David’s Story – A case of Idiopathic Limbic Encephalitis or Antibody-negative Limbic Encephalitis
Posted on September 08, 2018
Sometimes all the signs and symptoms point to an autoimmune encephalitis(AE), but the all-important antibody is not found in serum or cerebral spinal fluid. This story illustrates well such a scenario and highlights the difficulties of making a diagnosis and initiating the appropriate treatments for a probable case of AE.
We are grateful to David for sharing his story with us and wish him continued healing.
In their paper, Graus, et. al., A Clinical Approach to Diagnosis of Autoimmune Encephalitis, have listed the criteria for probable autoimmune encephalitis in the 1st panel of their position paper, reproduced below:
Panel 1: Diagnostic criteria for possible autoimmune encephalitis
Diagnosis can be made when all three of the following criteria have been met:
1. Subacute onset (rapid progression of less than 3 months) of working memory deficits (short-term memory loss), altered mental status*, or psychiatric symptoms
2. At least one of the following:
- New focal CNS findings
- Seizures not explained by a previously known seizure disorder
- CSF pleocytosis (white blood cell count of more than five cells per mm3)
- MRI features suggestive of encephalitis
3. Reasonable exclusion of alternative causes (appendix)
*Altered mental status defined as decreased or altered level of consciousness, lethargy, or personality change. Brain MRI hyperintense signal on T2-weighted fluid-attenuated inversion recovery sequences highly restricted to one or both medial temporal lobes (limbic encephalitis), or in multifocal areas involving grey matter, white matter, or both compatible with demyelination or inflammation.
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