Superficial siderosis of the CNS: report of three cases and review of the literature

Superficial siderosis (SS) is a rare entity. A chronic subarachnoid hemorrhage (SAH) leads to the deposition of ferric pigments and ions in the surface of the central nervous system (CNS). The cause of this hemorrhage is identified in half of the cases. The disease progresses slowly and the damage is often irreversible by the time diagnosis is established. The management focuses on the identification and ablation of the bleeding source. Knowledge about this entity is based on sporadic reports. We describe three cases of superficial siderosis and briefly review the literature.     

Superficial siderosis of the central nervous system

Summary The clinical and necropsy findings are described in nine cases of superficial siderosis of the central nervous system. Four of these had undergone cerebral hemispherectomy in childhood. All four showed multiple haemorrhagic spots in the cavity membrane and the walls of the remaining lateral ventricle. Of the other cases, to had repeated ruptures of cerebral aneurysms; one showed a destructive lesion of the cerebellar vermis; one, a hydrocephalic child, showed evidence of repeated haemorrhage in the area of a malformed aqueduct; and the last, a baby, had bilateral subdural haematomata.From the histological similarities of these cases and previously reported ones, and from the reported findings in experimental animals, it is argued that the common cause of superficial siderosis is recurrent intraventricular or subarachnoid haemorrhage.

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Zusammenfassung Klinischer Verlauf und Autopsiebefunde von 9 Fällen mit Randzonensiderose des ZNS werden beschrieben. Bei 4 Fällen war eine Hemisphärektomie in der Kindheit erfolgt. Sie boten multiple Blutungsreste in der Membran der Resthöhle sowie in den Wänden der verbliebenen Seitenventrikel. Von den übrigen Beobachtungen boten 2 wiederholte Aneurysmarupturen von Hirngefäßen; 1mal bestand eine destruktive Läsion im Kleinhirnwurm. 1 hydrocephales Kind bot Hinweise auf wiederholte Blutungen im Bereich einer Fehlbildung des Aquädukts. Der letzte Fall, 1 Kleinkind, bot beidseitiges subdurales Hämatom.Auf Grund der histologischen Ähnlichkeiten dieser Fälle und früherer Beobachtungen sowie tierexperimenteller Befunde werden als gemeinsame Ursachen der Randzonensiderose wiederholte Blutungen in den inneren oder äußeren Hohlräume des ZNS angenommen.

     

Superficial siderosis of the central nervous system

The course of a patient suffering from superficial siderosis of the central nervous system for 37 years is presented and diagnostic and therapeutic approaches are evaluated. The syndrome is clinically defined by slowly progressing deafness, cerebellar ataxia, myelopathy and neuropsychological deficits in combination with recurrent xanthochromia of the cerebrospinal fluid with siderophages. The diagnosis may be confirmed by computed tomography, which shows degeneration of the cerebellar vermis, and by magnetic resonance imaging, demonstrating iron deposits on the surface of brain, brain stem and spinal cord. Therapy should seek to identify and remove the source of bleeding, since pharmacotherapy with iron-depleting drugs is of limited effectiveness.     

Superficial siderosis of the central nervous system: neuroradiological evaluation of two cases.

We present 2 cases of superficial siderosis of the central nervous system secondary to chronic subarachnoid bleeding. The diagnosis was made with MR and cerebrospinal fluid (CSF) examination. MR showed, in T2-weighted images, superficial hypointensity of the cervical cord, brainstem, cerebellum, and basal cisterns due to hemosiderin deposits. CSF was xanthochromic, with increased protein content. In 1 of the 2 cases also the CT examination was positive, showing a hyperdense rim around the brainstem. A complete neuroradiological evaluation (MR, CT, angiography and myelography) did not permit to detect the source of the bleeding.     

Superficial siderosis of the central nervous system]

We report five cases of superficial siderosis of the central nervous system. All patients developed progressive deafness and cerebellar ataxia associated with pyramidal tract signs or mental deterioration. The cerebrospinal fluid examinations usually revealed an elevated protein level, without other abnormalities. Magnetic resonance imaging typically showed a hypointense rim around the cerebral and cerebellar hemispheres, the brainstem and the spinal cord on T2-weighted images. A definite source of bleeding was only found in two patients. The literature on superficial siderosis is reviewed. The etiologies and the pathogenesis are discussed.     

