CNS Patology - III

1
CNS Patology - III

• Motor Neuron Diseases

Intracranial Tumors
Jaroslava Dušková Inst.
Pathol. 1st. Med. Fac. https//www1.lf1.cuni.cz/j
dusk/ Charles University, Prague
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Neurodegenerative Diseases

• genetic abnormality
• modified protein
• pathologic structures
• loss of neurons

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Neurodegenerative Diseases

• I. Polyglutamine diseases
• (multiple Cytosin AdeninGuanin CAG
  complexes)
• m. Huntington
• II. ? pathies, ? synucleinopathies
• m. Alzheimeri, m. Parkinsoni (Lewy bodies)

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Motor Neuron Diseases

• Axonopathies
• toxic
• toxoinfectious
• metabolic (drugs!)
• avitaminoses
• traumatic
• malignancy associted

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Motor Neuron Diseases

• Neuronopathies
• Poliomyelitis anterior acuta
• Poliomyelitis anterior chronica
• Sclerosis amyotrophica lateralis ALS
• Paralysis progressiva bulbaris

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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

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Amyotrophic Lateral Sclerosis

• Def.
• motor neuron disease affecting both 1st and 2nd
  neuron of pyramidal tract

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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.)

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Amyotrophic Lateral Sclerosis

• Morphology
• macro
• micro

• atrophy of gyrus praecentralis
• atrophy of ventral roots
• atrophy of muscles (simian hand)
• loss of neurons (GPC, ant. horns)
• funicular demyelinisation
• atrophy (denervation type)

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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

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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

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Amyotrophic Lateral Sclerosis

• Etiopathogenesis (?)
• autoimmune
• genetic factors (9, 18, 21)
• excitotoxic damage (glutamate release inhibitors
  prolong the survival)

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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. 200564(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.

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CNS neoplasms

• primary CNS neo
• approx. 2 of all cancers
• approx. 20 of cancers in children under 15
• secondary
• more frequented than the primary

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CNS neoplasms - manifestation

• epilepsy
• focal deficits palsies
• raised intracranial pressure
• headache
• vomiting
• clouding of consciousness, coma
• papiledema
• hydrocephalus

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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.

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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

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WHO Histological Typing of Tumours of
the CNS

• III. TUMORs of MENINGEAL and RELATED TISSUES
• meningioma
• meningeal sarcoma
• xantomatous tumours
• melanoma (prim.meningeal)
• melanomatosis

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WHO Histological Typing of Tumours of
the CNS

• I. NEUROEPITHELIAL
• astrocytic
• oligodendendroglial
• ependymal, choroid plexus
• pineal cell
• neuronal
• poorly differentiated, embryonal

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WHO Histological Typing of Tumours of
the CNS

• I. NEUROEPITHELIAL
• astrocytic
• oligodendendroglial
• ependymal, choroid plexus
• pineal cell
• neuronal
• poorly differentiated, embryonal

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WHO Histological Typing of Tumours of
the CNS

• I. NEUROEPITHELIAL
• astrocytic
• oligodendendroglial
• ependymal, choroid plexus
• pineal cell
• neuronal
• poorly differentiated, embryonal

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WHO Histological Typing of Tumours of
the CNS

• I. NEUROEPITHELIAL
• astrocytic
• oligodendendroglial
• ependymal, choroid plexus
• pineal cell
• neuronal
• poorly differentiated, embryonal

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WHO Histological Typing of Tumours of
the CNS

• II. NERVE SHEATH CELLS TUMOURS
• neurilemmoma
• neurogenous sarcoma
• neurofibroma
• neurofibrosarcoma

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WHO Histological Typing of Tumours of
the CNS

• IV. PRIMARY LYMPHOMAS
• V. VASCULAR TUMOURS
• hemangioblastoma
• hemangiosarcoma

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WHO Histological Typing of Tumours of
the CNS

• VI. GERMINAL TUMOURS
• germinoma
• embryonal carcinoma
• choriocarcinoma
• teratoma

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WHO Histological Typing of Tumours of
the CNS

• VII. DYSONTOGENETIC TUMOURS
• and T. LIKE LESIONS
• craniopharyngeoma
• Rathkes cyst
• epidermoid cyst
• dermoid cyst
• colloid cyst of 3rd ventricle
• enterogenous cyst, pituicytoma, nasal glioma

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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)

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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

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WHO Histological Typing of Tumours of
the CNS

• XI. METASTATIC TUMORS
• mostly carcinomas !!!
• XII. UNCLASSIFIED