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]

• Asymptomatic

  Because of the good availability of magnetic resonance imaging, asymptomatic cases of superficial siderosis of the central nervous system are increasingly discovered. SS cases are increasingly reported in the literature. We present three new cases. [ncbi.nlm.nih.gov]

  Our patient’s myxopapillary ependymomas were otherwise asymptomatic, as these tumours often are, so that they are often diagnosed late. [jnnp.bmj.com]

  At present, it is generally accepted that SS-CNS is due to small, chronic, repeated and often asymptomatic subarachnoid hemorrhages (SAHs) with various causes and that it typically occurs years after the initial bleeding. [journals.plos.org]

• Pharyngitis

  Review of her medical records showed that she received an eight-week course of 60 Co-γ radiotherapy for pharyngeal carcinoma, which was administered at 700 Gy/week through the pre-auricular field and ancillary to the bilateral infraorbital field. [smj.org.sg]

• Aspiration

  In the following days the patient's neurological condition deteriorated and he died after an episode of pulmonary aspiration. [elsevier.es]

• Macula

  Gomori stain revealed iron deposits within the spiral ligament, stria vascularis and in the subepithelial mesenchymal tissue of the maculae of the vestibular system. [ncbi.nlm.nih.gov]

• Blurred Vision

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

• Visual Impairment

  An elderly patient with SSNS presenting with ataxia, depression and severe visual impairment was admitted to the Unit of Geriatrics of the University Hospital of Perugia, Italy. [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]

  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]

  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]

• Dysuria

  MRI-enhanced gradient echo T2 star-weighted angiography. ( 25647424 ) Zhao H....Gong X. 2015 19 Superficial siderosis of the central nervous system. ( 26240112 ) Sampaio L....Pinto M. 2015 20 Superficial siderosis of the central nervous system presenting with dysuria [malacards.org]

• Neurologic Manifestation

  The superficial siderosis (SS) of the central nervous system (CNS) is a rare condition characterized by a wide range of neurological manifestations directly linked to an acquired iron-mediated neurodegeneration. [ncbi.nlm.nih.gov]

  The most common neurologic manifestations include progressive gait ataxia, sensorineural hearing loss, and corticospinal tract signs. [plu.mx]

  Gordon RM, Silverstein A (1970) Neurologic manifestations in progressive systemic sclerosis. Arch Neurol 22:126–134 PubMed Google Scholar 11. [doi.org]

  The deafness and gait disturbance, which were his chief complaints, were followed by other neurological manifestations including pyramidal tract signs, anosmia, and ageusia. [scienceopen.com]

• 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]

• Peripheral Neuropathy

  Peripheral neuropathy isseen in 1020% of the patients. Ischemic infarction ofdiVerent nerves may result in a painful mononeuropathymultiplex. Unilateral or bilateral optic neuropathy has alsobeen seen in SSc [12]. [docslide.com.br]

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]

• Cerebrospinal Fluid Abnormality

  It is associated with cerebrospinal fluid abnormalities consistent with recurrent bleeding into the subarachnoid space. The usual symptoms are hearing loss, ataxia, spastic paraparesis, sensory and sphincter deficits, and mental deterioration. [ncbi.nlm.nih.gov]

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]

It shows how worrying a delayed diagnosis of secondary SS may be in cases like this one, where a slow growing, bleeding lesion with quite favourable prognosis following complete surgical resection could have been removed earlier, with earlier and more [jnnp.bmj.com]

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 patients with progressive bilateral cochleo-vestibular deficit of unknown etiology, MRI is the examination of choice to confirm SS. [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]

[…] 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]

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

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