scholarly journals Superficial siderosis: Case report and literature review

2013 ◽  
Vol 141 (3-4) ◽  
pp. 219-222
Author(s):  
Nikola Kresojevic ◽  
Igor Petrovic ◽  
Natasa Dragasevic­Miskovic ◽  
Vladimir Kostic
Keyword(s):  
Spinal Cord ◽  
Hearing Loss ◽  
Mr Imaging ◽  
Brain Mri ◽  
Therapeutic Interventions ◽  
Superficial Siderosis ◽  
Mri Findings ◽  
Hand Tremor ◽  
Hemosiderin Deposition ◽  
Clinical Triad

Introduction. Superficial siderosis (SS) is caused by chronic subarachnoid bleeding and is characterized by free iron and hemosiderin deposition along the pial and subpial structures of central nervous system. SS leads to progressive and irreversible CNS damage. The most common causes of chronic subarachnoidal bleeding are tumors, head and spinal cord trauma, arteriovenous malformations and aneurysms. SS is characterized by clinical triad: sensorineural hearing loss, cerebellar ataxia and piramydal signs. Brain MR imaging is the investigation of choice for the diagnosis of SS. Typical findings include hypointensities seen on T2?weighted MR imaging around the brain, cerebellum, brain stem, spinal cord, VIII cranial nerve and atrophy of cerebellum and medulla. Case Outline. A 71-year?old female patient noticed hand tremor in the middle of the third decade of life, and later slowly progressive bilateral hearing loss. At the age of 64 she developed un? steady gate, hand clumsiness and dysarthria, to became incapable of independent walking and standing five years later. Clinical course and brain MRI findings were typical for SS, but additional investigation did not reveal the couse of subarahnoidal bleeding. Conclusion. SS represents a rare and under?recognized condition that must be considered in all patients with cerebellar syndrome of unknown cause. Early diagnosis of SS in some cases with identified cause of chronic bleeding allowes therapeutic interventions that may prevent further progression of the disease.

Clival meningocele causing bilateral hearing loss in a child due to superficial siderosis of the central nervous system: case report

2018 ◽  
Vol 21 (5) ◽  
pp. 498-503 ◽  
Author(s):  
Stephen J. Johans ◽  
Kevin N. Swong ◽  
Daniel J. Burkett ◽  
Michael P. Wemhoff ◽  
Sean M. Lew ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Hearing Loss ◽  
Case Report ◽  
Subarachnoid Space ◽  
Case Reports ◽  
Hearing Threshold ◽  
Superficial Siderosis ◽  
Dural Defect ◽  
Hemosiderin Deposition

Superficial siderosis (SS) of the CNS is a rare and often unrecognized condition. Caused by hemosiderin deposition from chronic, repetitive hemorrhage in the subarachnoid space, it results in parenchymal damage in the subpial layers of the brain and spinal cord. T2-weighted MRI shows the characteristic hypointensity of hemosiderin deposition, classically occurring around the cerebellum, brainstem, and spinal cord. Patients present with progressive gait ataxia and sensorineural hearing impairment. Although there have been several studies, case reports, and review articles over the years, the clear pathophysiology of subarachnoid space hemorrhage remains to be elucidated. The proposed causes include prior intradural surgery, prior trauma, tumors, vascular abnormalities, nerve root avulsion, and dural abnormalities.Surgical repair of a dural defect associated with SS has been shown to be efficacious at preventing symptomatic progression. There have been several reports of dural defects within the spinal canal treated with surgery. Here, the authors present the first known case of a dural defect of the ventral skull base, namely a clival meningocele, presumed to be causing SS. In this case report, a 10-year-old girl with a history of head trauma at the age of 3 years was found to have a clival meningocele 3 years after her original trauma. On follow-up imaging, the patient was found to have radiographic growth of the meningocele along with evidence of SS of the CNS. The patient was treated conservatively until she began to have progressive hearing loss. It was presumed that the growing meningocele was the source of her SS. An endoscopic endonasal transclival approach with a multilayer dural reconstruction was performed to fix the dural defect and repair the meningocele in hopes of mitigating the progression of her symptoms. At her 12-month postoperative follow-up, she was doing well, with audiometry showing a slightly decreased hearing threshold in the left ear but improved speech discrimination bilaterally. Postoperative MRI showed a stable level of hemosiderin deposition and meningocele repair. Long-term follow-up will be necessary to evaluate for continued clinical stabilization or possible improvement.

