Burst- suppression in EEG is seen in? (LQ)
|A||Subacute sclerosing panencephalitis|
|B||Creutzfeldt Jacob disease|
|D||All of the above|
Subacute sclerosing panencephalitis
Prion (PrPSC) is an altered form of normal protein (PrPC) that can transform other normal protein into prion protein hence infectivity.
Prion Diseases are (Ref. Hari-18th ed., pg- 3441)
1). Creutzfeldt- Jakob disease
2). Gerstmann Sträussler-Scheinker syndrome
4). Fatal familial insomnia
Clinical feature of Creutzfeldt- Jakob disease. (AIPG 2008)
1). The unique feature of this disease is that it can be transmitted as infectious, congenital or degenerative
2). 10 – 12 % cases are familial.
3). Progressive, degenerative illness is caused by prions (i.e., infectious proteinaceous particles) and is associated with a spongiform encephalopathy (Tiny cavities in the brain). (AIIMS May 2012)
4). The gene for the prion protein is on chromosome 20; approximately 10% of cases are hereditary.
Illness can develop because of infection or somatic and germ cell mutations. The CSF 14-3-3 protein is a marker for Creutzfeldt - Jakob disease.
5). It can be transmitted by corneal transplant also.
6). Patients has severe dementia with cerebellar ataxia and diffuse myoclonic jerks.
7). Cortical blindness can occurs
8). The EEG often demonstrates periodic discharges and an abnormal background rhythm.
9). Death usually occurs within several months of the onset of the disease.
Extra Edge: EEG characteristic in diseases other than epilepsy:
1). Subacute sclerosing panencephalitis (SSPE)
Characteristic periodic pattern with bursts every 3 to 8 seconds of high voltage, sharp slow waves followed by periods of attenuated (falt) background) (Burst Suppression)
2). Creutzfeldt Jacob disease (CJD)
(Repetitive high voltage, triphasic and polyphasic sharp discharges are seen in most advanced cases)
3). Hepatic encephalopathy
Characteristic Symmetric high voltage triphasic slow wave in frontal region.
Periodic lateralizing epileptiform discharges (PLEDs)