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Images in Neurology |

Idiopathic Superficial Siderosis

Thiago Cardoso Vale, MD; Rodrigo Santiago Gomez, MD; Antônio Lúcio Teixeira, PhD
Arch Neurol. 2011;68(10):1334-1335. doi:10.1001/archneurol.2011.566.
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A 49-year-old man presented with a 6-year history of progressive worsening of gait, speech, and hearing. Results of a neurological examination revealed a cerebellar ataxia with wide-based unsteady gait, dysarthria, moderate bilateral auditory loss, mild cognitive impairment, and bilateral Babinski signs. The initial laboratory workup produced normal results for a routine blood chemistry screen, thyroid and liver function tests, vitamin B12 and folic acid levels, and serologic samples for human immunodeficiency, human T-lymphotropic, herpes simplex, and hepatitis viruses, as well as for syphilis and bacterial and parasitological infections. Nontraumatic and reddish cerebrospinal fluid (CSF) analysis showed a white blood cell count of 5/μL, 186 mg/dL of proteins, a normal glucose level, and a red blood cell count of 11 230 × 106/μL. (To convert the white blood cell count to No. of cells ×109 per liter, multiply by 0.001; to convert the red blood cell count to No. of cells ×1012 per liter, multiply by 1.) The CSF fluid did not change its color while collection tubes were filled. Cranial computed tomography (CT) results were normal, and brain magnetic resonance (MR) imaging (Figure) showed hemosiderin deposition around the midbrain and cerebellar folia. Cerebral and spinal cord angiography results were unremarkable, and no cause for the hemosiderin deposition was detected.

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Figure. Magnetic resonance (MR) imaging of the brain in our patient. A, T2-weighted sagittal brain MR imaging shows hemosiderin deposition (red arrows) in the midbrain, retrosplenium, mesencephalic tegmentum, and cingulate sulcus. B, Fluid-attenuated inversion recovery–sequenced axial brain MR imaging shows hemosiderin deposition in the cerebellar folia and pons (red arrows).




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