scholarly journals Idiopathic Superficial Siderosis of the Central Nervous System

2021 ◽  
Author(s):  
Shakila Meshkat ◽  
Parnia Ebrahimi ◽  
Abbas Tafakhori ◽  
Aidin Taghiloo ◽  
Sajad Shafiee ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hearing Loss ◽  
Sleep Disturbances ◽  
Brain Mri ◽  
Spastic Paraparesis ◽  
Central Nervous System Disease ◽  
Neurological Deficits ◽  
Superficial Siderosis ◽  
System Disease

Abstract Background: Regardless of the cause of the superficial siderosis (SS) disease, which is bleeding, the source of bleeding cannot be found in some cases.Case presentation: In this article, we report two cases with idiopathic SS. Case 1 presented with bilateral hearing loss, cognitive impairment, sleep disturbances, and tremors. Case 2 presented with sensory neural hearing loss, ataxia, and spastic paraparesis. In both cases, brain MRI indicated evidence of SS. CT myelogram and SPECT with labeled RBC couldn't help finding the source of occult bleeding.Conclusion: SS is a rare central nervous system disease caused by the deposition of hemosiderin in the brain and spinal cord, which results in the progression of neurological deficits. The cause of this hemorrhage is often subarachnoid haemorrhage, intradural surgery, carcinoma, arteriovenous malformation, nerve root avulsion, and dural abnormality. The condition progresses slowly and, by the time diagnosis is confirmed, the damage is often irreversible. In our cases, brain MRI clarified the definitive diagnosis, but we could not find the source of bleeding. SS should be considered in cases with ataxia and hearing loss, even if no source of bleeding is found.

scholarly journals Evaluation of Brain Magnetic Resonance Imaging (MRI) Findings of Neonates with Clinical Seizure Admitted to Yazd Shahid Sadoughi Hospital and its Effect on Diagnostic and Therapeutic Interventions of Newborns from 2015-2016

2020 ◽  
Author(s):  
Razieh Fallah ◽  
Mohammad Javad Asadi ◽  
Reza Nafisi Moghadam ◽  
Mohammad Hossein Ahrar Yazdi
Keyword(s):  
Magnetic Resonance Imaging ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Common Type ◽  
Therapeutic Interventions ◽  
Resonance Imaging ◽  
Mri Findings ◽  
Clinical Seizure ◽  
Magnetic Resonance Imaging Mri ◽  
Abnormal Brain

Introduction: In neonatal period, brain magnetic resonance imaging (MRI) is the best neuroimaging to find etiology of seizure. The aim of this study was to evaluate brain MRI findings of neonates with clinical seizure and its effect on diagnostic and therapeutic interventions of newborn. Methods: In a retrospective study, medical records and brain MRI findings of neonates with clinical seizure admitted to Neonatal Intensive Care Unit or Pediatric Ward of Shahid Sadoughi Hospital, Yazd, Iran from September 2018 and before were evaluated. The data were analyzed using SPSS version 16 software , the required indicators and tables were prepared and Fisher exact test and Chi-square test were used to determine the relationship between qualitative variables and independent t-test was used to compare the means in the two groups Results: Twenty-five girls and 38 boys were studied. The cause of seizures was found in 94% and the most common cause of congenital hypoxia was in 22 infants (35%). The most common type of seizure was tonic in 23 infants (36.5%) and the most common type of generalized seizure was in 47 infants (75%). Brain MRI was abnormal in 19 neonates (30%). Based on the MRI results, there was a change in therapeutic interventions (brain surgery) in four infants (6.3%) and in diagnostic interventions (metabolic tests) in 19 infants (30%). Abnormal brain MRI was more frequent in neonates by cesarean section (46%)  than  normal vaginal delivery (19%), (p= 0.01) and also neonates with partial seizure (37.5%) had an abnormal MRI than  generalized seizure (21%), (p= 0.03). Mean of hospitalization days was longer in neonates with abnormal brain MRI (12.32±2.76 days) than neonates with normal MRI (8.57±2.82 days) (P = 0.02). Conclusion: Based on the results of this study, brain MRI might be useful in finding intracranial pathology that causes seizure in neonates by cesarean section to detect birth asphyxia and in newborns with partial seizure.  

