中枢神经系统表面铁沉积症合并腰椎管内病变1例报道

中枢神经系统表面铁沉积症(superficial siderosis of the central nervous system,SSCNS)是一组由慢性蛛网膜下腔出血导致含铁血黄素沉积于中枢神经系统表面引起的罕见疾病,以神经感觉性耳聋、小脑共济失调、锥体束征为典型临床表现.本文报道了1例手术治疗SSCNS合并椎管内病...     

Superficial siderosis of the central nervous system: a neglected cause of sensorineural hearing loss.

Superficial siderosis of the central nervous system is a rare, often unrecognized condition in which there is a deposition of intracellular and extracellular hemosiderin in the leptomeninges, subpial tissue, spinal cord, and cranial nerves as a result of chronic bleeding in the subarachnoid space. On the basis of our cases and a review of the literature, the majority of patients manifest sensorineural hearing loss in addition to cerebellar dysfunction, pyramidal tract signs, and progressive mental deterioration. Recent imaging techniques--particularly CT and MRI--can define the presence of iron deposition, confirming the diagnosis ante mortem. From post mortem specimens, histologic sections demonstrate severe damage to the eighth nerve, with microglial metabolism of iron. Cases will be presented to illustrate these points.     

马尾部粘液乳头状室管膜瘤继发中枢神经系统表面铁沉积症1例报道

中枢神经系统表面铁沉积症（superficial siderosis of the central nervous system,SSCNS）是一种因含铁血黄素沉积于软脑膜下、脑组织、脑神经和脊髓表面所导致的罕见神经系统变性综合征,1908年由Hamill首次报道。其特征性临床表现为：感音神经性耳聋、小脑共济失调、锥体束征阳性。特征性的影像学表现为T2WI MRI上显示脑、脊髓表面线样低信号影。病理结果可见脑干、小脑及脊髓表现含铁血黄素、铁及铁蛋白沉积。其病因尚未明确,目前普遍认为慢性、隐匿的微量红细胞反复或持续漏入蛛网膜下腔是其主要病因。导致蛛网膜下腔出血的病因很多,中枢神经系统肿瘤是SSCNS的病因之一,但由马尾部粘液乳头状室管膜瘤引起SSCNS的病例报道很少。由于病例少见,对该病认识较晚,并且长期随访资料缺乏,病因诊断不够重视,导致漏诊、误诊率较高。我们收治1例马尾部粘液乳头状室管膜瘤导致SSCNS的病例,结合文献,探讨其可能的病因、症状特点、诊治要点及预后,以加深临床医生对此类疾病的认识。     

Bulbar amyotrophic lateral sclerosis: patterns of progression and clinical management

Patients with bulbar amyotrophic lateral sclerosis (ALS) are often referred to the otolaryngologist/head and neck surgeon and speech pathologist for evaluation and management of dysphagia and dysarthria. These patients comprise an unusual group because of the progressive and multi-system nature of their illness. The neuromuscular disabilities associated with bulbar ALS cause a myriad of related symptoms associated with swallowing, speech, and respiration. Although the rate of progression cannot be predicted, a general pattern of progression is noted. Bulbar disease accounts for the majority of the worst symptoms of ALS. The loss of the ability to swallow changes eating from a pleasurable task to a burden of survival. Loss of communication effectively imprisons the patient in a state of isolation. The progressive weakness of respiration, predominantly a spinal rather than bulbar manifestation, is the cause of death for nearly all ALS patients and is also discussed. The general patterns of progression of bulbar ALS are outlined in this paper. The development of symptoms are correlated with specific treatment recommendations to aid the clinician in devising an orderly plan of management for this progressive disease.     

Metastases of central nervous system neoplasms. Case report

Tumor-to-tumor metastasis is a rare occurrence. Fewer than 100 cases have been reported, many being metastases from carcinomas to benign intracranial neoplasms, most often meningiomas. A case is presented of carcinoma metastatic to a glioma. The patient, who presented for evaluation of bifrontal headache, was found on computerized tomography to have a partially calcified right frontal mass. Craniotomy revealed an oligodendroglioma containing foci of adenocarcinoma. Further work-up disclosed an infiltrative ductal adenocarcinoma of the breast. It has been suggested that tumors of the central nervous system may provide a fertile substrate or an immunological "haven" for metastases.     

Superficial siderosis of the central nervous system following cervical nerve root avulsion: the importance of early diagnosis and surgery

Summary Superficial siderosis (SS) of the central nervous system is an insidious, progressive, irreversible and debilitating neurological disorder caused by recurrent haemorrhage within the subarachnoid space. The subsequent deposition of haemorrhagic breakdown products in the spinal cord and nervous tissues leads to the loss of neurones and myelin, and to the development of a neurological deficit. In a small number of patients, the source of haemorrhage is related to traumatic cervical nerve root avulsion occurring several years prior to the onset of symptoms. Surgical ablation of the source has been shown to halt the progression of the disease, at least in the short term. We review the literature on SS secondary to cervical nerve root avulsion and report a further case in which surgical management was successful in halting disease progression. We emphasize that early detection and recognition of the initial non-progressive symptoms related to this poorly known disease, coupled with timely surgical management, minimizes the degree of neurological disability.     

