What is Dementia in Creutzfeldt-Jakob Disease?
Dementia is a chronic or progressive brain disorder in which higher cortical functions are impaired, including memory, thinking, orientation, understanding, counting, learning ability, language and judgment. Consciousness is not darkened. Symptoms are present for at least 6 months, although a more progressive onset of dementia is possible. Long-term medication, vitamin B12 and B6 deficiencies, metabolic imbalances due to kidney and liver diseases, as well as endocrine pathology, vascular pathology, depression, infections, head injuries, alcoholism, and tumors contribute to dementia. In the diagnosis of dementia, additional symptoms, such as delirium, hallucinations, and depression, are also noted.
Causes of Dementia in Creutzfeldt-Jakob Disease
The disease is caused by a slow-infection virus similar to chickens and scrapie, as well as a spongioform encephalopathy virus of cows. As a result of the lesion, after a long incubation period (up to 20 years), encephalopathy develops with astrocyte proliferation. Probably the participation of autoimmune processes at one of the stages of the disease. The defeat of gray and white matter occurs on their border in different parts of the brain.
Prevalence
The incidence of the disease is about one case per 1 million population per year, but it will increase significantly with the appearance of natural foci of slow infection viruses. In particular, as a result of an epidemic of spongioforne encephalopathy at the end of the 20th century, 11 cases of the disease were registered in the UK each year.
Symptoms of Dementia in Creutzfeldt-Jakob Disease
Disease prone patients aged 30 to 50 years, but it is possible that children’s dementia (Kramer – Polnova and Geller) belong to the same circle. Disease duration from 2 months to 2 years. Mortality exceeds 80%. We have described cases of recovery from this disease with the release of organic asthenia.
In the first stage (within a few hours), undeveloped delusions or delusional fantasies, hallucinatory-paranoid inclusions, twilight disorders of consciousness and epileptic seizures arise. Patients are confused, characterized by “floating attention”, the questions are answered periodically not essentially, they look around. Episodes of violent laughter and crying. Temperature is low-grade.
In the second stage, pyramidal and extrapyramidal disorders with choreoathetoid movements, catatonic episodes, amentia, cerebellar ataxia are noted. There is a distant-oral reflex. In position on the stomach crawling movements. Convincing changes in cerebrospinal fluid are absent.
In the third stage, spontaneous recovery is possible with the release of asthenia, but more often there is a lethal outcome.
Diagnosis of Dementia in Creutzfeldt-Jakob Disease
The diagnosis is based on the identification of a short period of polymorphic psychopathological symptoms with violent movements, the addition of pyramidal and extrapyramidal disorders. On the EEG, multiple peaks are detected in all leads. On CT – diffuse blurring the boundaries of gray and white matter.
Differential diagnostics
It should be differentiated from other meningitis, in particular, purulent and serous, in which specific changes in cerebrospinal fluid, meningeal symptoms and disorders of consciousness, both quantitative, from stunning to coma, and delirium are determined. Difficult differential diagnosis with leukoencephalitis (Schilder, Van Bogarde), in which mental disorders can be ahead of neurological. They usually begin with asthenia, irritability, absurd behavior, abortive auditory, olfactory and visual hallucinations, fear, anxiety, episodes of enuresis and encopresis. In the future is determined by the violation of understanding, non-criticism, euphoria, memory impairment. However, apraxia occurs early, a violation of the scheme of the body. Extrapyramidal rigidity, hyperkinesis, cerebellar and frontal ataxia, pyramidal and pseudobulbar disorders are detected neurologically. Schilder’s encephalitis is characterized by visual disturbances, including blindness, seizures, and, at the terminal stage, decerebration rigidity, trophic disorders and cachexia. Meanwhile, emotional attachment to loved ones is maintained.
Dementia Treatment for Creutzfeldt-Jakob Disease
There is no specific treatment. Immediate resuscitation and symptomatic therapy are needed. Antibiotic therapy is ineffective, but treatment with large doses of nootropics in combination with parenteral nutrition and hormones inspires some optimism.