Superficial siderosis of the central nervous system and anticoagulant therapy: a case report

Superficial siderosis of the central nervous system is a rare condition characterized by deposition of haemosiderin in the leptomeninges and in the subpial layers of the brain and spinal cord. With the widespread use of magnetic resonance imaging, an increasing number of cases of superficial siderosis are being discovered, secondary forms being more frequent than idiopathic ones. We report a 78-year-old man in oral anticoagulant therapy, who presented neurosensory hearing loss, gait ataxia and spastic paraparesis. Magnetic resonance imaging suggested the diagnosis of superficial siderosis of the central nervous system, without an evident bleeding source.     

Idiopathic infratentorial superficial siderosis of the central nervous system: case report and review of literature

The superficial siderosis (SS) of the central nervous system (CNS) is a rare condition characterized by a wide range of neurological manifestations directly linked to an acquired iron-mediated neurodegeneration. First described more than 100 years ago, only recently SS has been divided into two distinct entities, according to the distribution of iron deposition in the CNS: cortical superficial siderosis (cSS) and infratentorial superficial siderosis (iSS). Here we describe an adult case of iSS, with detailed clinical and radiological features. Moreover, we extensively review the literature of SS, particularly focusing on the pathogenesis, clinical-radiological classification, diagnostic algorithm and treatment options of this rare condition.     

中枢神经系统表面铁沉积症一例

目的 讨论中枢神经系统表面铁沉积症的病例特点及诊断.方法 对1例患者进行临床相关资料分析.结果 中枢神经系统表面铁沉积症是由于铁沉积在中枢神经系统表面而产生.临床典型特征为:进行性感音性听力下降,小脑性共济失调和锥体束征,磁共振的梯度回波序列T2*加权像上可见颅脑及脊髓表面显著低信号带,脑脊液中铁蛋白含量增高.结论 中枢神经系统表面铁沉积症容易误诊,通过病史及体格检查并结合相关检查可以在疾病的早期确诊.磁共振的梯度回波序列T2*加权像及脑脊液的相关检查有助于诊断.     

Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches

OBJECTIVE: Superficial siderosis of the central nervous system (CNS) is a rare chronic progressive disorder caused by chronic subarachnoid hemorrhage. We present four patients with superficial siderosis of the CNS to describe the characteristic symptoms, and to discuss the pathogenetic heterogeneity and possible new therapeutic approaches. RESULTS: The causes of chronic subarachnoid bleeding in superficial siderosis were different. In two patients surgical treatment of ependymoma or cerebral cavernomas were the underlying diseases. No cause was detected in one patient. For the first time, we present one patient with vasculitis of the central nervous system associated with systemic hemochromatosis in superficial siderosis. Therapeutic approaches included exstirpation of cavernomas as the source of chronic bleeding in one patient, immunosuppressive therapy and venupunctures in the patient with vasculitis and hemochromatosis, and symptomatic treatment with chelating agents and antioxidants. The patients remained clinically stable for the follow-up period of up to 2 years. CONCLUSIONS: Our cases underline the pathogenetic heterogeneity of superficial siderosis and favor the early diagnosis for prompt initiation of therapy. Besides treatment of the underlying condition, antioxidants and radical scavengers may be effective in halting the progression of the disease.     

类似僵人综合征的中枢神经系统表面含铁血黄素沉积症

该文报道一例临床表现类似僵人综合征的中枢神经系统表面含铁血黄素沉积症.患者为男性,27岁,因"双耳听力下降伴行走困难4年"入院.患者临床表现为进行性感应神经性耳聋、小脑性共济失调及脊髓病变,肌张力明显升高,导致脊柱前突,类似僵人综合征.头颅及脊髓MRI显示中脑、桥脑、小脑蚓部及脑沟内,双侧岛叶、颞叶以及脊髓表面均可见条形短T2信号环绕.该例患者脊髓的损害除了累及脊髓的皮质脊髓束、脊髓丘脑束、脊髓侧角外,还主要累及到脊髓的中间神经元,从而出现僵人综合征样表现.     