Three cases of superficial siderosis of the central nervous system and review of the literature

Superficial siderosis of the central nervous system results from chronic or intermittent hemorrhage into the subarachnoid space that causes hemosiderin deposition in subpial layers of the brain and the spinal cord leading to neuronal damage. Patients present with progressive and debilitating symptoms that typically include adult-onset slowly progressive cerebellar gait ataxia and sensorineural hearing impairment. Regardless of extensive investigations, the origin of the hemorrhage is often not clear. Because of the good availability of magnetic resonance imaging, asymptomatic cases of superficial siderosis of the central nervous system are increasingly discovered. SS cases are increasingly reported in the literature. We present three new cases. The etiology, pathogenesis, clinical features, and treatment options of SS are reviewed.     

Primary angiitis of the central nervous system mimicking tumor-like lesion--case report

A 60-year-old man presented with a rare case of primary angiitis of the central nervous system mimicking a tumor-like lesion and manifesting as slight disorientation, left hemiparesis, and motor aphasia. Computed tomography showed multiple low density lesions in the left frontal lobe, brain stem, and right parietal lobe. Magnetic resonance images revealed a slightly enhanced mass lesion in the right parietal lobe with surrounding brain edema. Serum, cerebrospinal fluid, and other image examinations did not show any abnormal findings, so surgical removal of the right parietal mass was performed. Histological examination revealed that the mass consisted of hemorrhagic infarction without cellular atypia. Proliferations of endothelial cells in small and medium arteries, and infiltration of macrophages in the perivascular space were detected in the infarction tissues. The histological diagnosis was primary angiitis of the central nervous system.     

Signet-ring cell lymphoma of the central nervous system. Case report

Signet-ring cell lymphoma is a rare tumor described in the lymph nodes, skin, tonsils, thyroid, salivary gland, and ocular orbit. This is the first case report of a B-cell signet-ring cell lymphoma in the central nervous system with Dutcher and Russell bodies, signet-ring nuclei, intracisternal crystalline inclusions, and immunoglobulin M positivity. The patient is doing well 2 years after surgery and postoperative radiation therapy. No definitive prognostic characteristics have been elucidated. This disease entity can only be suspected on histological grounds and confirmed by immunocytochemical and ultrastructural studies.     

Phenylpropanolamine: an over-the-counter drug causing central nervous system vasculitis and intracerebral hemorrhage. Case report and review

Phenylpropanolamine (PPA) is the major ingredient in more than 70 over-the-counter preparations including diet pills, nasal decongestants, and the legal "look-alike" stimulants. Structurally and functionally similar to amphetamine and ephedrine, PPA has recently been associated with several neurological manifestations including psychosis, stroke, severe headache, seizures, and intracerebral hematoma. We report a case of intracerebral hematoma and subarachnoid hemorrhage in a young woman with angiographic and biopsy-proven vasculitis of the central nervous system (CNS) induced by PPA in her diet pills. From review of the literature, we distinguish drug-induced vasculitis as a separate entity from primary CNS vasculitis, both clinically and pathologically. This report should alert physicians, in general, to this potentially fatal side effect of PPA, a commonly used over-the-counter drug. Also, neurosurgeons in particular should consider the possibility of drug-induced vasculitis when faced with cases of intracerebral or subarachnoid hemorrhage without apparent cause.     

Malignant lymphoma of the central nervous system presenting with central neurogenic hyperventilation. Case report.

The case is described of a 72-year-old woman who presented with a progressive right hemiparesis and central neurogenic hyperventilation. Pathological and radiological studies revealed diffuse infiltration of a malignant lymphoma into the entire central nervous system and the upper spinal cord. The authors review 12 cases of tumor-induced central neurogenic hyperventilation and discuss the pathophysiology of this condition.     

Actinomycosis of the central nervous system: surgical treatment of three cases

Three cases of actinomycotic brain infection are described, 2 of which manifested as cerebral abscess, the third as epidural empyema. Complete resolution of the infection was always achieved by means of surgical treatment and prolonged antibiotic therapy. The cases reported emphasize the importance of a combined approach in the treatment of this unusual cause of brain infection.     

Lumbar vertebral hemangioma mimicking lateral spinal canal stenosis: Case report and review of literature

Abstract

Context

Hemangiomas are the commonest benign tumors of the spine. Most occur in the thoracolumbar spine and the majority are asymptomatic. Rarely, hemangiomas cause symptoms through epidural expansion of the involved vertebra, resulting in spinal canal stenosis, spontaneous epidural hemorrhage, and pathological burst fracture.

Findings

We report a rare case of a 73-year-old woman, who had been treated for two months for degenerative neurogenic claudication. On admission, magnetic resonance imaging and computed tomographic scans revealed a hemangioma of the third lumbar vertebra protruding to the epidural space producing lateral spinal stenosis and ipsilateral nerve root compression. The patient underwent successful right hemilaminectomy for decompression of the nerve root, balloon kyphoplasty with poly-methyl methacrylate (PMMA) and pedicle screw segmental stabilization. Postoperative course was uneventful.

