Abstracts

Rationale: Seizures are rare as a presenting symptom in CJD patients; however, seizures happen in about 15-20% of patients during the disease process. Unfortunately, evidence is lacking on the best treatment, and most reports showed inefficacy.


Methods: We are reporting 3 cases of CJD that had seizures or status epilepticus during the CJD disease process. EEGs were reviewed for each patient to characterize the seizures, and the ASMs medications used, and their clinical and EEG efficacy were reported. ASMs used in our patients were Levetiracetam, Valproate, Phenytoin, lacosamide, and lorazepam. The literature review of CJD cases with seizures as a presenting symptom or happened during the disease course, included reports of ASMs used and comments on their efficacy. Additional ASMs in the literature were Phenobarbital, Topiramate, Clonazepam, and Propofol.


Results: The ASMs showed a temporary effect on EEG if any. The epileptiform discharges reappeared with the same frequency within 24 hours of starting the ASM (after a loading dose) of one of the patients. Another patient that had partial status epilepticus early in the course of the disease had a better response to ASM at the beginning that weaned off with the disease progression, and notably, lorazepam showed the most significant effect of stopping the partial seizures temporarily. The third patient did not show any response to ASMs unfortunately. The literature review showed unsuccessful seizure treatment with many ASMs, which included deep sedation, with a reoccurrence of the epileptiform discharges on reducing the sedation, with no clear clinical improvement. Interestingly, one report stated some benefits of Clonazepam for the first two weeks.


Conclusions: Seizures treatment in CJD patients is challenging, with a lack of response on many ASMs. Benzodiazepines showed some efficacy on EEG, with limited clinical benefit. Considering ASMs such as Clonazepam, Lorazepam, and probably try Clobazam as possible options.


Funding: none