Surgical treatment of superficial siderosis associated with a spinal arteriovenous malformation

1998 ◽  
Vol 89 (6) ◽  
pp. 1029-1031 ◽  
Author(s):  
Wouter I. Schievink ◽  
Paul J. Apostolides ◽  
Robert F. Spetzler
Keyword(s):  
Hearing Loss ◽  
Mr Imaging ◽  
Arteriovenous Malformation ◽  
Superficial Siderosis ◽  
Spinal Angiography ◽  
Content Type ◽  
Spinal Arteriovenous Malformation ◽  
History Of ◽  
The Central Nervous System ◽  
Fine Print

✓ In many patients with superficial siderosis of the central nervous system (CNS) no source of bleeding can be established, despite extensive examinations. The authors report a patient with superficial siderosis and a spinal arteriovenous malformation (AVM) that was not visible on magnetic resonance (MR) imaging or myelography but was identified on angiographic studies. This 71-year-old man presented with a 2-year history of progressive gait difficulties and hearing loss. Examination showed ataxia, hearing loss, and quadriparesis. On MR imaging superficial siderosis of the brain and spinal cord as seen; however, MR imaging of the CNS, as well as cerebral angiography and myelography studies, did not reveal the source of hemorrhage. Spinal angiography revealed a small slow-flow pial AVM at the C-5 level originating from the anterior spinal artery. A C-5 corpectomy was performed and the AVM was obliterated. The patient did well and reported no further progression of his symptoms during 3 months of follow up. Spinal angiography is indicated to complete the evaluation of patients with superficial siderosis, even if results of spinal MR imaging and myelography studies are normal. Obliteration of spinal AVMs may successfully prevent the progression of superficial siderosis.

scholarly journals MRI differences between MOG antibody disease and AQP4 NMOSD

2020 ◽  
Vol 26 (14) ◽  
pp. 1854-1865 ◽  
Author(s):  
Sara Salama ◽  
Majid Khan ◽  
Amirali Shanechi ◽  
Michael Levy ◽  
Izlem Izbudak
Keyword(s):  
Spinal Cord ◽  
White Matter ◽  
Area Postrema ◽  
Brain Mri ◽  
White Matter Lesions ◽  
Mri Findings ◽  
Mri Features ◽  
Clinical Presentations ◽  
The Brain ◽  
Aqp4 Antibody

Background: MOG antibody and AQP4 antibody seropositive diseases are immunologically distinct subtypes of neuromyelitis optica spectrum disorders (NMOSD) with similar clinical presentations. MRI findings can be instrumental in distinguishing MOG antibody disease from AQP4 antibody NMOSD. Objectives: The aim of this study is to characterize the neuroradiological differences between MOG antibody disease and AQP4 antibody NMOSD with the aim to distinguish between the two entities. Methods: This is a retrospective study of 26 MOG and 25 AQP4 seropositive patients in which MRI features of the brain, spinal cord, and orbit were compared. Results: The majority of the abnormal findings in the MOG cohort were located on orbital MRIs, while spinal cord magnetic resonance (MR) abnormalities were more common in the AQP4 cohort. Brain abnormalities showed some overlap, but cortical gray/juxtacortical white matter involvement was distinct to MOG patients, while area postrema involvement was a rare feature. Conclusion: Cortical gray/juxtacortical white matter lesions on brain MRI might help distinguish MOG antibody disease from AQP4-positive NMOSD. These findings could be of value in distinguishing the two entities as early as the first presentation.

