The patient presented with a sudden episode of vertigo which was treated as an isolated symptom. [cureus.com]

Symptoms lasted from few seconds to minutes and presented several times during the day. Neither behavioural changes nor disorientation were present. [clinmedjournals.org]

Discussion Our patient’s clinical presentation fulfilled the criteria for Heidenhain variant of sCJD given the isolated visual disturbance at the onset of disease, cortical ribboning on MRI and his rapid clinical decline.2,4,11 Other visual presentations [dovepress.com]

In this respect, the findings of the present study highlight the fact that, despite their relative rarity, CJD cases presenting with isolated visual symptoms may belong to different disease variants that are characterized by a distinct profile of laboratory [content.iospress.com]

In this paper, we present the case of a middle-aged man diagnosed with CJD Heidenhain Variant (HvCJD). [mdpi.com]

• Episodic Headache

  Despite this temporary improvement, the episodic headaches recurred daily and progressed to include vertigo, vomiting and poor memory capacity. [cureus.com]

• Myoclonus

  blurred vision, disturbed perception of objects 2 Dementia, myoclonus, rigidity, seizures, coma 3 14 F 79 Disturbed perception of colors and objects, visual field restriction 2 Ataxia, myoclonus, dementia, aphasia, coma 4 15 F 70 Disturbed perception [content.iospress.com]

  Sporadic segmental myoclonus in the upper extremities were also noted. Patient died three weeks after admission. Figure 1: MRI findings. [clinmedjournals.org]

  […] usually characterized by rapidly evolving dementia, with cerebellar ataxia, visual disturbances and myoclonus, leading to akinetic mutism. [mdpi.com]

  Other common features include myoclonus, visual hallucinations, cerebellar dysfunction (such as ataxia and nystagmus), pyramidal or extrapyramidal signs (such as spasticity, rigidity, dystonia, or bradykinesia), and eventually akinetic mutism 22. [radiopaedia.org]

  However, in HvCJD, the classic manifestation is cortical blindness due to involvement of the parieto-occipital cortex, which can be accompanied by myoclonus and progressive dementia(1,3). [ncbi.nlm.nih.gov]

• Insomnia

  Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene. N Engl J Med 1992; 326: 444-9. [ Links ] 68. Lugaresi E, Medori R, Montagna P, Baruzzi A, Cortelli P, Lugaresi A, et al. [scielo.cl]

  […] medications included levothyroxine 0.112 mg orally once per day, inhaled budesonide 200 microgram (mcg) per dose once daily, aluminum and magnesium hydroxide 10-20 mL orally every 4 hours (hrs) and lorazepam 1 mg sublingual as needed for agitation and insomnia [cureus.com]

  Folia Neuropathol 50, 20–45. [46] Parchi P, Capellari S, Gambetti P (2000) Intracerebral distribution of the abnormal isoform of the prion protein in sporadic Creutzfeldt-Jakob disease and fatal insomnia. [content.iospress.com]

• Dysmetria

  In the final stages, the patient presented monophasic speech, asynergy and prevalent right limb dysmetria during spontaneous motility and startle reaction. [mdpi.com]

  Six months later, her difficulties were followed by cerebellar signs (ataxia, dysmetria), myoclonus, dysphagia, and dysphonia. [content.iospress.com]

• Vertigo

  The patient presented with a sudden episode of vertigo which was treated as an isolated symptom. [cureus.com]

  In particular, according to a recent paper, the most common misdiagnoses are viral encephalitis, paraneoplastic disorder, depression, vertigo, Alzheimer’s disease, stroke, unspecified dementia, central nervous system vasculitis, peripheral neuropathy [mdpi.com]

• Nystagmus

  Other common features include myoclonus, visual hallucinations, cerebellar dysfunction (such as ataxia and nystagmus), pyramidal or extrapyramidal signs (such as spasticity, rigidity, dystonia, or bradykinesia), and eventually akinetic mutism 22. [radiopaedia.org]

Iatrogenic CJD Iatrogenic CJD is where the infection is accidentally spread from someone with CJD through medical or surgical treatment. [nhs.uk]