Superficial siderosis of the central nervous system secondary to spinal ependymoma

Superficial siderosis of the central nervous system is a syndrome caused by deposition of hemosiderin in the subpial layers of the central nervous system, occurring as a result of recurrent asymptomatic or symptomatic bleeding into the subarachnoid space. We report a rare case of superficial siderosis in a 33-year-old man who presented with sensorineural hearing loss. The diagnosis of superficial siderosis on MRI brain studies led to further investigations with detection of a spinal ependymoma at L1–L2, compressing the cauda equina. Gross total resection of the tumor arrested the progression of the neurological deterioration. Our report underlies the importance of early diagnosis and surgical management, with imaging examination of the full neuroaxis to identify the source of bleeding, to halt disease progression and improve prognosis.     

Superficial siderosis of the central nervous system many years after neurosurgical procedures

Recurrent haemorrhage into the subarachnoid space causes superficial siderosis, which clinically manifests as cerebellar ataxia, sensorineural hearing loss, and myelopathy. Two patients developed clinical, radiological, and biochemical evidence of superficial siderosis many years after surgery. One had two posterior fossa operations, a left temporal craniectomy, and radiotherapy for a presumed brain tumour before developing clinical evidence of superficial sidersosis 37 years later. The other had small bilateral subdural collections from recurrent shunt revisions following posterior fossa surgery for a Chiari malformation, and then developed deafness and ataxia. The first patient currently has the longest recorded delay between presumed subarachnoid bleeding and clinical manifestations of superficial siderosis. Both patients provide further evidence that superficial siderosis of the central nervous system, a progressive neurodegenerative vascular condition, may be a delayed complication of neurosurgical procedures.     

Superficial siderosis of the central nervous system secondary to a thalamic hamartoma

A patient with radiologically evident superficial siderosis of the central nervous system secondary to a thalamic hamartoma is reported. Cerebral hamartomas are rare, benign congenital lesions which when vascular can cause repeated subarachnoid haemorrhages, which results in deposition of iron (haemosiderin) in the leptomeninges and in the subpial layers of the brain and spinal cord. Progressive sensorineural deafness and cerebellar ataxia, which are typical clinical manifestations of superficial siderosis, may then appear. The importance of prompt diagnosis and treatment is highlighted.     

Primary central nervous system lymphoma report of 32 cases and review of the literature

We retrospectively analyzed 32 cases of primary central nervous system lymphoma (PCNSL). Five cases were diagnosed in the period 1987-1994, for 27 cases in the period 1995-2002. There were 17 men and 15 women whose median age was 69 years. Three patients were immunodeficient. The commonest symptoms were focal deficit (16 patients) and cognitive/behaviour disturbances (14 patients). Radiologically, a total of 47 contrast-enhancing lesions were observed in 32 patients; 18 patients had deep-seated lesions. All but two patients underwent histological diagnosis following craniotomy (11 patients) and/or stereotaxic biopsy (22 patients); diagnosis was obtained on CSF cytology in one patient with a third ventricle tumour. In the last patient, the diagnosis was based on the finding of marked tumour shrinkage under corticotherapy, despite two negative histological examinations. Treatment included surgical resection (10 patients), chemotherapy (25 patients) and/or radiotherapy (12 patients). According to the therapeutic recommendations of the GELA (Groupe d'Etude des Lymphomes de l'Adulte), 19 patients received at least two courses of high-dose methotrexate; intrathecal chemotherapy was used in 20 patients with methotrexate and/or cytosine arabinoside. Radiation therapy consisted of whole brain irradiation followed by a boost on tumour site. Nine patients received a combined treatment of chemotherapy and radiotherapy. Twelve patients showed rapid progression to death. At the time of last contact, 28/32 patients (88%) had died, all from PCNSL disease or from complications due to its treatment. The median survival time was 13.9 months. We conclude that PCNSL is an increasingly frequent tumour. The diagnosis is obtained by stereotactic biopsy in the majority of cases. The prognosis appears dismal despite an intensive multidisciplinary therapeutic approach.