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Superficial siderosis of the central nervous system (SSCN) in a disease characterized by chronic deposition of hemosiderin in the leptomeninges, subpial tissue, spinal cord, and cranial nerves. Previously the diagnosis of SSCN could only be made at autopsy or during a neurosurgical procedure. Now, however, a diagnosis of SSCN can be made non-invasively by magnetic resonance imaging (MRI). We present the case of a 50-year-old male with SSCN accompanied by bilateral sensorineural hearing loss which gradually progressed to total deafness over a seven year period. This patient also had associated bilateral caloric weakness with episodes of severe recurrent headaches over the two preceding years. The deafness and gait disturbance, which were his chief complaints, were followed by other neurological manifestations including pyramidal tract signs, anosmia, and ageusia. High-field MRI on T-2 weighted images of the CNS showed diffuse marginal hypointensity of the cerebrum, brain stem, and cerebellum. Atrophy of the cerebellum and brain stem was also apparent. Low signal intensity along the proximal segment of the acoustic nerve and the facial nerve was noted from the cistern to the internal auditory canal. Neither bilateral transtympanic promontory nor round window electrical stimulation elicited any sound sensation. In this case it was thought that the acoustic nerve alone or both the acoustic nerve and the cochlea were affected by this disease. Consequently, cochlear implantation was not indicated. The source of bleeding into the subarachnoid space could not be detected despite thorough examination. This diagnosis will be made only by physicians who are aware of this rare entity and have knowledge of the characteristic clinical pathology.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of superficial siderosis of the central nervous system with total deafness]. 847 54

A 13-year-old boy with superficial siderosis of the central nervous system was reported. There were many members, including the proband, with sensory high tone hearing loss in his maternal family, but they did not have other neurological symptoms. Paroxysmal and pulsatile severe headache, and vomiting without aura appeared recurrently at the age of 8. His consciousness was alert and no other abnormal sign or symptom was seen during the attacks. The electroencephalogram and cranial computed tomogram revealed no abnormality. The T2 weighted magnetic resonance image of the cranium showed a superficial low intensity zone on the cerebellar vermis, frontal and parietal lobes of the cerebrum, and cervical and upper portion of the thoracic spinal cord at the age of 12, when he had severe headache and vomiting. Cerebrospinal fluid showed xanthochromia with mild elevation of the protein level during an attack, and a light bloody appearance during the asymptomatic state. The diagnosis of superficial siderosis of the central nervous system was made by these findings of magnetic resonance image and cerebrospinal fluid. The bleeding sources to the subarachnoid space could not be detected by cerebral angiography.
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PMID:[A case with superficial siderosis of the central nervous system accompanied by sensory high tone hearing loss and recurrent headache and vomiting]. 875 36

A 58-year-old woman, with recurrent headaches, exhibited cerebellar alaxic gait, anosmia, deafness and a pyramidal syndrome, with a progressive onset. In cerebrospinal fluid there was erythrocytes and siderophages. MRI on T2-weighted images revealed a marginal hypo-intensity, leading to the diagnostic of superficial siderosis of the central nervous system. None haemorragic lesion was found. The patient was given Trientine. Unfortunately she worsened on later examinations.
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PMID:[Case of superficial hemosiderosis of the central nervous system treated with trientine]. 977 49

A case of superficial siderosis that appeared in a case of suprasellar embryonal carcinoma is reported. A 24-year-old man presented polydipsia and vertigo. MRI revealed a suprasellar tumor. The tumor contained high intense spots on T1-weighted images, suggesting intratumoral hemorrhage. He underwent a surgery, which proved it as embryonal carcinoma pathologically. Cerebrospinal fluid was xanthochromic at surgery, suggesting terminal hemorrhage. After surgery, he received a total dose of 56 Gy of irradiation. Tumor decreased in size and symptoms improved. However, he presented occipital headache 7 months after surgery. MRI showed disseminated tumors in the subarachnoid spaces. He received irradiation for the whole spine and adjuvant chemotherapy. During treatment, MRI demonstrated low signals on the surface of the brain stem, suggesting the superficial siderosis. The lesions spread to the surface of the cerebellum and tentorium cerebelli. Superficial siderosis is characterized by the deposition of hemosiderin in the leptomeninges, cranial nerves and spinal cord. The etiology of the hemosiderin deposition is thought to be chronic or recurrent bleeding into subarachnoid space. Experimentally, similar lesions have been produced in the animals following intrathecal injection of blood or hemolysed red cells. In the literature, MRI demonstrated a rim of marked hypo-intensity on T2-weighted images, consistent with hemosiderin deposits, on the surface of cerebellum and brain stem. Gradient-echo sequences have been more sensitive than T2-weighted images of spin echo sequences. In the present case, the superficial siderosis seems to be due to chronic tumoral hemorrhage. This phenomenon could be related to chemotherapy using CDDP and etoposide.
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PMID:[Superficial siderosis appeared in a case of suprasellar embryonal carcinoma]. 984 53

Almost 95 cases of superficial siderosis of the central nervous system have been reported in the literature. These patients showed a clinical syndrome characterized by ataxia, deafness, pyramidal system involvement, and mental deterioration with xanthochromic cerebrospinal fluid and neuroradiological findings of hemosiderin deposits. About 30% of the patients had headache as an accompanying symptom. In the present case report, we describe a 33-year-old man with the typical clinical features of superficial siderosis, who complained, since aged 8, of a severe recurrent frontal headache often associated with loss of consciousness occurring after at least 2 hours of pain. The MRI and CSF findings were consistent with subarachnoid bleeding. In our patient, headache due to meningeal irritation by subarachnoid blood induced seizures as a probable reflex of extreme pain. Carbamazepine and nimodipine prophylaxis dramatically reduced the frequency of headaches and seizures.
Headache 1999 Oct
PMID:Central nervous system superficial siderosis, headache, and epilepsy. 1127 63

