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Differential Diagnosis

Differential diagnosis and mimics of Angiography-Positive CNS Vasculitis 

Noninflammatory CNS vasculopathies:

  • Dissection
  • Thrombembolic disease
  • Intracranial atherosclerosis.
  • Hemoglobin disorders
  • Antiphospholipid antibody (aPL) syndrome
  • Fibromuscular dyplasia
  • Genetic collagen vascular disorders(Marfan Syndrome, Ehlers-Danlos Syndrome, others)
  • Moyamoya disease

Conditions associated with cerebral vasospasms:

  • Channelopathies including familiar hemiplegic migraine and calcium channelopathy
  • Reversible cerebral vasoconstrictive syndrome (RCVS)
  • Idiopathic vasospasms

Genetic syndromes with associated vasculopathy:

  • Neurofibromatosis type 1 (NF1)
  • Down’s syndrome
  • Posterior fossa malformations–hemangiomas–arterial anomalies–cardiac defects–eye abnormalities–sternal cleft and supraumbilical raphe syndrome (PHACES)
  • Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
  • Fabry disease
  • Homocysteinuria

Atherosclerosis (Adults)

Other syndromes with associated cerebral vasculopathy:

  • Cogan syndrome (vasculopathy plus inflammatory eye disease [interstitial keratitis] and vestibular auditory dysfunction)
  • Susac syndrome (noninflammatory vasculopathy resulting in retinopathy, hearing loss and encephalopathy)



Differential diagnosis of Angiography-Negative CNS Vasculitis 

Nonvasculitic inflammatory brain diseases:

  • Demyelinating diseases (acute demyelinating encephalomyelitis, multiple sclerosis, idiopathic or demyelinating optic neuritis)
  • Antibody-mediated inflammatory brain diseases (anti-NMDAR encephalitis, antibody-mediated limbic encephalitis, neuromyelitis optica, Hashimoto’s encephalitis, post-mycoplasma encephalitis, celiac-disease-associated encephalitis, Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections or PANDAS)
  • Granulomatous inflammatory brain diseases (Neurosarcoidosis)


  • Syphillis
  • Tuberculosis
  • JC Virus
  • Other bacterial, viral or parasitic

Metabolic diseases with associated inflammatory or ischemic brain lesions:

  • Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)
  • Rolandic mitochondrial encephalomyopathy (ROME)
  • Polymerase gamma deficiency (POL-γ)
  • Malignancies
  • Angiocentric lymphoma









The above table is modified from M. Twilt ., The spectrum of CNS vasculitis in children and adults. Nat Rev Rheumatol. 2011 Dec 20;8(2):97-107.