In this patient, 2 microbleeds (MBs) were also present (not shown). (C, D) An 84-year-old woman with CAA with disseminated, bilateral frontal and parietal superficial siderosis (e.g., arrows), in whom no ICHs or MBs were present. [ncbi.nlm.nih.gov]

[…] be the first symptom, which may be accompanied by tinnitus.5,13 Cerebellar dysarthria is common and nystagmus may be present.9 A myelopathic presentation is also recognized. [jamanetwork.com]

We were unable to identify the exact source of the bleeding, however, bleeding was present in the subarachnoid space. [nnjournal.net]

Histologically, Prussian blue stains the iron blue and shows it to be present in all ocular epithelial structures and in areas of trabecular meshwork scarring and retinal gliosis. [eyewiki.org]

• Weakness

  There was a weak association between disease duration and multiattribute scores for HUI3 (R = 0.353; adjusted R2 = 0.096; b = −0.008; p = 0.047) but not EQ5D. [n.neurology.org]

  After that, the patient showed graual improvement of the weakness. One month after the surgery, strength was 4+/4+ bilaterally in both lower extremities. [ncbi.nlm.nih.gov]

  The weakness of MRI diagnosis, however, relates to the unknown relationship between radiographic findings and symptomatic disease. In a study of 13 patients with iSS diagnosed with MRI findings, only 15% had clinical symptoms ( 10 ). [frontiersin.org]

  Physical findings: Individuals with Superficial Siderosis of the CNS may have difficulty with: • Ability to smell and taste; • Co-ordination and weakness of limbs; • Difficulty with bladder and bowel functions; • Bilateral sciatica; and • Pain in lower [secure.ssa.gov]

• Pain

  Physical findings: Individuals with Superficial Siderosis of the CNS may have difficulty with: • Ability to smell and taste; • Co-ordination and weakness of limbs; • Difficulty with bladder and bowel functions; • Bilateral sciatica; and • Pain in lower [secure.ssa.gov]

• Asymptomatic

  While it takes 6 months for hemosiderin to deposit after weekly autologous red cell injection in animal models ( 23 ), human patients experience an asymptomatic period anywhere from 2 to 51 years ( 8 ). [frontiersin.org]

• Impaired Balance

  Ataxia: Impaired balance and coordination Myelopathy: Severe compression of the spinal cord The neural damage from Superficial Siderosis may be permanent, and disability will continue to progress even if an active bleed source is repaired. [massgeneral.org]

• Anosmia

  With continued bleeding, other areas of the brain can degenerate leading to a myriad of other symptoms seen in superficial siderosis including urinary problems headaches, anosmia, diplopia, bowel problems, ageusia, cranial nerve palsies, and dementia. [clinicaltrials.gov]

  Disturbances of the bladder Anosmia - Loss of sense of smell. [en.wikipedia.org]

  Sensory symptoms or a sensory level are rarely present.8 Anosmia or hyposmia are common but underreported, and olfaction is rarely tested.9 Extraocular nerve palsies are uncommon but have been reported.2 Also reported are optic9 and trigeminal neuropathy [jamanetwork.com]

  Other symptoms include urinary pathology, headaches, anosmia, ageusia, diplopia, bowel problems, cranial nerve palsies, memory deterioration, personality changes, and nystagmus ( 21 ). [frontiersin.org]

• Cough

  These people have had persistent breathlessness, coughing and decreased lung function. [en.wikipedia.org]

• Hyposmia

  Sensory symptoms or a sensory level are rarely present.8 Anosmia or hyposmia are common but underreported, and olfaction is rarely tested.9 Extraocular nerve palsies are uncommon but have been reported.2 Also reported are optic9 and trigeminal neuropathy [jamanetwork.com]

• Hypertension

  It has been suggested that pial siderosis may result in sclerosis of the veins, with venous hypertension and prominent vasculature.17 An interruption in the circumferential hypointensity may indicate root avulsion and is possibly caused by absence of [jamanetwork.com]

• Tinnitus

  It possible that despite not including hearing as a domain, the EQ5D anxiety/depression domain was partially able to capture the effect of tinnitus on HRQoL because it is well known to be related to anxiety and stress. [n.neurology.org]

  In the present case, the patient exhibited ataxia, diplopia, voiding difficulty and bilateral tinnitus with preserved hearing. [ncbi.nlm.nih.gov]

  The most common clinical presentation is slowly progressive cerebellar ataxia, which is often associated with hearing impairment.2,3 Sensorineural hearing loss (at times with vestibular failure) may be the first symptom, which may be accompanied by tinnitus [jamanetwork.com]

  According to a longitudinal single case report characterizing hearing loss in SSCNS showed over a 15-year period, showed prodromal tinnitus followed by sensorineural hearing loss and eventually total deafness ( 24 ). [frontiersin.org]

• Hearing Problem

  The median time interval between the onset of hearing symptoms and the diagnosis was 4 (IQR 11) years; 12% participants had the onset of hearing problems after the diagnosis (all within 3 years), and 10% participants reported the onset of hearing problems [n.neurology.org]

• Incontinence

  He also reports of incontinence, but no reports of seizures. CCSF findings revealed the etiology and pathology mechanism of SSCNS. T2-weighted MRI and SWI showed that reliable changes of the head imaging. [nnjournal.net]

• Nystagmus

  […] progressive cerebellar ataxia, which is often associated with hearing impairment.2,3 Sensorineural hearing loss (at times with vestibular failure) may be the first symptom, which may be accompanied by tinnitus.5,13 Cerebellar dysarthria is common and nystagmus [jamanetwork.com]

  Other symptoms include urinary pathology, headaches, anosmia, ageusia, diplopia, bowel problems, cranial nerve palsies, memory deterioration, personality changes, and nystagmus ( 21 ). [frontiersin.org]

