Related Subjects:
| Anti-NMDA (NMDAR) Receptor Encephalitis
| Herpes Simplex Encephalitis (HSV)
| Acute Encephalitis
| Limbic Encephalitis
| Paraneoplastic Limbic Encephalitis (Dementia)
| Hashimoto's (Steroid Responsive) Encephalopathy
| Acute Disseminated Encephalomyelitis
| Dementias
π§ About Paraneoplastic Limbic Encephalitis (PLE)
- Definition: PLE is a rapidly progressive dementia associated with malignancies, especially lung (small cell), breast, and testicular cancers.
- Prognosis: β οΈ Often poor, as it usually reflects underlying cancer with aggressive progression.
ποΈ Limbic Structures Involved
- Hippocampus (memory)
- Amygdala (emotion)
- Hypothalamus (autonomic control)
- Insular & cingulate cortex (integration of behaviour & cognition)
β‘ Aetiology
- Autoimmune: Triggered by an anti-tumour immune response that cross-reacts with limbic neurons.
- Associated Malignancies:
- Small cell lung cancer π«
- Breast cancer ποΈ
- Testicular cancer β½
- Autoantibodies: Anti-Hu (lung), Anti-Ta (testis), Anti-Ma (various).
- Occult Cases: Sometimes no cancer is identified despite autoantibodies.
π©Ί Clinical Features
- π§© Neuropsychiatric: Delirium, confusion, memory loss, rapid personality changes.
- π Severe Amnesia: Subacute onset, with profound short-term memory loss.
- β‘ Seizures: Limbic-related, often temporal lobe.
- π§ Extended Involvement: Brainstem or cerebellum β gait ataxia, diplopia.
- π΄ Consciousness: Episodes of drowsiness, unresponsiveness.
- β€οΈ Autonomic Dysfunction: BP instability, arrhythmias.
π¬ Investigations
- π§ CSF: Elevated protein or mild pleocytosis (inflammatory picture).
- π§ MRI: Temporal lobe hyperintensities (T2/FLAIR).
- π EEG: Temporal lobe slowing or epileptiform discharges.
- π§ͺ Antibody Testing:
- Anti-Hu β SCLC
- Anti-Ta β testicular
- Anti-Ma β various
- πΌοΈ Malignancy Search: CXR, CT chest/abdomen, PET; testicular ultrasound, mammography as indicated.
π Management
- π― Treat underlying cancer: Key to improving neurological outcome.
- π Immunosuppression:
- High-dose corticosteroids (first-line).
- IVIg or plasmapheresis in resistant cases.
- Cyclophosphamide or rituximab sometimes considered.
- β‘ Supportive: Antiepileptics for seizures, autonomic stabilisation.
- π Prognosis: Guarded, depends on cancer control and antibody type (onconeuronal antibodies tend to respond poorly).
π References
- Dalmau J, Rosenfeld MR. (2008). Paraneoplastic Syndromes Affecting the Nervous System. Clin Neurol Neurosurg, 110(3), 227-233.
- HΓΆftberger R, Rosenfeld MR. (2015). Antibody-Mediated CNS Disorders. J Neuro-Oncol, 123(1), 87-99.
- Graus F, Dalmau J. (2012). Paraneoplastic Neurological Syndromes. Lancet Neurol, 11(1), 1-9.
- NINDS: Paraneoplastic Limbic Encephalitis