Superficial siderosis and nonobstructive hydrocephalus due to subependymoma in the ventricle: An illustrative case report

Background: Intraventricular tumors can generally result in obstructive hydrocephalus as they grow. Rarely, however, some intraventricular tumors develop superficial siderosis (SS) and trigger hydrocephalus, even though the tumor has hardly grown. Here, we present an illustrative case of SS and nonocclusive hydrocephalus caused by subependymoma of the lateral ventricles. Case Description: A 78-year-old man with an intraventricular tumor diagnosed 7 years ago had been suffering from gait disturbance for 2 years. He also developed cognitive impairment. Intraventricular tumors showed little growth on annual magnetic resonance imaging (MRI). MRI T2-star weighted images (T2*WI) captured small intratumoral hemorrhages from the beginning of the follow-up. Three years before, at the same time as the onset of ventricular enlargement, T2*WI revealed low intensity in the whole tumor and cerebral surface. Subsequent follow-up revealed that this hemosiderin deposition had spread to the brain stem and cerebellar surface, and the ventricles had expanded further. Cerebrospinal fluid (CSF) examination revealed xanthochromia. The tumor was completely removed en bloc. Histopathological findings were consistent with those of subependymoma. Although CSF findings improved, SS and hydrocephalus did not improve. Therefore, the patient underwent a lumboperitoneal shunt for CSF diversion after tumor resection. Conclusion: Some intraventricular tumors cause SS and nonobstructive hydrocephalus due to microbleeding, even in the absence of tumor growth. T2*WI and, if necessary, timely CSF examination can allow identification of presymptomatic SS. This follow-up strategy may provide a favorable course by facilitating early intervention in patients with intraventricular lesions, not just subependymomas.

Cochlea implantation in patients with superficial hemosiderosis

Introduction Superficial hemosiderosis is a sub-form of hemosiderosis in which the deposits of hemosiderin in the central nervous system damage the nerve cells. This form of siderosis is caused by chronic cerebral hemorrhages, especially subarachnoid hemorrhages. The diversity of symptoms depends on the respective damage to the brain, but in most of the cases it shows up as incipient unilateral or bilateral hearing loss, ataxia and signs of pyramidal tracts. We are investigating the question of whether cochlear implantation is a treatment option for patients with superficial hemosiderosis and which strategy of diagnostic procedure has to be ruled out preoperatively. Materials and methods In a tertiary hospital between 2009 and 2018, we examined (N = 5) patients with radiologically confirmed central hemosiderosis who suffered from profound hearing loss to deafness were treated with a cochlear implant (CI). We compared pre- and postoperative speech comprehension (Freiburg speech intelligibility test for monosyllables and HSM sentence test). Results Speech understanding improved on average by 20% (monosyllabic test in the Freiburg speech intelligibility test) and by 40% in noise (HSM sentence test) compared to preoperative speech understanding with optimized hearing aids. Discussion The results show that patients with superficial siderosis benefit from CI with better speech understanding. The results are below the average for all postlingual deaf CI patients. Superficial siderosis causes neural damages, which explains the reduced speech understanding based on central hearing loss. It is important to correctly weigh the patient's expectations preoperatively and to include neurologists within the therapy procedure.

The contribution of susceptibility-weighted imaging (SWI) in the central nervous system superficial siderosis’ evaluation in 3 Tesla magnetic resonance imaging (MRI)

