Patologie CNS - III

1. Patologie CNS - III Nemoci motorick�ch neuronu

2. Neurodegenerativn� choroby genetick� abnormalita modifikovan� protein patologick� struktury ztr�ta neuronu

3. Neurodegenerativn� choroby I. Polyglutaminov� nemoci (mnohotn� Cytosin� Adenin�Guanin CAG komplexy) m. Huntington II. ?� patie, ? �synukleinopatie m. Alzheimeri, m. Parkinsoni (Lewyho tel.)

4. Nemoci motorick�ch neuronu Axonopatie toxick� toxoinfekcn� metabolick� (pol�kov�!) avitaminosy traumatick� sdru�en� s malignitou

5. Nemoci motorick�ch neuronu Neuronopatie Poliomyelitis anterior acuta Poliomyelitis anterior chronica Sclerosis amyotrophica lateralis ALS Paralysis progressiva bulbaris

6. Nemoci motorick�ch neuronu 1. spastick� spin�ln� paralysa 2. progresivn� bulb. paralysa C m. Aran Duchenne T (poliomyelitis ant. chronica) m. Werdnig Hoffmann L myatonia congenita Oppenheim 1. + 2. ALS

7. Amyotrofick� later�ln� sklerosa Def. nemoc pyramidov� dr�hy z atrofie 1. i 2. neuronu

8. Amyotrofick� later�ln� sklerosa Klinika zac. mezi 10.� 60. rokem obrny spasticko - chab� neurogenn� atrofie svalu ruky �opic� ruka� bulb�rn� poruchy smrt behem nekolika let (aspir. bpn.)

9. Amyotrofick� later�ln� sklerosa Morfologie makro: mikro: atrofie gyrus praecentralis atrofie predn�ch korenu atrofie svalu (�opic� ruka) �bytek neuronu (GPC, pr. rohy) demyelinizace provazcu atrofie denervacn�ho typu

10. Progresivn� bulb�rn� paralysa Klinika porucha fonace, polyk�n�, tachykardie, du�nost (z insuf. vagu) Morfologie atrofie neuronu nn. IX, X, XI, XII. �v�kac�ch svalu, jazyka Prognosa infaustn�

11. Kazuistika ALS mu� 52 let (ridic) *1943 �1999 cervenec 1991 fyzick� n�maha (j�zda na horsk�m kole) prvn� pr�znaky porucha v�slovnosti prechodn� , pozdeji trval� expresivn� af�zie polyk�n� centr�ln� hemiparesa dx., pozdeji i sin. progrese v prubehu 4 let smrt bronchopneumoni�

12. Sclerosis amyotrophica lateralis Etiopatogeneze (?) autoimmunn� poliovirus (Lansing) defektn� interferuj�c� c�stice (DIP) VROZEN� IMUNOPROFIL & Z�SKAN� FAKTORY