Susac-Syndrom: ein diagnostisches Chamäleon

2019 ◽  
Vol 98 (04) ◽  
pp. 268-275
Author(s):  
M. I. Stefanou ◽  
D. Doycheva ◽  
A. Ebrahimi ◽  
J.-M. Dörr ◽  
U. Ziemann
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Interdisciplinary Collaboration ◽  
Immunosuppressive Treatment ◽  
Response To Therapy ◽  
Sensorineural Hearing ◽  
Neurological Deficits ◽  
Mri Findings ◽  
Diagnostic Features ◽  
Clinical Triad

AbstractSusac’s syndrome (SuS) is a rare, probably autoimmune endotheliopathy of the central nervous system, retina and inner ear. It is characterized by a clinical triad of encephalopathy, branch retinal artery occlusions (BRAOs) and sensorineural hearing loss. To date, more than 300 cases of SuS have been reported in the literature. However, SuS remains an under- and misdiagnosed entity in the clinical setting. This report presents an exemplary case of a patient, who was initially misdiagnosed with relapsing-remitting multiple sclerosis. At initial presentation, the patient did not demonstrate the complete clinical triad, and the interval between symptom onset and diagnosis was 4 months. Typical diagnostic features, which enabled the diagnosis of SuS were: a) MRI findings with T2-hyperintense snowball-like lesions of the corpus callosum and subcortical white matter and hyperintense lesions in diffusionweighted imaging with reduced apparent diffusion coefficient; b) BRAOs and vessel wall hyperfluorescence in fluorescein angiography and a significant thickness reduction of the inner retinal layers in optical coherence tomography; c) bilateral sensorineural hearing loss. The patient was aggressively treated with cyclophosphamide, rituximab, glucocorticoids and acetylsalicylic acid with a good response to therapy. This report draws attention to the need to take SuS into consideration in the differential diagnosis at the interface of neurological, psychiatric, ophthalmological and otorhinolaryngological disorders. As SuS may result in severe and persistent neurological deficits, an interdisciplinary collaboration is fundamental for the prompt diagnosis and initiation of adequate immunosuppressive treatment.

scholarly journals Idiopathic superficial siderosis of the central nervous system

2021 ◽  
Vol 8 (1) ◽  
Author(s):  
Shakila Meshkat ◽  
Parnia Ebrahimi ◽  
Abbas Tafakhori ◽  
Aidin Taghiloo ◽  
Sajad Shafiee ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hearing Loss ◽  
Sleep Disturbances ◽  
Brain Mri ◽  
Spastic Paraparesis ◽  
Central Nervous System Disease ◽  
Neurological Deficits ◽  
Superficial Siderosis ◽  
Intracranial Surgery

Abstract Background Regardless of the cause of the superficial siderosis (SS) disease, which is bleeding, the source of bleeding cannot be found in some cases. Case presentation In this article, we report two cases with idiopathic SS. Case 1 presented with bilateral hearing loss, cognitive impairment, sleep disturbances, and tremors. Case 2 presented with sensory neural hearing loss, ataxia, and spastic paraparesis. In both cases, brain MRI indicated evidence of SS. CT myelogram and SPECT with labeled RBC couldn’t help finding the source of occult bleeding. Conclusion SS is a rare central nervous system disease caused by the deposition of hemosiderin in the brain and spinal cord, which results in the progression of neurological deficits. The cause of this hemorrhage is often subarachnoid haemorrhage, intracranial surgery, carcinoma, arteriovenous malformation, nerve root avulsion, and dural abnormality. The condition progresses slowly and, by the time diagnosis is confirmed, the damage is often irreversible. In our cases, brain MRI clarified the definitive diagnosis, but we could not find the source of bleeding. SS should be considered in cases with ataxia and hearing loss, even if no source of bleeding is found.

scholarly journals Idiopathic Superficial siderosis of the central nervous system

2020 ◽  
Author(s):  
Shakila Meshkat ◽  
Parnia Ebrahimi ◽  
Abbas Tafakhori ◽  
Aidin Taghiloo ◽  
Sajad Shafiee ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hearing Loss ◽  
Sleep Disturbances ◽  
Brain Mri ◽  
Spastic Paraparesis ◽  
Central Nervous System Disease ◽  
Neurological Deficits ◽  
Superficial Siderosis ◽  
System Disease

