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]

• Angiomatosis

  Rushworth : Treatment of encephalotrigeminal angiomatosis (Sturge-Weber disease) by hemispherectomy. Arch. Dis. Child. 35, 433–447 (1960). Google Scholar Foncin, J. F., J. Gaches, L. Saimot et E. [link.springer.com]

• Blurred Vision

  A 69-year-old man presented with headache, unsteady gait, blurred vision, and progressive hearing loss. [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]

  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]

  Subjective hearing impairment after subarachnoid haemorrhage: Prevalence and risk factors. Journal of the Neurological Sciences, Vol. 372, Issue., p. 184. CrossRef Google Scholar Fukui, Melanie B. and Meltzer, Carolyn Cidis 2009. [cambridge.org]

• Progressive Hearing Loss

  A 69-year-old man presented with headache, unsteady gait, blurred vision, and progressive hearing loss. [ncbi.nlm.nih.gov]

  "Superficial siderosis syndrome with progressive hearing loss and bilateral vestibular failure, 51 years after a neurosurgical procedure: diagnostic value of combined MRI and video head impulse test." J Neurol 264(2): 391-393. [dizziness-and-balance.com]

  He complained of a progressive hearing loss bilaterally as well as of recurrent episodes of positional vertigo and peripheral facial nerve palsy which occurred during several days for 10 years. [ispub.com]

• Suggestibility

  Magnetic resonance imaging suggested the diagnosis of superficial siderosis of the central nervous system, without an evident bleeding source. [ncbi.nlm.nih.gov]

• Gait Ataxia

  The most common neurologic manifestations include progressive gait ataxia, sensorineural hearing loss, and corticospinal tract signs. [ncbi.nlm.nih.gov]

• 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 focussed 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 focussed on identifying the causative lesion, although often even this is not possible. [radiopaedia.org]

In this case report, the presentation, diagnosis, pathogenesis, treatment and prognosis of SS are reviewed. Case report A 58-year-old man suffered from recurrent peripheral facial nerve palsy and bilateral hearing loss. [ispub.com]

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]

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]

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]