Superficial cerebral siderosis as strokemimic
Francesco Brigo, Markus Covi, Raffaele Nardone, Frediano Tezzon & Piergiorgio Lochner Acta Neurologica Belgica ISSN 0300-9009 Acta Neurol Belg DOI 10.1007/s13760-013-0218-2
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Author's personal copy Acta Neurol Belg DOI 10.1007/s13760-013-0218-2
LETTER TO THE EDITOR
Superficial cerebral siderosis as stroke-mimic Francesco Brigo • Markus Covi • Raffaele Nardone Frediano Tezzon • Piergiorgio Lochner
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Received: 5 March 2013 / Accepted: 7 June 2013 Ó Belgian Neurological Society 2013
Dear Editor, Superficial siderosis of the central nervous system results from hemosiderin deposition in the subpial layers of the brain and spinal cord [1]. A clinical history of subarachnoid hemorrhage is often absent, and in most cases patients present with slowly progressive gait ataxia and sensorineural hearing impairment [1]. We describe a patient diagnosed with superficial cortical siderosis presenting with sudden occurrence of aphasia and facial palsy mimicking a cerebrovascular event. A 87-year-old man presented with expressive aphasia and hemisensory loss involving right hand and face. No headache was reported. His previous medical history was notable for high blood pressure treated with valsartan 80 mg/day. Neurological examination revealed a mild expressive aphasia with right central facial palsy. A CT head without contrast showed a rim of cortical hyperdensity on the left-side central sulcus (Fig. 1a). T2weighted and gradient-echo magnetic resonance imaging (MRI) showed a T2 hypointensity along the cortical sulci of both sides, indicative of hemosiderin deposition
F. Brigo (&) Section of Clinical Neurology, Department of Neurological, Neuropsychological, Morphological and Movement Sciences, University of Verona, Piazzale L.A. Scuro 10, 37134 Verona, Italy e-mail:
[email protected] F. Brigo M. Covi R. Nardone F. Tezzon P. Lochner Department of Neurology, Franz Tappeiner Hospital, Merano, Italy R. Nardone Department of Neurology, Christian Doppler Clinic, Paracelsus Medical University, Salzburg, Austria
(Fig. 1b). Diffusion-weighted MRI, performed when neurological deficits were still present, showed no sign of recent ischemia and MR-angiography did not show evidence of a vascular malformation or aneurysm. Spin-echo and gradient-echo sequences failed to reveal any source for bleeding such as amyloid angiopathy or cavernomas, and no evidence of hemosiderin deposition in posterior fossa was found. Since the presence of aphasia pointed to a cortical disturbance, a CSF examination was not performed; however, a cervical spine MR with contrast did not show any vascular abnormality. Laboratory examinations, duplex ultrasound imaging and EEG revealed no abnormalities. Within 2 days the aphasia and facial palsy recovered. A diagnosis of superficial cerebral siderosis was made and the patient discharged. Superficial siderosis of the central nervous system is characterized by hemosiderin deposition in those parts of the central nervous system adjacent to cerebrospinal fluid, with following discoloration of the leptomeninges and adjacent parenchyma. The most involved sites are the superior vermis, cerebellar folia, basal frontal lobe, temporal cortex, brainstem, spinal cord, and cranial nerves I and VIII [1]. The clinical features are related to the site of hemosiderin deposition, and in most cases patients present with slowly progressive cerebellar ataxia (often with dysarthria), often associated with hearing loss (sometimes with vestibular failure), which may be accompanied by tinnitus [2]. However, also pyramidal and sensory signs are common and may relate to brainstem or spinal cord involvement. Sensory symptoms or a sensory level are rarely present [1]. Hemosiderin deposition around the brain, brainstem and spinal cord results in the characteristic hypointensity seen on T2-weighted MRI. Gradient-echo T2-weighted images
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hyperdensity led to further neuroradiological investigations. MRI was therefore performed and its findings ruled out a cerebral ischemia, pointing to a superficial cortical siderosis. The reported case suggests therefore that, although rarely, superficial siderosis of the central nervous system may present with neurological deficits which occur suddenly, rather than evolving slowly. Superficial cerebral siderosis should therefore be taken into consideration in the differential diagnosis of cerebrovascular accidents as in some cases it may mimic stroke. None.
Fig. 1 a CT head showing a rim of cortical hyperdensity over the left-side central sulcus (arrow); b gradient-echo T2-weighted MRI: hypointensity along the cortical sulci of both sides, indicative of hemosiderin deposition
Conflict of interest
have higher sensitivity for hemosiderin deposition and may easily lead to a correct diagnosis, due to the magnetic susceptibility effects of blood degradation products, such as ferritin and hemosiderin, detected by these MRI sequences [3]. In our patient the sudden onset of neurological deficits was highly suggestive of a cerebrovascular event. However, the presence of a left-sided rim of cortical
1. Kumar N (2007) Superficial siderosis: associations and therapeutic implications. Arch Neurol 64:491–496 2. Kumar N, Cohen-Gadol AA, Wright RA, Miller GM, Piepgras DG, Ahlskog JE (2006) Superficial siderosis. Neurology 66:1144–1152 3. Offenbacher H, Fazekas F, Schmidt R, Kapeller P, Fazekas G (1996) Superficial siderosis of the central nervous system: MRI findings and clinical significance. Neuroradiology 38:S51–S56
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