Docstoc

CNS_III_MND_Tutexts

Document Sample
CNS_III_MND_Tutexts Powered By Docstoc
					                      CNS Patology - III
                                Motor Neuron
                                 Diseases



Intracranial Tumors
Jaroslava Dušková                Inst. Pathol. 1st. Med. Fac.
https://www1.lf1.cuni.cz/~jdusk/ Charles University, Prague
Neurodegenerative Diseases
  genetic abnormality

  modified protein

   pathologic structures

   loss of neurons
 Neurodegenerative Diseases
I. Polyglutamine diseases
   (multiple Cytosin– Adenin–Guanin CAG
                               complexes)
                            m. Huntington

II. – pathies,  –synucleinopathies
   m. Alzheimeri, m. Parkinsoni (Lewy bodies)
Motor Neuron Diseases
Axonopathies
    toxic
    toxoinfectious
    metabolic (drugs!)
    avitaminoses
    traumatic
    malignancy associted
Motor Neuron Diseases
Neuronopathies
   Poliomyelitis anterior acuta
   Poliomyelitis anterior chronica
   Sclerosis amyotrophica lateralis ALS
   Paralysis progressiva bulbaris
   Motor Neuron Diseases
                                  2.       1.
1. paralysis spastica spinalis
2. paralysis progressiva bulbaris C

  m. Aran Duchenne                     T
  (poliomyelitis ant. chronica)

   m. Werdnig Hoffmann         L
   myatonia congenita Oppenheim
1. + 2. ALS
Amyotrophic Lateral Sclerosis

Def.
    motor neuron disease affecting
 both 1st and 2nd neuron of pyramidal
 tract
Amyotrophic Lateral Sclerosis
Clinical features
   start:10 – 60 yrs
   palsies spastic/ feeble

   neurogenous hand muscle atrophy
                              „simian hand“
   bulbar disturbances

   death in several years (aspir. bpn.)
Amyotrophic Lateral Sclerosis
Morphology
  macro:        atrophy of gyrus praecentralis
                atrophy of ventral roots
                atrophy of muscles („simian“ hand)
  micro:        loss of neurons (GPC, ant. horns)
                funicular demyelinisation
                atrophy (denervation type)
Paralysis progressiva bulbaris
Clinical features
     fonation and deglution disturbances
       tachycardia, dyspnoe (insuff. n. X)
Morphology
     neuronal atrophia nn. IX, X, XI, XII.
              chewing muscles, tongue
Prognosis          fatal
Case Report                                 ALS
man 52 yrs (driver)               *1943      †1999
July 1991
    physical exercise        (mountain bike trip)
                               first symptoms
Disturbance of
    pronounciation
        transient , later standing expressive aphasia

     swallowing
     central hemiparesis dx., later sin.
Progression during 4 years death from
                             bronchopneumonia
    Amyotrophic Lateral Sclerosis
Etiopathogenesis                       (?)
   autoimmune
   genetic factors (9, 18, 21…)
   excitotoxic damage (glutamate release
    inhibitors prolong the survival)
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.
CNS neoplasms
   primary CNS neo:
    – approx. 2% of all cancers
    – approx. 20% of cancers in children under 15
 secondary
    – more frequented than the primary
CNS neoplasms - manifestation
    epilepsy
    focal deficits –palsies
    raised intracranial pressure
     – headache
     – vomiting
     – clouding of consciousness, coma
     – papiledema
    hydrocephalus
WHO Histological Typing
    of Tumours of the CNS (1)
 I.     NEUROEPITHELIAL TISSUE T.
 II.    NERVE SHEATH CELLS T.
 III.   MENINGEAL & RELATED TISSUES T.
 IV.    PRIMARY LYMPHOMAS
 V.     T. OF BLOOD VESSEL ORIGIN
 VI.    GERM CELL T.
WHO Histological Typing
    of Tumours of the CNS (2)
 VII. MALFORMATIVE and T.-LIKE
                      LESIONS
 VIII. VASCULAR MALFORMATIONS
 IX. ANTERIOR PITUITARY T.
 X. LOCAL EXTENSIONS of REGIONAL T.
 XI. METASTATIC
 XII. UNCLASSIFIED
WHO Histological Typing
    of Tumours of the CNS
III. TUMORs of MENINGEAL and
              RELATED TISSUES
  – meningioma
  – meningeal sarcoma
  – xantomatous tumours
  – melanoma (prim.meningeal)
  – melanomatosis
WHO Histological Typing
    of Tumours of the CNS
I. NEUROEPITHELIAL
  – astrocytic
  – oligodendendroglial
  – ependymal, choroid plexus
  – pineal cell
  – neuronal
  – poorly differentiated, embryonal
WHO Histological Typing
    of Tumours of the CNS
I. NEUROEPITHELIAL
  – astrocytic
  – oligodendendroglial
  – ependymal, choroid plexus
  – pineal cell
  – neuronal
  – poorly differentiated, embryonal
WHO Histological Typing
    of Tumours of the CNS
I. NEUROEPITHELIAL
  – astrocytic
  – oligodendendroglial
  – ependymal, choroid plexus
  – pineal cell
  – neuronal
  – poorly differentiated, embryonal
WHO Histological Typing
    of Tumours of the CNS
I. NEUROEPITHELIAL
  – astrocytic
  – oligodendendroglial
  – ependymal, choroid plexus
  – pineal cell
  – neuronal
  – poorly differentiated, embryonal
WHO Histological Typing
    of Tumours of the CNS
II. NERVE SHEATH CELLS TUMOURS
 –   neurilemmoma
 –   neurogenous sarcoma
 –   neurofibroma
 –   neurofibrosarcoma
WHO Histological Typing
    of Tumours of the CNS
IV. PRIMARY LYMPHOMAS

V.     VASCULAR TUMOURS
     – hemangioblastoma
     – hemangiosarcoma
WHO Histological Typing
    of Tumours of the CNS
VI. GERMINAL TUMOURS
 – germinoma
 – embryonal carcinoma
 – choriocarcinoma
 – teratoma
WHO Histological Typing
    of Tumours of the CNS
VII. DYSONTOGENETIC TUMOURS
                        and T. LIKE LESIONS
 – craniopharyngeoma
 – Rathke´s cyst
 – epidermoid cyst
 – dermoid cyst
 – colloid cyst of 3rd ventricle
 – enterogenous cyst, pituicytoma, nasal glioma
WHO Histological Typing
    of Tumours of the CNS
VIII. VASCULAR MALFORMATIONS
 – capillary teleangiectasia
 – cavernous hemangioma
 – a.– v. malformation
 – venous malformation
 – Sturge Weber (cerebrofacial / trigeminal
                            angiomatosis)
WHO Histological Typing
    of Tumours of the CNS
IX. PITUITARY TUMOURS
   – adenomas
   – carcinomas
X. LOCAL TUMOURS EXTENSIONS
   – glomus jug. tumour
   – chordoma
   – chondroma            – chondrosarcoma
   – esthesioneuroblastoma – cylindroma
WHO Histological Typing
    of Tumours of the CNS

XI. METASTATIC TUMORS

   mostly carcinomas !!!


XII. UNCLASSIFIED

				
DOCUMENT INFO
Shared By:
Categories:
Tags:
Stats:
views:10
posted:9/6/2012
language:Latin
pages:29