8E42

ICD-11 MMS 2026-01

Superficial siderosis of the nervous system

Description

Superficial siderosis is the deposition of haemosiderin in the central nervous system as a result of chronic or recurrent subarachnoid haemorrhage due to vascular anomalies, aneurysms, vascular tumours, neurosurgery, cervical root lesions, head injury and trauma. Clinical feature of Superficial siderosis include sensorineural deafness, cerebellar ataxia, pyramidal weakness and less frequently dementia, loss of sphincter control, anosmia, anisocoria, sensory disturbance, extra-ocular motor palsies, sciatica and lower motor neuron signs. The diagnosis may be confirmed by pure tone audiometry, neuroimaging, spinal fluid analysis, angiography to identify a potential bleeding source and where appropriate genetic testing.

Index terms

  • Superficial siderosis of the nervous system
  • Superficial siderosis due to vascular anomalies
  • Superficial siderosis due to an aneurysm
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  • Superficial siderosis due to an arteriovenous malformation
  • Superficial siderosis due to a cavernoma
  • Superficial siderosis due to other vascular anomalies
  • Superficial siderosis due to central nervous system tumours
  • Superficial siderosis due to nerve root avulsion
  • Superficial siderosis due to previous neurosurgery
  • Superficial siderosis due to head injury or trauma
  • Superficial siderosis due to cerebral amyloid angiopathy
  • Superficial siderosis due to other causes
  • Superficial siderosis of unknown aetiology

Exclusions from above levels

  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 19 – Certain conditions originating in the perinatal period
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  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • 8E40 – Disorders of the meninges excluding infection
  • 8E41 – Pachymeningitis
  • 8E43 – Pain disorders
  • 8E44 – Post anoxic brain damage
  • 8E45 – Locked-in syndrome
  • 8E46 – Reye syndrome
  • 8E47 – Encephalopathy, not elsewhere classified
  • 8E48 – Encephalitis, not elsewhere classified
  • 8E49 – Postviral fatigue syndrome
  • 8E4A – Paraneoplastic or autoimmune disorders of the nervous system

Foundation entity

Superficial siderosis of the nervous system – http://id.who.int/icd/entity/931843870

Part of: 08: Diseases of the nervous system