Epilepsy caused by superficial hemosiderosis of the central nervous system

Autor: Zeljka Petelin Gadze, Davorka Milat, Ervina Bilić, Filip Derke, Marija Bošnjak Pašić
Rok vydání: 2017
Předmět:
Zdroj: Neurological Sciences. 39:781-783
ISSN: 1590-3478
1590-1874
DOI: 10.1007/s10072-017-3169-1
Popis: Superficial hemosiderosis of the central nervous system (CNS) is an uncommon and often unrecognized disorder caused by small repeated hemorrhages into the subarachnoid space, resulting in CNS hemosiderin deposition in the subpial layers. The etiology is reported to be idiopathic in 35% of cases, CNS tumor in 15%, trauma in 13% and arteriovenous malformation in 9% of cases. It is rarely caused by intradural neurosurgical operations, nerve root avulsion, or other causes of subarachnoid hemorrhage. Xanthochromia is a common finding. Also, magnetic resonance imaging (MRI) of the CNS reveals a rim of hypointensity on T2-weighted images involving the surface of the brainstem, cerebellum, and cortical fissures. The classical symptoms include sensorineural hearing impairment, visual impairment, slowly progressing cerebellar ataxia and pyramidal signs, but this syndrome rarely clinically presents with seizures. To date, there are only few reported cases of seizures caused by superficial siderosis. A 70-year old retired senior medical nurse has presented with a sensorineural hearing loss in the last 10 years, slowly progressing gait ataxia, hyposmia, spastic paraparesis, urinary incontinence and epilepsy - occipital lobe seizures with visual hallucinations, forced blinking, and contralateral eye deviation, followed by impared awareness, which pointed to the propagation of epileptogenesis into the temporal lobe, and with secondary generalization (confirmed with video-EEG monitoring). Seizures started at the age of 69. In her history she had hypertension, hyperlipidemia and cerebellar stroke at the age of 45, as well as head contusion and brain concussion in a traffic accident at the age of 46. Gait ataxia was initially connected to cerebellar stroke, but due to progressive course we have searched for other causes. MRI provided the etiologic diagnosis based on typical low-density signals from the brain, brainstem, cerebellar and spinal cord surfaces on T2 and gradient-echo weighted sequences (T2*) – hemosiderosis as a consequence of a previous subarachnoid hemorrhage. Cerebrospinal fluid (CSF) analysis revealed xanthocromia. Multislice CT angiography of the head and neck blood vessels and well as spinal cord digital subtraction angiography did not reveal signs of aneurysms of arteriovenous malformation, what is a diagnostic challenge for clinicians. Due to idiopathic etiology of hemosiderosis, surgical treatment was not possible, and clinicians focused on alleviating symptoms of main disorder and other chronic diseases. Symptomatology of symptomatic seizures, which in our opinion were caused by hemosiderosis, has been managed well with oxcarbazepine (600 mg/day, given in a twice-a- day regimen) in the follow-up period of five years, but the neurological deficit persists. By observing clinical and neuroradiological changes in this patient, the diagnosis of idiopathic superficial hemosiderosis of the central nervous system has been performed. According to our knowledge, it is the first reported patient with clinical presentation of occipital lobe seizures possible caused by hemosiderin deposition. We also want to point out that hemosiderin deposition for an extended period should be considered to contribute to the pathogenesis of epilepsy.
Databáze: OpenAIRE
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