Conclusion

In the elderly, this rare presentation of spinal stenosis due to hemangiomas may be encountered. Decompression and vertebral augmentation by means balloon kyphoplasty with PMMA plus segmental pedicle screw fixation is recommended.     

Lumbar vertebral hemangioma mimicking lateral spinal canal stenosis: Case report and review of literature

Context

Hemangiomas are the commonest benign tumors of the spine. Most occur in the thoracolumbar spine and the majority are asymptomatic. Rarely, hemangiomas cause symptoms through epidural expansion of the involved vertebra, resulting in spinal canal stenosis, spontaneous epidural hemorrhage, and pathological burst fracture.

Findings

We report a rare case of a 73-year-old woman, who had been treated for two months for degenerative neurogenic claudication. On admission, magnetic resonance imaging and computed tomographic scans revealed a hemangioma of the third lumbar vertebra protruding to the epidural space producing lateral spinal stenosis and ipsilateral nerve root compression. The patient underwent successful right hemilaminectomy for decompression of the nerve root, balloon kyphoplasty with poly-methyl methacrylate (PMMA) and pedicle screw segmental stabilization. Postoperative course was uneventful.

Conclusion

In the elderly, this rare presentation of spinal stenosis due to hemangiomas may be encountered. Decompression and vertebral augmentation by means balloon kyphoplasty with PMMA plus segmental pedicle screw fixation is recommended.     

An atypical spinal meningioma with CSF metastasis: fatal progression despite aggressive treatment. Case report

The authors report the case of a 23-year-old man who presented with a C1-3 spinal mass. Following intraspinal decompression the tumor was histologically classified as an atypical meningioma (World Health Organization grade II). Two further surgical interventions resulted in almost total removal of the meningioma. In addition, radiotherapy was performed. During the 1.5-year follow-up period the diagnostic examinations identified a local tumor recurrence, an intraspinal C-6 metastasis, and a segmental instability with anterior C2-3 slippage and C3-4 kyphosis. The tumor was resected and occipitocervical stabilization was performed. Histological examination showed no change in malignancy. Despite additional hydroxyurea-based chemotherapy, the patient presented 4 months later with a hemiparesis and a massive recurrence of the tumor mass involving the posterior fossa and the upper thoracic spine. Because there were no further therapeutical options, the patient died. The authors discuss more aggressive therapeutic options in addition to surgery in patients with metastatic atypical meningiomas. The results in the reported case indicate that meningiomas associated with cerebrospinal fluid metastasis may represent a higher grade of malignancy.     

Fibro-osseous lesions of the central nervous system: report of four cases and literature review.

Fibro-osseous lesions, also reported as calcifying pseudoneoplasms of the neural axis, are uncommon lesions of the CNS. We report four additional cases: two extraaxial and two intraaxial, in patients ages 33, 47, 49, and 59 years at presentation. Fibro-osseous lesions involving the CNS demonstrate variable proportions of fibrous stroma, bone, palisading spindle to epithelioid to multinucleated cells in association with a highly distinctive, perhaps pathognomonic, chondromyxoid-like matrix often distributed in a nodular pattern. This histopathologically distinctive lesion can be seen in many regions of the neuraxis, often with a dural association, and most commonly along the vertebral column. It appears to be a slow-growing lesion and, with wide excision, the prognosis is excellent. The etiology remains unclear, but the preponderance of data favors a reactive rather than neoplastic process. If this putative pseudotumor is not recognized histopathologically, a neoplastic or infectious differential might result in inappropriate investigations and potentially harmful therapies.     

Primary angiitis of the central nervous system mimicking dissemination from brainstem neoplasm: a case report

BACKGROUND: Diagnosis of PACNS is very difficult because of the variety of clinical manifestation, especially neurologic and MRI findings. We report the case of a 68-year-old woman with PACNS. CASE DESCRIPTION: Her clinical symptoms mimicked malignant brainstem neoplasm disseminated to third and fourth ventricles and cervical spinal cord. Brain biopsy could not be correctly diagnostic. In spite of a trial of high-dose steroids, repeated MRI showed an increasing number of lesions. Two weeks after surgery, she died of respiratory dysfunction. Postmortem examination suggested PACNS, after consideration of histories, laboratory and radiologic findings, and the lack of systemic disease. CONCLUSION: Although brain biopsy represents the gold standard measure, combination with several other examinations might be necessary to arrive at an early and definitive diagnosis. PACNS should be considered the differential diagnosis of intracranial tumors.     

Intraparenchymal schwannomas of the central nervous system: An additional case report and review

Intraparenchymal location of schwannomas in the central nervous system (CNS) is rare. Occasional cases involving the cerebrum, cerebellum, brain stem, and spinal cord have been reported. We report here an additional case of thoracic intramedullary schwannoma in a 42 year old woman. The literature concerning intraparenchymal schwannomas in the CNS is reviewed and discussed.