Refractory Partial Status Epilepticus in the Setting of Anti-GAD65 Autoimmune Encephalitis in an 18-Year-Old Female with an MRI Contraindication: A Case Report
Lilla Simon1, Yakov Isakov1, Monika Mikolajczak1, Tracey Nicole Webb1, Phillip Regina1, Daniel Kaufman1, Anusha Sanivarapu1, Amber Mitchell1
1Neurology, Northwell/Nuvance Health
Objective:
To present a rare case of refractory partial status epilepticus due to anti-GAD65 autoimmune encephalitis in an 18-year-old female with an MRI contraindication, highlighting diagnostic and management challenges.
Background:
Anti-GAD65 autoimmune encephalitis is an uncommon condition, especially in teenagers, and typically manifests as a drug-resistant epilepsy. This case is unique due to the patient's MRI contraindication, a factor not previously reported in anti-GAD65 encephalitis cases.
Design/Methods:
NA
Results:

An 18-year-old female with a history of mitral clip repair presented with new-onset seizures including right arm clonic movements, then progressing to generalized tonic activity. The patient had stopped Keppra due to side effects. She was then started on zonisamide. MRI was contraindicated due to her mitral clip. Continuous EEG showed left intermittent seizures. She demonstrated left facial twitching. Despite increasing zonisamide, EEG showed left-sided PLEDs and right-sided non-convulsive seizures. This raised concern for partial status epilepticus (possibly due to HSV or autoimmune encephalitis). Acyclovir and fosphenytoin were initially started, with EEG continuing to show seizures. Lumbar puncture was then performed and demonstrated positive GAD65 antibodies.

 

The patient was diagnosed with partial status epilepticus secondary to anti-GAD65 autoimmune encephalitis. She was transferred to the ICU, intubated, and started on a versed drip. She was treated with multiple anti-epileptic drugs, IVIG, and later rituximab. Seven sessions of plasma exchange (PLEX) were then completed. Patient started to show improvement after rituximab, and especially after PLEX. After extubation, she quickly returned to baseline and her medications were tapered. She was discharged on fycompa, vimpat, and a prednisone taper with outpatient follow-up.

Conclusions:
This case highlights the importance of early diagnosis of anti-GAD65 autoimmune encephalitis in young patients with new-onset seizures, even without MRI. Prompt treatment, including PLEX, can prevent progression to status epilepticus and support recovery.
10.1212/WNL.0000000000215521
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