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Differential Diagnosis

Differential diagnosis and mimics of Angiography-Positive CNS Vasculitis in Children

  •  Noninflammatory CNS vasculopathies:
    • Dissection
    • Thrombembolic disease
    • Hemoglobin disorders
    • Antiphospholipid antibody (aPL) syndrome
    • Fibromuscular dyplasia
    • Genetic collagen vascular disorders (Marfan Syndrome, Ehlers-Danlos Syndrome, others)
    • Moyamoya disease (idiopathic)
  • Conditions associated with cerebral vasospasms:
    • Channelopathies including familiar hemiplegic migraine and calcium channelopathy
    • Reversible vasoconstrictive syndrome (RVS)
    • 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
  • Other syndromes with associated cerebral vasculopathy:
    • Cogan syndrome (vasculopathy plus inflammatory eye disease [interstitial keratitis] and vestibularauditory dysfunction)
    • Susac syndrome (noninflammatory vasculopathy resulting in retinopathy, hearing loss and encephalopathy)

Differential diagnosis and mimics of Angiography-Negative CNS Vasculitis in Children 

  • 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 encephalitis, post-mycoplasma encephalitis, celiac-disease-associated encephalitis, Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections or PANDAS)
    • T-cell mediated inflammatory brain diseases (Rasmussen's encephalitis)
    • Granulomatous inflammatory brain diseases (Neurosarcoidosis)
  • Infections
    • Tuberculosis
    • JC virus
  • 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.