13. Hypothesis: A motor neuron toxin produced by a clostridial species residing in gut causes ALS.Longstreth WT Jr, Meschke JS, Davidson SK, Smoot LM, Smoot JC, Koepsell TD.University of Washington, Seattle, Washington, USA.Med Hypotheses. 2005;64(6):1153-1156. A yet-to-be-identified motor neuron toxin produced by a clostridial species causes sporadic amyotrophic lateral sclerosis (ALS) in susceptible individuals. Undetected it resides in the gut and chronically produces a toxin that targets the motor system, like the tetanus and botulinum toxins. Some of the toxin would cross to neighboring cells and to the upper motor neuron and similarly destroy these motor neurons. Weakness would relentlessly progress until not enough motor neurons remained to sustain life. If this hypothesis were correct, treatment with appropriate antibiotics or antitoxins might slow or halt progression of disease, and immunization might prevent disease. a yet-to-be-identified motor neuron toxin produced by a clostridial species causes sporadic amyotrophic lateral sclerosis (ALS) in susceptible individuals. This clostridial species would reside undetected in the gut and chronically produce a toxin that targets the motor system, like the tetanus and botulinum toxins. After gaining access to the lower motor neuron, the toxin would be transported back to the cell body, as occurs with the tetanus toxin, and destroy the lower motor neuron - the essential feature of ALS. Again like the tetanus toxin, some of the toxin would cross to neighboring cells and to the upper motor neuron and similarly destroy these motor neurons. Weakness would relentlessly progress until not enough motor neurons remained to sustain life. If this hypothesis were correct, treatment with appropriate antibiotics or antitoxins might slow or halt progression of disease, and immunization might prevent disease.a yet-to-be-identified motor neuron toxin produced by a clostridial species causes sporadic amyotrophic lateral sclerosis (ALS) in susceptible individuals. This clostridial species would reside undetected in the gut and chronically produce a toxin that targets the motor system, like the tetanus and botulinum toxins. After gaining access to the lower motor neuron, the toxin would be transported back to the cell body, as occurs with the tetanus toxin, and destroy the lower motor neuron - the essential feature of ALS. Again like the tetanus toxin, some of the toxin would cross to neighboring cells and to the upper motor neuron and similarly destroy these motor neurons. Weakness would relentlessly progress until not enough motor neurons remained to sustain life. If this hypothesis were correct, treatment with appropriate antibiotics or antitoxins might slow or halt progression of disease, and immunization might prevent disease.

14. Neoplasie CNS prim�rn� CNS neoplazie: cca. 2% v�ech malignit cca 20% malignit u det� mlad��ch 15 let sekund�rn� - metastatick� castej�� ne� prim�rn�

15. CNS neoplasie � klin. projevy epilepsie fok�ln� v�pady � par�zy, plegie zv��. intrakrani�ln� tlak bolest hlavy zvracen� (zejm. u det�) zastren� vedom�, coma ed�m papily hydrocephalus

16. Histologick� klasifikace tumoru CNS (WHO 2000) 128 ICD-O k�dovan�ch jednotek I. NEUROEPITELOV� T. II. N�DORY NERVOV�CH OBALU N�DORY MENING N�DORY C�VN�HO PUVODU PRIM�RN� MELANOCYT�RN� L�ZE LYMFOMY (prim�rn�) 128 k�dovan�ch jednotek128 k�dovan�ch jednotek

17. WHO Histologick� klasifikace tumoru CNS V. GERMIN�LN� TUMORY VI. N�DORY SEL�RN� OBLASTI VII. METASTATICK�

18. WHO Histologick� klasifikace tumoru CNS I. NEUROEPITELOV� N�DORY astrocytick� oligodendrogli�ln� ependym�ln�, choroid�ln�ho plexu pinealomy neuron�ln� �patne diferencovan�, embryon�ln�

19. WHO Histologick� klasifikace tumoru CNS II. N�DORY NERVOV�CH OBALU neurilemmom neurogenn� sarkom neurofibrom neurofibrosarkom

20. WHO Histologick� klasifikace tumoru CNS III. TUMORY MENING A SOUVISEJ�C�CH TK�N� meningeom meninge�ln� sarkom xantomatosn� tumory prim. melanom mening melanomatosa

21. WHO Histologick� klasifikace tumoru CNS IV. PRIM�RN� MALIGN� LYMFOMY

22. WHO Histologick� klasifikace tumoru CNS V. GERMIN�LN� TUMORY germinom embryon�ln� karcinom choriokarcinom teratom

23. WHO Histologick� klasifikace tumoru CNS V. GERMIN�LN� TUMORY VI. N�DORY SEL�RN� OBLASTI (kraniofaryngeom, n�dory hypof�zy) VII. METASTATICK� N�DORY

24. WHO Histologick� klasifikace tumoru CNS VII. TUMORY METASTATICK� (zejm. karcinomy a melanom)

25. Pseudotumory (dif. dg.!) Cysty Rathkeho cysta epidermoidn� cysta dermoidn� cysta koloidn� cysta 3. komory C�VN� MALFORMACE kapil�rn� teleangiektazie a.� v. malformace nejsou v nov� klasifikaci WHOnejsou v nov� klasifikaci WHO