INTRODUCTION: Superficial siderosis of the central nervous system (CNS) is an uncommon neurological condition, characterized clinically by cerebellar ataxia, neurosensorial deafness, anosmia, myelopathy and cognitive deterioration. It is associated with the deposition of haemosiderin in the subpial layers of the brain, cerebellum and spinal cord, following chronic bleeding (often clinically silent) in the subarachnoid space. Histopathologically there is gliosis, neurone loss and demyelination of the CNS. CLINICAL CASE: We present the case of a 60 year old woman with a history of progressive worsening of a disorder of myelopathic type with signs of pyramidal liberation and sphincter incontinence associated with cerebellar ataxia, anosmia and bilateral hypoacusia. Initially she had unsteadiness, frequent falls and weakness of the legs. Her deafness was more obvious during the previous year. On lumbar puncture there was hemorrhagic CSF with increased red blood cells, iron, ferritin and protein. High field encephalic MR showed a hypointense image in T2 which surrounded the fissure of Sylvius, the brain stem, medulla oblongata, cerebellar hemispheres and sulci of the cerebellar vermis, suggestive of hemosiderosis, atrophy of the cerebellar vermis and slight cortical atrophy. Angioresonance of the intracranial vessels showed that there were no signs of aneurysms or vascular malformations. Transcranial Doppler studies were normal. CONCLUSIONS: Superficial siderosis of the CNS should be included in the differential diagnosis of the parethospastic and ataxic syndromes. The extensive study done ruled out any secondary cause such as chronic bleeding secondary to an arteriovenous malformation or bleeding aneurysm.
INTRODUCTION: Superficial siderosis of the central nervous system (CNS) is an uncommon neurological condition, characterized clinically by cerebellar ataxia, neurosensorial deafness, anosmia, myelopathy and cognitive deterioration. It is associated with the deposition of haemosiderin in the subpial layers of the brain, cerebellum and spinal cord, following chronic bleeding (often clinically silent) in the subarachnoid space. Histopathologically there is gliosis, neurone loss and demyelination of the CNS. CLINICAL CASE: We present the case of a 60 year old woman with a history of progressive worsening of a disorder of myelopathic type with signs of pyramidal liberation and sphincter incontinence associated with cerebellar ataxia, anosmia and bilateral hypoacusia. Initially she had unsteadiness, frequent falls and weakness of the legs. Her deafness was more obvious during the previous year. On lumbar puncture there was hemorrhagic CSF with increased red blood cells, iron, ferritin and protein. High field encephalic MR showed a hypointense image in T2 which surrounded the fissure of Sylvius, the brain stem, medulla oblongata, cerebellar hemispheres and sulci of the cerebellar vermis, suggestive of hemosiderosis, atrophy of the cerebellar vermis and slight cortical atrophy. Angioresonance of the intracranial vessels showed that there were no signs of aneurysms or vascular malformations. Transcranial Doppler studies were normal. CONCLUSIONS: Superficial siderosis of the CNS should be included in the differential diagnosis of the parethospastic and ataxic syndromes. The extensive study done ruled out any secondary cause such as chronic bleeding secondary to an arteriovenous malformation or bleeding aneurysm.