1/20. Superficial siderosis of the CNS associated with multiple cavernous malformations.Superficial siderosis of the CNS due to chronic, recurrent subarachnoid hemorrhage is an uncommon and potentially debilitating disorder. The classic clinical manifestation is progressive bilateral sensorineural hearing loss (SNHL), although ataxia and pyramidal signs also are observed frequently. Cavernous malformations rarely present with subarachnoid hemorrhage. We describe an unusual case of a young patient who presented with progressive, bilateral SNHL who was found to have superficial CNS siderosis associated with multiple cavernous malformations.- - - - - - - - - - ranking = 1keywords = siderosis (Clic here for more details about this article) |
2/20. Superficial siderosis of the central nervous system. A case report on examination by ECoG and DPOAE.This is a case of superficial siderosis of the central nervous system (SSCN). The diagnosis of SSCN was based on the result of T2-weighted magnetic resonance imaging and on suggestive clinical manifestations. The pure-tone audiogram showed bilateral progressive sensorineural hearing loss with a poor speech discrimination score and Jerger type IV. The remarkable elevation of the detective threshold of cochlear microphonics on electrocochleography was found and distortion product otoacoustic emission (DPOAE) showed no response: These electrophysiologic examinations, including electrocochleography and DPOAE, revealed that the progressive sensorineural hearing loss in this case was caused by both retrocochlear and cochlear damages.- - - - - - - - - - ranking = 0.83333333333333keywords = siderosis (Clic here for more details about this article) |
3/20. hydroxychloroquine ototoxicity in a child with idiopathic pulmonary haemosiderosis.hydroxychloroquine, a quinoline compound, rarely causes ototoxicity. According to the few reports in existence, hydroxychloroquine-induced ototoxicity occurred following prolonged therapy in adult patients with rheumatoid arthritis and lupus erythematosus. We report a case of unilateral sensorineural hearing loss in a 7-year-old girl with idiopathic pulmonary haemosiderosis, after 2 years of hydroxychloroquine treatment. Sensorineural hearing loss has previously been reported with hydroxychloroquine treatment, but this is the first report in a child and associated to idiopathic pulmonary haemosiderosis and has the characteristic of being unilateral.- - - - - - - - - - ranking = 1keywords = siderosis (Clic here for more details about this article) |
4/20. Superficial siderosis of the meninges and its otolaryngologic connection: a series of five patients.OBJECTIVE: To study the otolaryngologic disorders in patients with superficial siderosis of the meninges. BACKGROUND: Superficial siderosis of the central nervous system is a rare disorder characterized by progressive bilateral hearing loss and ataxia caused by recurrent bleeding into the subarachnoid space. The cerebellum, eighth cranial nerve, and olfactory nerve are particularly susceptible to the deposition of hemosiderin, which is responsible for the symptoms. The diagnosis is confirmed by magnetic resonance imaging. methods: The clinical notes of five patients with superficial siderosis of the meninges were reviewed with the intent of reporting the otolaryngologic symptoms and signs, the clinical investigations, and treatments. RESULTS: Four of the five patients had sensorineural deafness, two had smell disturbances, and three had gait abnormalities. magnetic resonance imaging was the most important investigation used to identify the condition. CONCLUSIONS: Superficial siderosis of the meninges is an important differential diagnosis in patients with progressive sensorineural deafness.- - - - - - - - - - ranking = 1.3333333333333keywords = siderosis (Clic here for more details about this article) |
5/20. Longitudinal analysis of hearing loss in a case of hemosiderosis of the central nervous system.OBJECTIVE: To describe cochleovestibular aspects of superficial hemosiderosis of the central nervous system. BACKGROUND: Superficial hemosiderosis of the central nervous system is a rare disease in which cochleovestibular impairment, cerebellar ataxia, and myelopathy are the most frequent signs. Chronic recurrent subarachnoidal hemorrhage with bleeding into the cerebrospinal fluid is the cause of deposition of hemosiderin in leptomeningeal and subpial tissue, cranial nerves, and spinal cord. Removing the cause of bleeding can prevent irreversible damage to these structures. Because this is the only effective treatment, an early diagnosis is crucial. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENT: A 72-year-old woman with superficial hemosiderosis of the central nervous system that developed when she was age 39. methods: Neurologic and imaging diagnostic examinations and longitudinal evaluation of cochleovestibular features were performed. neurosurgery was not performed. RESULTS: Progressive bilateral sensorineural hearing loss and severe vestibular hyporeflexia developed within 15 years, which can be attributed to lesions in the cochleovestibular system. Additional pathology of the central nervous system developed later. CONCLUSION: The patient demonstrated cochlear and vestibular findings that are typical of this pathologic condition. It is the first documented case with extensive serial audiometry used to precisely outline the degree of hearing deterioration during the course of the disease.- - - - - - - - - - ranking = 1.1666666666667keywords = siderosis (Clic here for more details about this article) |
6/20. The importance of suspecting superficial siderosis of the central nervous system in clinical practice.Once the central nervous system surface is greatly encrusted with haemosiderin, even removing the source of bleeding will have little effect on the progression of clinical deterioration. Superficial siderosis of the central nervous system is rare and insidious, but magnetic resonance imaging has turned a previously late, mainly autoptical diagnosis into an easy, specific, in vivo, and possibly early one. Avoiding long diagnostic delay will be very important in those cases susceptible of causal treatment.- - - - - - - - - - ranking = 0.83333333333333keywords = siderosis (Clic here for more details about this article) |
7/20. hearing loss and vertigo in superficial siderosis of the central nervous system.PURPOSE: Otoneurologic findings in patients with superficial siderosis (SS) of the central nervous system are described. A 20-year-old man with acute vertigo, dizziness, with a history of head trauma in childhood; an 87-year-old woman with severe disequilibrium appearing after peridural anesthesia; and a 55-year-old woman with recurrent episodes of positional vertigo and progressive ataxia, suffering from a lumbar ependymoma are described; all patients complained of progressive bilateral hearing loss and tinnitus.methods and results Otoneurologic examination showed bilateral sensorineural hearing loss, disturbed ocular pursuit and optokinetic nystagmus, incomplete visual suppression of the vestibulo-ocular reflex during rotatory pendular testing, right hyporeflexia, and bilateral caloric areflexia. Cerebral magnetic resonance imaging (MRI) revealed rims of hypointensity surrounding the brainstem and linear hypointensities following the surface of the cerebellar folia. CONCLUSIONS: The etiology stems from subarachnoid hemorrhage, but the source of bleeding may remain obscure. Bilateral hearing loss is described in 95% and disequilibrium in 90% because of peripheral vestibular deficit and cerebellar ataxia. In patients with progressive bilateral cochleo-vestibular deficit of unknown etiology, MRI is the examination of choice to confirm SS.- - - - - - - - - - ranking = 0.83333333333333keywords = siderosis (Clic here for more details about this article) |
8/20. Delayed central nervous system superficial siderosis following brachial plexus avulsion injury. Report of three cases.Chronic subarachnoid hemorrhage may cause deposition of hemosiderin on the leptomeninges and subpial layers of the neuraxis, leading to superficial siderosis (SS). The symptoms and signs of SS are progressive and fatal. Exploration of potential sites responsible for intrathecal bleeding and subsequent hemosiderin deposition may prevent disease progression. A source of hemorrhage including dural pathological entities, tumors, and vascular lesions has been previously identified in as many as 50% of patients with SS. In this report, the authors present three patients in whom central nervous system SS developed decades after brachial plexus avulsion injury. They believe that the traumatic dural diverticula in these cases may be a potential source of bleeding. A better understanding of the pathophysiology of SS is important to develop more suitable therapies.- - - - - - - - - - ranking = 0.83333333333333keywords = siderosis (Clic here for more details about this article) |
9/20. 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.- - - - - - - - - - ranking = 0.83333333333333keywords = siderosis (Clic here for more details about this article) |
10/20. Superficial siderosis of the central nervous system following cervical nerve root avulsion: the importance of early diagnosis and surgery.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.- - - - - - - - - - ranking = 0.83333333333333keywords = siderosis (Clic here for more details about this article) |
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