Creutzfeldt -Jacob disease is a rare form of dementia associated with neuropathological finding of accumulation of prion proteins in the brain with rapid deterioration and disability [1]. Alteration in the level of consciousness has been studied in dementia and other hypoxic brain injuries. Attempt to modulate the effect of pharmacological agents and deep brain stimulation to attain the maximum level of consciousness is ongoing but the evidence is lacking [2]. This report supports the use of zolpidem in the arousal from altered consciousness in a patient with sporadic CJD.
Case ReportA 59 year old woman presented with 7 months history of gradual onset visual disturbances which progressed to near complete visual loss (diagnosed as cortical blindness). She also developed behavioural changes characterized by reduced spontaneity, decreased social interaction, and progressive reduction in speech output, along with worsening memory for recent events and gait ataxia. Two weeks prior to admission, she became progressively mute with poor comprehension, and also developed double incontinence, generalised myoclonic jerks, was bed -bound and was unable to take foods orally. There was no history of seizures and no past history of tissue or organ transplantation or eating bovine brain. There was no family history of dementia or neurological illnesses except Parkinsonism in patient's elder brother.On examination she was awake and was opening eyes spontaneously with no verbal output. She was not obeying simple commands but would localize pain. She had severe rigidity of all limbs (lower limbs slightly more than upper limbs), intermittent myoclonic jerks which were not stimulus sensitive, and brisk deep tendon reflexes with bilaterally extensor plantar reflex. Grasp reflexes were present bilaterally.Investigations including CBC, metabolic panel, thyroid profile and serum B12 levels were normal. TPO antibody, autoimmune encephalitis workup for antibodies against NMDA, AMPA 1 and 2, VGKC (LGI-1 and CASPR), GABA 1 and 2, and autoimmune panel for vasculitis were negative. MRI Brain (Figure 1) showed cortical ribbon sign with bilateral involvement of caudate and putamen, which was highly suggestive of prion disease like CJD. EEG showed frequent bilateral periodic triphasic discharges with diffuse slowing of background activity. Patient met diagnostic criteria for probable CJD by World Health Organization (Figure 2).Patient had not responded earlier to trials of L-dopa, donepezil, memantine, amantadine and a course of methyl prednisolone. Care givers reported earlier noticing a marked improvement in her responsiveness when she was given tablet zolpidem for insomnia for two nights. Based on this history, a trial of immediate release tablet zolpidem 5 mg HS was started. Response was noticed 7 hours after the first dose in the form of patient spontaneously getting up from the bed, attempting to walk with assistance and taking food orally by self-removal of nasogastric tube. She also started initiating verbal communication with care give...