss, ataxia and upper motor neuron signs. SS was diagnostically evasive u...

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Published in:Archives of clinical neuropsychology 2017-06, Vol.32 (4), p.483-490
Main Authors: Gawryluk, Jodie R, Ritchie, Lesley J, Sicz, George, Kilgour, Andrea R, Schmidt, Brian J
Format: Article
Language:English
Subjects:
Brain Stem - diagnostic imaging
Cerebellum - diagnostic imaging
Cognition Disorders - diagnosis
Female
Gait Disorders, Neurologic - diagnostic imaging
Gait Disorders, Neurologic - etiology
Humans
Magnetic Resonance Imaging
Middle Aged
Neuropsychological Tests
Psychometrics
Siderosis - complications
Siderosis - diagnostic imaging
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Summary:Superficial siderosis (SS) is a neurodegenerative condition due to the long-term effects of hemosiderin deposition on the surface of the brain, cerebellum, brainstem, and spinal cord. SS symptoms include sensorineural hearing loss, ataxia and upper motor neuron signs. SS was diagnostically evasive until magnetic resonance imaging (MRI) became available. As the detection of SS improved, case studies have become more prevalent. To our knowledge, however, this is the first report of SS detailing a comprehensive neuropsychological assessment. The current study presents a right-handed female in her early 60s, with a university level of education, who was diagnosed with SS. Her neuropsychological profile showed impairment across multiple domains, including memory and executive function, with consistent behavioral findings. The results from a comprehensive neuropsychological assessment include dementia and a cerebellar cognitive affective syndrome. Neuropsychological evaluation of a patient with new cognitive impairment in combination with unexplained hearing loss, gait disorder, or myelopathy should lead to a referral for MRI that includes techniques sensitive for iron deposition, in order to rule out SS.
ISSN:0887-6177
1873-5843
DOI:10.1093/arclin/acx012