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, Dysarthria , Difficult Speech, Nystagmus, Gait abnormalities
- Brain-stem: Dysphagia
- Pyramidal signs / UMN:
- Myoclonic Jerking, Myoclonus
- Hyperreflexia
- Medulla oblangata esp dorsal motor nucleus of vagus and other medullary nuclei [2]
- Psychosis: Behavioral change
- Cortical Blindness: Visual Agnosia, Visual field deficits, Blindness
- Involuntary movement / Frontal Lobe Seizure (Focal / Partial seizure)
Paresthesia
Muscular Atrophy
Fasciculation
Muscular Spasm
Related disease
- Dementia: Memory weakness, Disorientation
- Parkinson disease
- 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]
Prosopagnosia
Presenting clinical symptoms included paranoid thoughts and agitation, rapidly progressive memory decline, prosopagnosia, and late development of myoclonus and mutism
Ref: Carrasco AE, Appleby BS, Cali I, Okhravi HR. Atypical Case of VV1 Creutzfeldt-Jakob Disease Subtype: Case Report. Front Neurol. 2022 May 9;13:875370. doi: 10.3389/fneur.2022.875370. PMID: 35614914; PMCID: PMC9124891.
- Approximately 10–18% of patients with sCJD report visual symptoms include blurred vision, nystagmus, diplopia, metamorphopsias, dyschromatopsia, supranuclear palsies, visuospatial disturbances, saccades, visual hallucinations, and anopsia (Cooper et al., 2005; Kropp et al., 1999; Lenk et al., 2018; Lueck et al., 2000; Ntantos et al., 2018; Proulx et al., 2008; Rabinovici et al., 2006; Will and Matthews, 1984; Wong et al., 2015). In the Heidenhain variant of sCJD, the visual symptoms are thought to arise from neurodegeneration of the visual occipital cortex (Baiardi et al., 2016). In other forms of sCJD, the neuroanatomic defects that underlie visual symptoms are less clear.
- Ref: Exp Eye Res. 2022 September ; 222: 109172. doi:10.1016/j.exer.2022.109172, Minimal change prion retinopathy: Morphometric comparison of retinal and brain prion deposits in Creutzfeldt-Jakob disease Vanessa S. Goodwilla
Sleep pathologies
- Hypersomnia
- Insomnia
- Restless legs symptoms and/or periodic limb movements of sleep
- Parasomnias
- Polysomnography also shows
- Poorly formed or absent sleep spindles and/or K-complexes
- Sleep-disordered breathing
- REM sleep without atonia
- REM sleep behavior disorder
Ref: Kang P, de Bruin GS, Wang LH, Ward BA, Ances BM, Lim MM, Bucelli RC. Sleep Pathology in Creutzfeldt-Jakob Disease. J Clin Sleep Med. 2016 Jul 15;12(7):1033-9. doi: 10.5664/jcsm.5944. PMID: 27250807; PMCID: PMC4918986.
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.