Objective. The aim of this study is to evaluate the magnetic resonance imaging (MRI) findings of central neural system (CNS) superficial siderosis cases and the diagnostic contribution of the susceptibility-weighted imaging (SWI) sequence to conventional imaging. Method. TSE T2-weighted and SWI-MRI of 26 patients diagnosed as CNS-superficial siderosis (CNS-SS) were retrospectively evaluated with 3-Tesla MRI. The localization and type of involvement of SS were reviewed. Results. The CNS-SS were divided into two categories as central amyloid angiopathy-SS (CAA-SS) and non central amyloid angiopathy-SS (non-CAA-SS). In non-CAA cases, the involvement was typical (classic) in 5 cases and atypical in 9 cases. In 12 of these cases (85.7%), SS findings were observed on both turbo spine echo (TSE) T2 images and SWI imaging, while in 2 cases (14.3%) SS was detected only on SWI images. In 7 of the CAA-SS cases, involvement was focal type SS (58.33%), while 5 cases had diffuse type SS (41.67%) involvement. In the vast majority of cases (n = 10) of this type of SS, involvement was detected only in SWI images, while siderosis was not detected in TSE T2 images. In addition, occult cerebral vascular malformation accompanying SS, which can be observed only in the SWI sequence, was found in a total of 4 cases. In the cross-matching statistical analysis performed between CAA-SS and non-CAA-SS groups and subgroups, SWI was found to be significantly superior to T2 in detecting SS in the CAA-SS group (p:0,007). Conclusions. SWI imaging was superior in detecting SS and detecting cerebral occult vascular malformation in CAA-SS cases. Although the detectability of SS by SWI was high in other groups, no statistically significant difference was found. Under these circumstances, we think that it will be beneficial to add SWI imaging to the routine imaging protocol in cases with suspected CNS-SS.

Cerebellar Superficial Siderosis in Cerebral Amyloid Angiopathy

Background and Purpose: Although evidence accumulates that the cerebellum is involved in cerebral amyloid angiopathy (CAA), cerebellar superficial siderosis is not considered to be a disease marker. The objective of this study is to investigate cerebellar superficial siderosis frequency and its relation to hemorrhagic magnetic resonance imaging markers in patients with sporadic and Dutch-type hereditary CAA and patients with deep perforating arteriopathy–related intracerebral hemorrhage. Methods: We recruited patients from 3 prospective 3 Tesla magnetic resonance imaging studies and scored siderosis and hemorrhages. Cerebellar siderosis was identified as hypointense linear signal loss (black) on susceptibility-weighted or T2*-weighted magnetic resonance imaging which follows at least one folia of the cerebellar cortex (including the vermis). Results: We included 50 subjects with Dutch-type hereditary CAA, (mean age 50 years), 45 with sporadic CAA (mean age 72 years), and 43 patients with deep perforating arteriopathy–related intracerebral hemorrhage (mean age 54 years). Cerebellar superficial siderosis was present in 5 out of 50 (10% [95% CI, 2–18]) patients with Dutch-type hereditary CAA, 4/45 (9% [95% CI, 1–17]) patients with sporadic CAA, and 0 out of 43 (0% [95% CI, 0–8]) patients with deep perforating arteriopathy–related intracerebral hemorrhage. Patients with cerebellar superficial siderosis had more supratentorial lobar (median number 9 versus 2, relative risk, 2.9 [95% CI, 2.5–3.4]) and superficial cerebellar macrobleeds (median number 2 versus 0, relative risk, 20.3 [95% CI, 8.6–47.6]) compared with patients without the marker. The frequency of cortical superficial siderosis and superficial cerebellar microbleeds was comparable. Conclusions: We conclude that cerebellar superficial siderosis might be a novel marker for CAA.

Long-term Risks of Persistent Ventral Spinal CSF Leaks in SIH: Superficial Siderosis and Bibrachial Amyotrophy

Background and Objectives:Superficial siderosis, bibrachial amyotropy, and spinal cord herniation are unusual but serious long-term sequelae of persistent spontaneous spinal CSF leaks in patients with spontaneous intracranial hypotension (SIH), particularly ventral spinal CSF leaks. However, the risk of developing such sequelae has not been established in this population. We undertook this study to determine the risk of these serious complications of persistent ventral spinal CSF leaks.Methods:This cohort study was conducted using data from a prospectively maintained data base of patients who meet the modified International Classification of Headache Disorders (ICHD)-III criteria for SIH. The patient population consisted of a consecutive group of patients with SIH and persistent ventral spinal CSF leaks, who were first seen within one year of onset of SIH symptoms and who had at least one year of follow-up.Results:Among 51 patients with SIH and a persistent ventral spinal CSF leak, superficial siderosis developed in six patients and bibrachial amyotropohy in two patients during 280 patient years of follow-up. The probability of these complications increased from 0% at 48 months, to 4.5% (95% confidence interval (CI): 1.0-28.0%) at 56 months, 10.5% (95% CI: 3.0-36.4%) at 96 months, 32.7% (95% CI: 15.0-62.8%) at 144 months, and 57.9% (95% CI: 30.2-87.6%) at 192 months. None of the patients developed spinal cord herniation.Discussion:Among patients with SIH and a persistent ventral spinal CSF leak, the risk of developing serious long-term sequelae is considerable. This study shows that early treatment of a ventral spinal CSF leak offers a unique opportunity to prevent neurologic disability from superficial siderosis and bibrachial amyotrophy.

