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
Autoimmune cases may respond to immunosuppression. Also see Paraneoplastic limbic encephalitis
About
- A treatable cause of delirium
Types
- Autoimmune: Antibodies to Voltage-gated Potassium channels, NMDA
- Paraneoplastic Limbic Encephalitis : (lung, breast, ovarian, testicular, Hodgkin's lymphoma, thymoma, and immature teratoma of the ovaries
)
Clinical
- Acute delirium. speech difficulties, myoclonic jerks
- Inattention, weakness, seizures, bizarre behaviour
- Psychiatric type syndromes, amnestic deficits
- REM associated sleep defects (VGKC Ab Limbic encephalitis)
Differentials
- Paraneoplastic syndrome
- Encephalitis (infective or autoimmune)
- Lewy-body dementia
- Prion disease
- Lupus cerebritis
- Toxic and metabolic encephalopathies
- Multiple sclerosis
- Hashimoto's encephalopathy
- Wernicke's encephalopathy
- Neurosyphilis,
- Primary vasculitis of the central nervous system
- Leptomeningeal involvement of malignancy
Investigations
- FBC, U&E, LFT, CRP, CXR: hyponatraemia (VGKC Ab Limbic encephalitis)
- CT Brain may be normal
- MRI: such as hyperintense signals on T2-weighted or fluid-attenuated inversion recovery (FLAIR) sequences, atrophic temporal-limbic structures on T1-
weighted images usually of temporal lobes and hippocampus
- EEG: may show slow wave activity or epileptiform activity
- CSF: protein and WCC may be elevated. CSF Protein 14-3-3 negative
- Voltage-gated potassium channel antibodies : high titres >400pmol/L
- CT Chest Abdomen Pelvis for malignancy
- FDG PET scan may show malignancy
- Malignancy associated autoantibodies: Hu, Ma2, Ovarian teratoma (Anti NMDA)
Management
- Autoimmune disease: Treatment is through immunosuppression in autoimmune limbic encephalitis. Usually, Plasma-exchange, steroids and IVIG have all been recommended. A trial of steroids may be considered when infection excluded. The dramatic response may be seen with VGKC ab positive disease
- Paraneoplastic: Management of tumour as possible. Limited responses to immunotherapy.
References