We present a case of superficial siderosis in a 43-year-old man who presented to the Emergency Department with sudden onset bilateral visual deterioration and a loss of consciousness. [ncbi.nlm.nih.gov]

• Hearing Impairment

  Progressive ataxia and hearing impairment are the most common manifestations of the disorder. We describe a male patient admitted to our hospital for recurrent seizures over the past 3 months. [ncbi.nlm.nih.gov]

  The most characteristic symptoms are cerebellar ataxia and sensorineural hearing impairment. T2-weighted gradient echo magnetic resonance imaging constitutes the diagnostic method of choice. [pubmed.ncbi.nlm.nih.gov]

  Case Report A 69 year old male presented with a five year history of unsteadiness, progressive hearing impairment and bilateral lower limbs tingling. Neurological examination revealed bilateral sensory deafness and gait ataxia. [imj.ie]

• Drop Attacks

  A 58-year-old female patient experienced 9 months of continuous progressive dizziness, difficulty with mobilization, drop attacks, and lack of hearing. The patient also had an extensive history of thoracic spinal surgeries. [ncbi.nlm.nih.gov]

Treatment and prognosis Unfortunately, no proven direct treatment exists for established siderosis, and workup is focused on identifying the causative lesion, although often even this is not possible. [radiopaedia.org]

[…] episodic encephalopathy due to a spinal dAVF has also previously been reported as a cause of symptomatic SS.[ 6 ] The two additional cases reported in the present case as well as the previous case reports highlight the need for a through cerebrovascular workup [surgicalneurologyint.com]

In two patients surgical treatment of ependymoma or cerebral cavernomas were the underlying diseases. No cause was detected in one patient. [ncbi.nlm.nih.gov]

Our report underlies the importance of early diagnosis and surgical management, with imaging examination of the full neuroaxis to identify the source of bleeding, to halt disease progression and improve prognosis. [ncbi.nlm.nih.gov]

Treatment and prognosis Unfortunately, no proven direct treatment exists for established siderosis, and workup is focused on identifying the causative lesion, although often even this is not possible. [radiopaedia.org]

Incidence, 30 days case-fatality rate and prognosis of stroke in Iquique, Chile: results of a two years community based prospective study (PISCIS Proyect). [scielo.cl]

Additional intracerebral bleeds are evidenced on susceptibility-weighted images in all three cases, which provide possible clues to the underlining etiology of superficial siderosis. [ncbi.nlm.nih.gov]

The etiologies and the pathogenesis are discussed. Read this article (multiple options) Comments Related Papers [readbyqxmd.com]

Chorea-Acanthocytosis (Neuroacanthocytosis; CHAC) ● Vacuolar protein sorting 13, yeast, homolog of, A (VPS13A;Chorein) ; Chromosome 9q21.2; Recessive Epidemiology: Especially common in Japan Genetics: Mutations Japanese: Frameshift deletion Other: Missense [neuromuscular.wustl.edu]

Stroke epidemiology: a review of population-based studies of incidence, prevalence, and case-fatality in the late 20th century. Lancet Neurol 2003; 2 (1): 43-53. [ Links ] 3. Tapia J, Corbalán R, Huete I, Valdés F, Villanueva C, Córdoba J, et al. [scielo.cl]

Deposition of free iron and hemosiderin in pial and subpial structures leads to intoxication of the central nervous system and represents the pathophysiological mechanism of superficial siderosis. [ncbi.nlm.nih.gov]

Sporadic cerebral amyloid angiopathy revisited: recents insights into pathophysiology and clinical spectrum. J Neurol Neurosurg Psychiatry 2012; 83: 124-37. [ Links ] 9. Scholz W. [scielo.cl]

The pathophysiology of the prominent vessels without vascular malformations seen during the work-up of SS is unknown. [ajnr.org]

This case is interesting insofar as the surgical treatment prevented further bleeding and possibly progression of the disease. [ncbi.nlm.nih.gov]

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

[…] sensitivity, are suited for detecting haemosiderin encrusting the leptomeninges and surface of brain cortex. 1– 4 Identification of the source of chronic bleeding into the subarachnoid spaces, which is obtained in about half of cases, may lead to removal preventing [jnnp.bmj.com]

Superficial siderosis is a progressive, neurological condition that must be recognized early in order to prevent further progression of neurologic symptoms. [pfmjournal.org]