Superficial siderosis associated with an iatrogenic posterior fossa dural leak identified on CT cisternography

Superficial siderosis refers to hemosiderin deposition along the pial surface of the brain and spinal cord. It results from chronic and repetitive low-grade bleeding into the subarachnoid space. Dural tears are a common cause of superficial siderosis. Although such tears typically occur in the spine, dural tears can also occur in the posterior fossa. In many cases, posterior fossa dural tears are iatrogenic, and patients may present with neuroimaging evidence of postoperative pseudomeningoceles. We present a case of superficial siderosis caused by a persistent posterior fossa dural leak. The patient presented with superficial siderosis 30 years after a Chiari I malformation repair. A pinhole-sized dural tear was identified preoperatively using computed tomography cisternography. The dural defect was successfully repaired. An additional small tear that was not seen on imaging was also identified at surgery and successfully repaired.

Hemosiderosis of Central Nervous System: a case report of a rare and underdiagnosed disease

Superficial Siderosis (SS) of Central Nervous System is a rare disease characterized by the deposit of hemosiderin in the brain and spinal cord. Clinically, it is characterized by progressive sensorineural ataxia and deafness associated with injury of superior motor neuron. The diagnosis is made by magnetic resonance imaging (MRI) of the encephalon and spinal cord. The objective of the study is to report the case of a patient with characteristic elements of the syndrome, accompanied in a private medical clinic.

Cognitive Impairment After Intracerebral Hemorrhage: A Systematic Review of Current Evidence and Knowledge Gaps

Background: Cognitive impairment (CI) is commonly observed after intracerebral hemorrhage (ICH). While a growing number of studies have explored this association, several evidence gaps persist. This review seeks to investigate the relationship between CI and ICH.Methods: A two-stage systematic review of research articles, clinical trials, and case series was performed. Initial search used the keywords [“Intracerebral hemorrhage” OR “ICH”] AND [“Cognitive Impairment” OR “Dementia OR “Cognitive Decline”] within the PubMed (last accessed November 3rd, 2020) and ScienceDirect (last accessed October 27th, 2020) databases, without publication date limits. Articles that addressed CI and spontaneous ICH were accepted if CI was assessed after ICH. Articles were rejected if they did not independently address an adult human population or spontaneous ICH, didn't link CI to ICH, were an unrelated document type, or were not written in English. A secondary snowball literature search was performed using reviews identified by the initial search. The Agency for Healthcare research and Quality's assessment tool was used to evaluate bias within studies. Rates of CI and contributory factors were investigated.Results: Search yielded 32 articles that collectively included 22,631 patients. Present evidence indicates a high rate of post-ICH CI (65–84%) in the acute phase (<4 weeks) which is relatively lower at 3 (17.3–40.2%) and 6 months (19–63.3%). Longer term follow-up (≥1 year) demonstrates a gradual increase in CI. Advanced age, female sex, and prior stroke were associated with higher rates of CI. Associations between post-ICH CI and cerebral microbleeds, superficial siderosis, and ICH volume also exist. Pre-ICH cognitive assessment was missing in 28% of included studies. The Mini Mental State Evaluation (44%) and Montreal Cognitive Assessment (16%) were the most common cognitive assessments, albeit with variable thresholds and definitions. Studies rarely (<10%) addressed racial and ethnic disparities.Discussion: Current findings suggest a dynamic course of post-ICH cognitive impairment that may depend on genetic, sociodemographic and clinical factors. Methodological heterogeneity prevented meta-analysis, limiting results. There is a need for the methodologies and time points of post-ICH cognitive assessments to be harmonized across diverse clinical and demographic populations.