• Dysarthria

  […] presentation is slowly progressive cerebellar ataxia, which is often associated with hearing impairment.2,3 Sensorineural hearing loss (at times with vestibular failure) may be the first symptom, which may be accompanied by tinnitus.5,13 Cerebellar dysarthria [jamanetwork.com]

• Unable to Walk

  […] with strangers or friends” (level 1) to “unable to be understood when speaking to other people (or unable to speak at all)” (level 5); Ambulation: from “able to walk around the neighbourhood without difficulty, and without walking equipment” (level 1 [n.neurology.org]

• Vertigo

  He had paroxysmal positional vertigo involving the right posterior canal, bilateral, symmetric high-frequency-hearing loss, decreased vertical angular VOR gain in the vertical canals, except for normal right posterior gain. [frontiersin.org]

• Gait Ataxia

  Patients present with slowly progressive gait ataxia and sensorineural hearing impairment. A history of prior intradural surgery or trauma is common. [jamanetwork.com]

[ edit ] There is no current cure for superficial siderosis, only treatments to help alleviate the current symptoms and to help prevent the development of further symptoms. [en.wikipedia.org]

At present, we have no effective treatment for SSCNS. [ 9 ] Surgical treatment can be used to remove the potential source of recurrent SAH in order to arrest the clinical deterioration, but a number of patients has been reported to have experienced aggravated [nnjournal.net]

Treatment with deferiprone, a lipid soluble iron chelator, has reduced hemosiderin deposition, as evidenced on MRI, in a cohort of 10 patients with SSCNS. [ncbi.nlm.nih.gov]

The mean treatment duration at the study participation was 4 (SD 3) years. [n.neurology.org]

Cummins et al. reported improved gait and hearing bilaterally after 4 months of deferiprone treatment; however, 6 months later, hearing deteriorated to pre-treatment levels ( 45 ). [frontiersin.org]

EOG abnormalities indicate severe metallosis and poor visual prognosis. The ERG is a sensitive way of monitoring intraocular injury from metallic ion poisoning. [eyewiki.org]

Any damage is thus permanent. [9] Symptoms can be treated. [5] Prognosis [ edit ] Outcome of pulmonary siderosis is often good if the inhalation of iron or rust dust is permanently avoided. [en.wikipedia.org]

EOG abnormalities indicate severe metallosis and poor visual prognosis. [eyewiki.aao.org]

Etiology There are various underlying etiologies for chronic SAH. A comprehensive review of the literature was first performed by Fearnley et al. in 1995, and much of the understanding of the initial epidemiology of SSCNS relied on this study. [frontiersin.org]

The etiologies of chronic subarachnoid bleeding are (in order of incidence): Idiopathic, Head/back trauma, A/V malformations, Current CNS tumor, Previously resected CNS tumor, CNS post-surgical (non-tumor), Amyloid angiopathy, Brachial plexus/root injury [clinicaltrials.gov]

Kumar NLindell EPWilden JADavis DH Role of dynamic CT myelography in identifying the etiology of superficial siderosis. Neurology 2005;65486- 488PubMedGoogle ScholarCrossref 19. [jamanetwork.com]

CCSF findings revealed the etiology and pathology mechanism of SSCNS. T2-weighted MRI and SWI showed that reliable changes of the head imaging. Combined with the patient’s clinical manifestations, the diagnosis of SSCNS was obtained. [nnjournal.net]

Virtually all ocular structures are involved in the siderotic process, resulting in possible glaucoma, cataract, iris color changes, mydriasis, retinal function destruction, and optic nerve atrophy.[1][5] Etiology Ocular siderosis is the result of an [eyewiki.aao.org]

Epidemiology Males aged 22-25 years old are most common patient demographic seen with metallic IOFB injuries.[3][7][9] Approximately 78% to 86% of all IOFBs are metallic. [eyewiki.aao.org]

A comprehensive review of the literature was first performed by Fearnley et al. in 1995, and much of the understanding of the initial epidemiology of SSCNS relied on this study. [frontiersin.org]

In this review, we provide an overview of the pathophysiology and methods of diagnosis of SSCNS vestibulopathy. [frontiersin.org]

All the above types of phagocytic cells suggested that the cerebral hemorrhage had not stopped or might be a sign of rebleeding. [ 8 ] This kind of performance was consistent with its pathogenesis and pathophysiology. [nnjournal.net]

The pathophysiology of SSCNS primarily results from hemosiderin desposition in the subaracnoid space. Hemosiderin is found within cells and appears to be a complex of ferritin, denatured ferritin and other materials. [ncbi.nlm.nih.gov]

[…] entry site for IOFBs is the cornea (82.9%), followed by the sclera (11.4%), and the limbus (5.7%).[11] If lodged in the posterior segment, the most likely location is intravitreal (61%), followed by intraretinal (14%) and subretinal (5%).[3][12][9][2] Pathophysiology [eyewiki.aao.org]

However, once the neurodegenerative process has begun, surgical intervention does not prevent the neurodegenerative disease from progressing. [clinicaltrials.gov]

An early diagnosis and prompt intervention directed at removal of the bleeding source may prevent progression. [jamanetwork.com]

[…] disease and is not well described in neurological texts, so it may go unnoticed until noticeable symptoms appear. [9] Treatment [ edit ] There is no current cure for superficial siderosis, only treatments to help alleviate the current symptoms and to help prevent [en.wikipedia.org]

91] Use of subconjunctival deferoxamine (10–100 mg) has been shown to prevent development of damaged due to ocular siderosis. [eyewiki.aao.org]

The deficits may evolve over a long period and could be potentially prevented if an effective hemosiderin chelator became available and used in a timely fashion. [frontiersin.org]