Abstract Background: Regardless of the cause of the superficial sidrosis (SS) disease, which is bleeding, the source of bleeding cannot be found in some cases. Case presentation: In this article, we report two cases with idiopathic SS. Case 1 presented with bilateral hearing loss, cognitive impairment, sleep disturbances and tremors. Case 2 presented with sensory neural hearing loss, ataxia, and spastic paraparesis. Brain MRI indicated evidence of SS. CT myelogram and SPECT/CT with labeled RBC couldn't help finding the source of occult bleeding. Conclusion: Superficial sisdorsis (SS) is a rare central nervous system disease caused by deposition of hemosiderin in brain and spinal cord which results in progression of neurological deficits. The cause of this hemorrhage is often subarachnoid haemorrhage, intradural surgery, carcinoma, arteriovenous malformation, nerve root avulsion, and dural abnormality. The condition progresses slowly and, by the time diagnosis is confirmed, the damage is often irreversible. In our cases, brain MRI clarified the definitive diagnosis but we could not find the source of bleeding. SS should be considered in cases with ataxia and hearing loss even if no source of bleeding is found.

Clinical and brain MR imaging effectively influences the ATP7B genotype of Neurologic Wilson patients

2020 ◽  
Author(s):  
Juan Wang ◽  
Yongzhu Han ◽  
Renmin Yang ◽  
Xuen Yu ◽  
Juncang Wu
Keyword(s):  
Mr Imaging ◽  
Gene Mutation ◽  
Wilson Disease ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Homozygous Mutation ◽  
Mri Findings ◽  
Its Gene ◽  
The Relationship ◽  
Mutation Type

Abstract Objective To understand the relationship between the two types of mutations in patients with Wilson disease (WD) and clinical practice, and to search for the clinical biological markers of the two types of mutations. Methods The hospitalized patients, who were in the affiliated hospital of neurology institute of anhui university of traditional Chinese medicine from May 2014 to May 2019, with p. arg778leu or p. pro992leu homozygous mutation type of neurologic WD, were selected and underwent demographic, clinical manifestations, serological indicators and brain MR imaging(MRI) data were analyzed to compare the differences of the two mutant types of neurologic WD. ResultsThe group of 103 patients with neurologic WD join in this research, including p.A rg778Leu mutant WD 65 cases and p.P ro992Leu mutant WD 38 cases. The two types of mutations in the WD demographic, clinical manifestation and most serological index indifference, and brain MRI findings have significant differences, especially the p.A rg778Leu mutant WD damage the thalamus(χ2 =17.834, P<0.001), midbrain(χ2 =12.579, P<0.001) and pons(χ2 =10.605, P=0.001)p.P ro992Leu mutant WD have obvious difference, the results of multivariate analysis were also different (P<0.05). Conclusions The demography, clinical features and serology of neurologic WD have nothing to do with its gene mutation type, and the MRI manifestations of brain are related to its gene mutation type, among which the ATP7B gene p.arg778leu mutation is more likely to involve thalamus, midbrain and pons.

Acute Flaccid Myelitis: A Recently Emerged Paralytic Syndrome in Children with Characteristic MRI Findings

Neurographics ◽  
2020 ◽  
Vol 10 (1) ◽  
pp. 66-70
Author(s):  
A. Jaju ◽  
R.A. Masum ◽  
R. Purohit ◽  
M.E. Ryan
Keyword(s):  
Spinal Cord ◽  
Mr Imaging ◽  
Respiratory Infections ◽  
Sudden Onset ◽  
Imaging Findings ◽  
Mri Findings ◽  
Neuroinflammatory Disease ◽  
Acute Flaccid Myelitis ◽  
Recent Outbreak ◽  
Enterovirus D68

Acute flaccid myelitis is a neuroinflammatory disease of the spinal cord that predominantly affects children and that presents with sudden-onset flaccid paralysis and distinctive MR imaging findings. The condition has been strongly linked to respiratory infections by enterovirus D68. Currently, in the absence of a definitive laboratory test, MR imaging findings are considered confirmatory for this condition. Herein, we describe the clinical and imaging findings of acute flaccid myelitis by using illustrative cases from the most recent outbreak in 2018.

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