Treatment and prognosis There is currently no curative treatment and the disease is invariably fatal with a mean survival of only seven months. [radiopaedia.org]

As such, the patient was provided symptomatic treatment with beta-histine 16 mg orally (three times a day), to which, the client reported immediate relief. [cureus.com]

The treatment of sCJD remains supportive, and we found early referral to palliative care was important for end-of-life management for the patient and his family. [dovepress.com]

Treatment is, therefore, only palliative care [7]. In this paper, we present the case of a middle-aged man diagnosed with CJD Heidenhain Variant (HvCJD). [mdpi.com]

The prognosis is bleak, and death usually occurs within one year(2,9). It is important to make the differential diagnosis of HvCJD. [ncbi.nlm.nih.gov]

While such analysis has a limited effect on the overall prognosis, given the terminal nature of CJD, it is critical towards the overall accurate diagnosis and rehabilitation. [cureus.com]

Treatment and prognosis There is currently no curative treatment and the disease is invariably fatal with a mean survival of only seven months. [radiopaedia.org]

Despite all the aforementioned diagnostic techniques, patient prognosis remains poor. Typically, progression is rapid up to death, which occurs on average after about 4-6 months following diagnosis. Treatment is, therefore, only palliative care [7]. [mdpi.com]

It is the most prevalent prion disease in the world, with idiopathic, genetic and iatrogenic etiology [2]. Classic symptoms of CJD include rapidly progressive cognitive decline, ataxia and myoclonus. [cureus.com]

The epidemiology of Creutzfeldt-Jakob disease: conclusion of a 15 year investigation in France and review of the world literature. Neurology 1987; 37: 895-904 Velásquez-Pérez L, Reembao-Borjez D, Guevara J, Guadarrama-Torres R. [medigraphic.com]

[…] fulfilling the international diagnostic criteria.4 Definitive diagnosis of sCJD is made by histopathologic examination through post-mortem autopsy or ante-mortem biopsy.4 The annual incidence of sCJD is approximately 1 per million population, but the epidemiology [dovepress.com]

Bovine Spongiform Encephalopathy: Epidemiological Studies. Veterinary Record 1988; 123: 638-44. [ Links ] 74. Kirkwood JK, Wells GAH, Wilesmith JW, Cunningham AA, Jackson SI. Spongiform encephalopathy in the Arabian oryx. [scielo.cl]

Finally, although the occurrence of PRES is becoming increasingly easier to identify, thanks to improved neuro-radiological investigations, its precise incidence is currently unknown, mostly because epidemiological studies tend to focus on patient populations [mdpi.com]

Thus, the results of laboratory investigations significantly differ between the two sCJD types, which is in agreement with the known different pathophysiology of the disease process (e.g., earlier and more rapidly evolving neuronal dysfunction in MM1) [content.iospress.com]

Since the link between variant CJD and BSE was discovered in 1996, strict controls have proved very effective in preventing meat from infected cattle entering the food chain. See preventing Creutzfeldt-Jakob disease for more information. [nhs.uk]

[…] of vCJD familial (fCJD) 10% of cases these individuals carry a PRPc mutation iatrogenic (iCJD) following administration of cadaveric human pituitary hormones (pre-1985) various transplants The United State of America's Centers for Disease Control and Prevention [radiopaedia.org]

Centers for Disease Control and Prevention. 2018. 5. Uttley L, Carroll C, Wong R, et al. Creutzfeldt-jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation. [dovepress.com]

Centers for Disease Control and Prevention (Atlanta) Classic Creutzfeldt-Jakob Disease. December, 2013. [ Links ] 18. Cartier L, Fernández J, Ramírez E. Genetic markers in four Chilean families with familial Creutzfeldt-Jakob disease. [scielo.cl]

Currently there is no treatment for these diseases, but clinical trials involving monoclonal antibodies that prevent the interaction between physiological and misfolded forms of the protein, as well as studies on the role of cofactors have been considered [mdpi.com]