Superficial siderosis of the central nervous system is a rare, progressive, irreversible and debilitating neurological disease characterized by the deposition of haemosiderin in the leptomeninges and the subpial layers of the brain and spinal cord. The main clinical findings are progressive bilateral sensorineural hearing loss, cerebellar ataxia and pyramidal tract signs. The present report describes a 49-year-old woman who presented with intermittent headache of 5 years duration. The pain had become more severe in the previous 6 months. Neurological examination revealed nothing abnormal. Computed tomography showed a cystic mass with apparent internal haemorrhage in the right frontal lobe and T(2)-weighted magnetic resonance imaging showed material of low signal intensity coating the entire surface of the brain. The mass was completely excised via craniotomy. A histopathological study identified the mass as a papillary glioneuronal tumour. The patient recovered well and is still neurologically normal 1 year later. This is the first documented case of superficial siderosis caused by this type of tumour.
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PMID:Superficial siderosis due to papillary glioneuronal tumor. 1704 63

Superficial siderosis of the central nervous system is a very rare disease related to hemosiderin deposits in the brain, brainstem, cerebellum and spinal cord due to chronic subarachnoid hemorrhage. Chronic increased intracranial pressure develops in about one-third of affected cases. We report a patient with superficial siderosis and sudden intracranial pressure crisis. A 29-year-old man experienced a subacute episode of headache, tinnitus and blurred vision. Magnetic resonance imaging of the brain revealed hemosiderin deposits characteristic of superficial siderosis. Extensive diagnostic work-up excluded causative pathologies of bleeding. Lumbar puncture and continuous intra-ventricular cerebrospinal fluid (CSF) pressure monitoring revealed continuous CSF pressure increase. Implantation of a ventriculo-peritoneal shunt led to complete clinical recovery. Our case emphasizes that patients with superficial siderosis may present with sudden elevation of intracranial pressure due to chronic intracranial hypertension. In this situation permanent CSF drainage provides a useful therapeutic option.
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PMID:Secondary intracranial hypertension with acute intracranial pressure crisis in superficial siderosis. 1865 43

Superficial siderosis (SS) of the central nervous system is a rare condition caused by chronic subarachnoid hemorrhage. Clinical manifestations typically include sensorineural hearing loss and cerebellar ataxia. Recurrent episodic encephalopathy in the setting of SS has not been reported. We describe a unique case of SS in a 67-year-old man with an 8-year history of episodic encephalopathy associated with headache and vomiting. The patient also had a history of progressive dementia, ataxia, and myelopathy. A diagnosis of superficial siderosis was made after magnetic resonance gradient-echo images showed diffuse hemosiderin staining over the cerebellum and cerebral convexities. No intracerebral source of hemorrhage was identified. The patient therefore underwent gadolinium-enhanced spinal MRI which suggested a possible vascular malformation. A therapeutic laminectomy subsequently confirmed an arteriovenous fistula which was resected. In SS, there are often long delays between symptom onset and definitive diagnosis. Early identification is facilitated by magnetic resonance imaging with gradient-echo sequences. When no source of hemorrhage is identified intracranially, then total spinal cord imaging is indicated to assess for an occult source of hemorrhage as occurred in our case.
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PMID:Episodic encephalopathy due to an occult spinal vascular malformation complicated by superficial siderosis. 1985 21

A source of bleeding is often not evident during the evaluation of patients with superficial siderosis of the CNS despite extensive imaging. An intraspinal fluid-filled collection of variable dimensions is frequently observed on spine MR imaging in patients with idiopathic superficial siderosis. A similar finding has also been reported in patients with idiopathic intracranial hypotension. The authors report on a patient with superficial siderosis and a longitudinally extensive intraspinal fluid-filled collection secondary to a dural tear. The patient had a history of low-pressure headaches. His spine MR imaging and spine CT suggested the possibility of an underlying vascular malformation, but none was found on angiography. Repair of the dural tear resulted in resolution of the intraspinal fluid collection and CSF abnormalities. The significance of the association between superficial siderosis and idiopathic intracranial hypotension, and potential therapeutic and pathophysiological implications, are the subject of this report.
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PMID:A unifying hypothesis for a patient with superficial siderosis, low-pressure headache, intraspinal cyst, back pain, and prominent vascularity. 1989 95

Superficial siderosis is a rare condition caused by deposition of hemosiderin in the central nervous system. In recent years, it has been used to identify dural defects connecting the intrathecal space with the intraspinal fluid-filled collection seen in patients with superficial siderosis. However rare reports describe the association of low-lying cord in superficial siderosis patients. We present a patient with progressive cerebellar ataxia, sensorineural hearing loss, low-pressure headache, pyramidal signs and additional cervical and lumbar radiculopathy with neurogenic bladder which might be related to cervical disc herniation and tethered cord. CSF and Magnetic resonance imaging (MRI) studies confirmed the diagnosis of superficial siderosis. Dural defect is indirectly suspected by existence of fluid-filled collection ventral to the spinal cord and low-pressure headache in our patient. Underlying causes of low-pressure headache and possible dural defect should be searched for as an associated and possibly treatable condition in superficial siderosis and provide further insights into the mechanism of bleeding in our patient who have an intraspinal fluid-filled collection.
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PMID:A patient with superficial siderosis, intraspinal cyst, low-pressure headache and low-lying cord. 2236 82


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