Focused Neuro-Otological Review of Superficial Siderosis of the Central Nervous System

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iSS can be associated with significant neurotologic and cerebellar morbidity; the recurrent SAH variant is frequently clinically symptomatic, has a shorter latency and greater neurotologic disability. In these cases, a thorough search and management of a covert source of bleeding may stop clinical progression. The frequency and clinical course of radiographic iSS after traumatic and post-aneurysmal SAH is largely unknown. Detection of radiographic iSS after trauma or aneurysm bleeding suggests that the slower clinical course could benefit from an effective intervention if it became available. The use of cochlear implants is a valid alternative with advanced hearing impairment.

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A Comprehensive Neuropsychological Assessment of a Case of Superficial Siderosis

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Superficial siderosis (SS) is a neurodegenerative condition due to the long-term effects of hemosiderin deposition on the sur- face 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 stud- ies have become more prevalent. To our knowledge, however, this is the first report of SS detailing a comprehensive neuropsychological assessment.

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Superficial Siderosis of the Central Nervous System Secondary to Chronic Bleeding From a Lumbar Paraganglioma

Case Study involving dementia A 73‐year‐old Caucasian male presented to the Hamilton General Hospital Emergency Room with confusion, slurred speech, lower extremity weakness and gait ataxia. According to the patient’s family, his symptoms began five years prior to this episode, when they noticed behavioral changes. He became aloof and introverted. He also complained of ‘ringing’ in his ears. An auditory examination at that time demonstrated hearing loss, for which he started wearing hearing aids. Subsequently, there was a progressive decline in his functional mobility and other activities of daily living. Upon physical examination, the patient was uncooperative. He had a short attention span with brief moments where he demonstrated mental competence. A Folstein mini mental status exam revealed baseline cognitive decline most likely due to his dementia (13/30). The cranial nerves examination demonstrated bilateral hearing loss. He had lower extremity weakness (more pronounced on the right than on the left),

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Cognitive and social impairments in patients with superficial siderosis

Superficial siderosis of the CNS is a rare condition, caused
by deposition of haemosiderin in the superficial layers
of the CNS due to repeated chronic subarachnoid or intraventricular
hemorrhage. Typically, the hindbrain structures,
especially the cerebellum, are most affected. There
is a surprising lack of studies investigating in detail the
behavioral functioning of patients with such a condition.
In this study, we document for the first time the cognitive,
social and emotional processing of six patients with a
confirmed clinical diagnosis of superficial siderosis.

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