Neurology Resident PGY3 Marshall University Joan C. Edwards School of Medicine
Rationale: Sporadic Creutzfeldt-Jakob disease (sCJD) is a progressive, fatal neurodegenerative disease characterized by rapidly progressive dementia, cerebellar ataxia, diffuse myoclonus, and a variety of visual and other neurological abnormalities. Electroencephalography (EEG) is one of the clinical criteria, mainly because of sharp bi- or tri-phasic complex lasting from 100 to 500 milliseconds (ms) and repeated at intervals of 500–2000 ms, known as "periodic sharp wave complexes" (PSWCs) reported in about two-thirds of patients with sCJD. Methods: Case 1: 60-year-old male presented with ataxia and bizarre behavior and speech difficulty for three months. The patient had a staring episode and extensor posturing/stiffening of the left upper extremity. Initial magnetic resonance imaging (MRI) brain showed no acute intracranial abnormality. Repeat MRI brain two weeks after admission was notable for cortical ribboning and hockey stick sign suggestive of Creutzfeldt–Jakob disease. The cerebrospinal fluid (CSF) autoimmune and paraneoplastic encephalitis panels were negative. CSF Protein 14-3-3 testing returned positive confirming CJD.Case 2: A 75-year-old male presented to the hospital for evaluation of altered mental status. He was found unconscious on the floor for an unknown duration with a history of behavioral changes and incoherent speech for almost two weeks. The patient’s thought process was tangential, easily distractible with expressive aphasia. MRI brain showed cortical ribboning and hyperintensity of basal ganglia on diffusion weighted imaging (DWI) with minimal fluid attenuation inversion recovery (FLAIR) hyperintensity, concerning CJD. CSF 14-3-3 is positive and paraneoplastic panel was negative.
Case 3: 66-year-old female who was admitted for worsening of gait ataxia, urinary incontinence, cognitive dysfunction, and tremor for 11 months. MRI brain done seven months ago showed no acute abnormality. The patient lost 20 pounds in the three months from decreased appetite and not being as active as before. Repeat MRI brain showed restricted diffusion of bilateral basal ganglia and cortical ribboning. CSF labs, including paraneoplastic and autoimmune panel, were negative, but 14-3-3 protein for CJD was positive.
Results: Case 1: Video EEG showed continuous periodic sharp wave complexes, suggestive of generalized periodic epileptiform discharges (GPED's) with a triphasic morphology at times (Figure 1). We started the patient with the whole five days of Intravenous immunoglobulin (IVIG), levetiracetam 1 gram (g) every eight hours (q8h), valproate 1g q8h and clobazam 10mg twice daily. Due to poor prognosis, the family opted palliative care measures.
Case 2: EEG showed generalized bursts of low voltage theta with occasional low voltage triphasic waves and bifrontal (left-lateralized) sharp waves at 1-2 hertz (Hz). Most of EEG recording shows widespread very low voltage electrical activity suggestive of encephalopathy (Figure 2). The patient was discharged to the long-term acute care facility.
Case 3: EEG showed no interictal epileptiform abnormalities or seizures. No focal slowing or generalized periodic discharges were seen. The patient was started on Olanzapine 5 mg daily at bedtime for agitation. The patient's family decided to discharge him to hospice due to poor prognosis. Conclusions: The variations of EEG are evident in all three patients mentioned in this case series ranging from GPED’s with a triphasic morphology, low voltage bifrontal sharp waves to almost no changes at all. The MRI brain changes with cortical ribboning and 14-3-3 protein for CJD was positive in all of the patients of this case series. Funding: Please list any funding that was received in support of this abstract.: None