Kreyttsfeldta-Jacob's disease
Contents:
Description:
Kroytsfeldt's disease — Jacoba, (Kreyttsfeldt's transcription — Jacoba is more widespread, it is called on names of the German doctors Hans Gerhard Creutzfeldt, Alfons Maria Jakob; synonyms: the pseudosclerosis is spastic, a syndrome of a kortiko-striospinalny degeneration, transmissible spongioformny encephalopathy, mad cow disease) — the progressing dystrophic disease of bark of a great brain, basal ганглиев and a spinal cord. It is considered the main display of spongy encephalopathy (a prionny disease).
Symptoms of the Disease of Kreyttsfeldta-Jacob:
Clinical criteria of dementia at BKYa are:
* quickly progressing — within 2 years — ("devastating") dementia with disintegration of all highest cortical functions; pyramidal disturbances (spastic paresis);
* extrapyramidal disturbances (choreoathetosis);
* myoclonus;
* ataxy, akinetic mutism;
* dysarthtia;
* epileptic seizures;
* visual disturbances (diplopia)
Disease stages:
1. A prodromal stage — symptoms are not specific and arise approximately at 30% of patients. They appear one weeks and months prior to emergence of the first symptoms of dementia and include an adynamy, sleep disorders and appetite, attention, memory and thinking, decrease in body weight, loss of a libido, change of behavior.
2. The initial period — visual disturbances, headaches, dizziness, instability and paresthesias are usually characteristic of the first symptoms of a disease. At the main part of patients gradually develops, is more rare — an acute or subacute debut. In certain cases, as at so-called amyotrophic forms, neurologic signs can precede the beginning of dementia.
3. The developed period — is usually noted the progressing spastic paralysis of extremities with the accompanying extrapyramidal signs, a tremor, rigidity and the characteristic movements. In other cases the ataxy, falling of sight or muscular fibrillation and an atrophy of an upper motor neuron can be noted.
Are characteristic of sporadic BKYa:
The terminal period — heavy dementia up to marasmus. All prionoza — quickly progressing diseases. The current can be subacute, but usually leads to death no more than in 1-2 years from the moment of accurately outlined clinical manifestation. The average duration of sporadic — about 8 months. Duration of a new form more also reaches on average 16 months. The average duration of a hereditary form — 26 months then there comes death.
Diagnosis.
BKYa has to be assumed in all cases of dementias which progress quickly within months or 1-2 years and are followed by multiple neurologic symptoms.
Certain BKYa:
* Characteristic neurologic and morphological including pathoanatomical and neuroradiological symptomatology.
* Proteazorezistentny PRR (according to Western-blottinga).
* Fasten identification - the associated fibrilla.
Probable BKYa:
* Progressirushchy dementia.
* A characteristic EEG-pattern (for sporadic BKYa).
* At least, 2 signs from following:
* o myoclonus;
o deterioration in sight;
o cerebellar symptomatology;
o pyramidal or extrapyramidal symptoms;
o akinetic mutism.
Possible BKYa:
* The progressing dementia.
* Atypical changes on EEG (or EEG cannot be carried out).
* At least, 2 signs from following:
* o myoclonus;
o deterioration in sight;
o cerebellar symptomatology;
o pyramidal or extrapyramidal symptoms;
o akinetic mutism.
o disease Duration — less than 2 years.
Etiologies of Kreyttsfeldta-Jacob:
Getting to an organism, the prion settles on a cell surface, interacting with normal proteins and changing their structure to pathological.
The pathological proteins collecting on a surface of a cell block the processes happening on a membrane that leads of cells to death.
The cells of a squirrel collecting on a surface start apoptosis (the programmed process of death of a cell).
Cell, trying to get rid of proteins on a surface, begins to make active oxygen connections, however protein on a surface does not allow them to go beyond a cell. Active agents destroy a cell.
Around the struck cells inflammatory processes with participation of the highly active enzymes affecting the next healthy cells begin.
Treatment of the Disease of Kreyttsfeldta-Jacob:
There is no causal treatment. The symptomatic treatment is carried out. At identification of BKYa it is necessary to cancel all medicines which can negatively influence mnestichesky functions and behavior of the patient. A set of potential therapeutic interventions at BKYa remain at the level of a discussion now. Traditional antiviral means: амантадин, interferona, passive immunization, vaccination of the person and animals — were inefficient.
Among the drugs which are positively influencing process:
* Brefeldin And, destroying Golgi's device, interferes with synthesis of PrPSc in the infected culture of cells.
* Blockers of calcium channels, in particular NMDA receptors, promote longer survival of the infected neyronalny cultures.
