Review Article

Revisiting the Molecular Mechanism of Neurological Manifestations in Antiphospholipid Syndrome: Beyond Vascular Damage

Table 1

Neurological manifestations of APS.

Neurological manifestations associated with antiphospholipid syndromeFeatures

Thrombotic manifestations
 Spinal cord strokeUncommon feature; less frequent than transverse myelitis
 Acute ischemic encephalopathyUncommon feature in APS secondary to SLE; presents with tetraparesis, confusion, and hyperreflexia
 Ischemic stroke
 Transient ischemic attack (TIA)
The most common manifestations of APS; important cause of juvenile stroke; any brain region can be interested
 Cerebral venous thrombosisUncommon vascular manifestation

Nonthrombotic manifestations
 HeadacheFrequent and often untreatable; no definite association between aPL positivity and type of headache [34]
 Multiple sclerosisAPS can mimic multiple sclerosis; no definite tests to differentiate these entities are available
 Transverse myelitisRare acute inflammatory manifestation; more common in APS secondary to SLE
 Sensorineural hearing lossAcute onset in the presence of aPL may be a manifestation of APS [37]
 Guillain-Barrè syndromeAntiphospholipid antibodies probably produced as a result of myelin damage
 Peripheral neuropathyMononeuritis multiplex due to vasculitis is most commonly found in SLE; axonal neuropathy can be asymptomatic in PAPS
 Cognitive dysfunction and dementiaCaused by multiple brain strokes; Alzheimer's disease-like dementia in 6% of cases
 Idiopathic intracranial hypertensionCan be the presenting feature of APS
 EpilepsyIn 10% of patients; primary or secondary to stroke
 Chorea and other movement disordersRarely due to stroke in the basal ganglia; frequent in patients with APS secondary to SLE