Title:‘Non-Criteria’ Neurologic Manifestations of Antiphospholipid Syndrome: A Hidden Kingdom to be Discovered
Volume: 15
Issue: 10
Author(s): Md. Asiful Islam, Fahmida Alam, Mohammad Amjad Kamal, Kah Keng Wong, Teguh Haryo Sasongko and Siew Hua Gan
Affiliation:
Keywords:
Antiphospholipid antibodies, antiphospholipid syndrome, central nervous system, neurological manifestations,
pathophysiology, treatment.
Abstract: Neurological manifestations or disorders associated with the central
nervous system are among the most common and important clinical characteristics
of antiphospholipid syndrome (APS). Although in the most recently updated (2006)
APS classification criteria, the neurological manifestations encompass only transient
ischemic attack and stroke, diverse ‘non-criteria’ neurological disorders or
manifestations (i.e., headache, migraine, bipolar disorder, transverse myelitis,
dementia, chorea, epileptic seizures, multiple sclerosis, psychosis, cognitive
impairment, Tourette’s syndrome, parkinsonism, dystonia, transient global amnesia,
obsessive compulsive disorder and leukoencephalopathy) have been observed in
APS patients. To date, the underlying mechanisms responsible for these abnormal
neurological manifestations in APS remain unclear. In vivo experiments and human observational
studies indicate the involvement of thrombotic events and/or high titers of antiphospholipid antibodies
in the neuro-pathogenic cascade of APS. Although different types of neurologic manifestations in APS
patients have successfully been treated with therapies involving anti-thrombotic regimens (i.e.,
anticoagulants and/or platelet antiaggregants), antineuralgic drugs (i.e., antidepressants, antipsychotics
and antiepileptics) and immunosuppressive drugs alone or in combination, evidence-based guidelines
for the management of the neurologic manifestations of APS remain unavailable. Therefore, further
experimental, clinical and retrospective studies with larger patient cohorts are warranted to elucidate the
pathogenic linkage between APS and the central nervous system in addition to randomized controlled
trials to facilitate the discovery of appropriate medications for the ‘non-criteria’ neurologic
manifestations of APS.
