Neurology: Case of the Month

Test Yourself — Patient 46

Superficial siderosis (Superficial hemosiderosis) of the CNS

1. The two most common symptoms reported in cases of superficial siderosis (SS) are:
   • [ A ] Hearing loss and myelopathy
   • [ B ] Hearing loss and ataxia
   • [ C ] Ataxia and dementia
   • [ D ] Dementia and myelopathy
   • [ E ] Ataxia and optic neuritis
2. The hemosiderin deposition found in cases of SS is most likely due to:
   • [ A ] Systemic iron overload
   • [ B ] A single large subarachnoid hemorrhage
   • [ C ] Chronic, repeated subarachnoid hemorrhage
   • [ D ] Intraparenchymal hemorrhage
3. In a patient with progressive ataxia and hearing loss, the clinical feature MOST consistent with a diagnosis of superficial siderosis (SS) is:
   • [ A ] Autosomal dominant family history
   • [ B ] History of epidural hematoma resulting from head trauma
   • [ C ] Cerebrospinal fluid xanthochromia
   • [ D ] Sudden onset of symptoms
   • [ E ] MRI evidence of iron deposition in the globus pallidus and putamen
4. If MRI of the brain is performed, the best sequences to obtain are:
   • [ A ] T1 weighted images with contrast
   • [ B ] T2 weighted, spin-echo images
   • [ C ] T2 weighted, fast spin-echo images
   • [ D ] T2 weighted, gradient-echo images
   • [ E ] Fluid attenuated inversion recovery (FLAIR) images
5. The most sensitive and specific finding for superficial siderosis (SS) on MRI of the brain is:
   • [ A ] Meningeal enhancement
   • [ B ] A hyperintense rim around the brainstem and/or cerebellum on FLAIR images
   • [ C ] A hypointense rim around the brainstem and/or cerebellum on T2 weighted images
   • [ D ] An arachnoid cyst in the posterior fossa
   • [ E ] Multiple cranial nerve enhancement, particularly the eighth cranial nerve

 

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