CJD, Creutzfeldt Jakob Disease
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Clinic
- It is the most important Prion miasm.
- A rare neurodegenerative disease that is clinically characterized by
- Rapidly progressive dementia
- Visual disturbance
- Movement disorders (myoclonus) (90%)
- Cerebellar ataxia (80%) [1]
- Nonspecific / Prodromal syndrome of fatigue Sleeping/ Eating disorders (33%)
- Cognitive symptoms such as Memory loss / Confusion or Behavioral changes / Personality changes / Hallucinations (33%)
- Focal neurological signs (33%) such as
- Ataxia
- Dysarthria
- Visual loss
- Hemiparesis
- Almost all patients enter a phase of Akinetic mutism before death.
- Although chorea is commonly observed in variant CJD (vCJD= NVCJD), it is considered a rare occurrence in sporadic CJD (sCJD).
Entities
- Cerebellar Ataxia: Incoordination
- Brain-stem, Medulla oblangata: Dysarthria , Difficult Speech, Nystagmus
- Medulla oblangata esp dorsal motor nucleus of vagus and other medullary nuclei [2]
- Psychosis: Behavioral change
- Cortical Blindness: Visual Agnosia, Visual field deficits, Blindness
- Pyramidal signs / UMN:
- Jerking: Myoclonic Jerking, Myoclonus
- Hyperreflexia
- Extrapyramidal [3]
- Dystonia: Rigidity
- Parkinsonism (Bradykinesia, Rigidity, Tremor)
- Involuntary movement / Frontal Lobe Seizure (Focal / Partial seizure)
- Tremor
- Myoclonus / Myoclonic Jerking
- Seizure
- Paresthesia
- Agnosia esp visual agnosia
Muscular Atrophy
Fasciculation
Muscular Spasm
Related disease
- Dementia: Memory weakness, Disorientation
Sleep pathology
Sleep pathology is common including
- Restless legs symptoms
- Sleep-disordered breathing
- Parasomnias (REM sleep without atonia, REM sleep behavior disorder, Periodic limb movements)
Dysphagia
As the disease process advances, destruction of the cerebral architecture in sensory, motor, and associated areas can lead to oropharyngeal dysphagia. [4]
Remedies
- ↑ Tan AH, Toh TH, Low SC, Fong SL, Chong KK, Lee KW, Goh KJ, Lim SY. Chorea in Sporadic Creutzfeldt-Jakob Disease. J Mov Disord. 2018 Sep;11(3):149-151. doi: 10.14802/jmd.18017. Epub 2018 Aug 9. PMID: 30086616; PMCID: PMC6182307.
- ↑ Iacono D, Ferrari S, Gelati M, Zanusso G, Mariotto S, Monaco S. Sporadic Creutzfeldt-Jakob Disease: Prion Pathology in Medulla Oblongata-Possible Routes of Infection and Host Susceptibility. Biomed Res Int. 2015;2015:396791. doi: 10.1155/2015/396791. Epub 2015 Sep 17. PMID: 26457299; PMCID: PMC4589575.
- ↑ Misdiagnosis of an Atypical Creutzfeldt-Jakob Disease: Analysis of One Case in China and Review of the Literature, Mengqi Zhanga1 , Zigao Wangb2 , Lijie Duana1 and Hengbing Zua1 * 1 Department of Neurology, Jinshan Hospital, Fudan University, China
- ↑ Salhab, Joseph DO1; Sharma, Saras DO2; Mohamadameen, Ranje DO2; Valladares, Michael DO2; Sobrado, Javier MD2; Mohan, Karthik MD3. Management of Dysphagia in a Patient with Creutzfeldt-Jakob Disease: 1230. American Journal of Gastroenterology 110():p S542, October 2015.