CNS Patology - III
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Transcript of CNS Patology - III
CNS Patology - III
Motor Neuron Diseases
Jaroslava Dušková Inst. Pathol. 1st. Med. Fac. https://www1.lf1.cuni.cz/~jdusk/ Charles University, Prague
Intracranial Tumors
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 Diseases1. paralysis spastica spinalis 2. paralysis progressiva bulbaris C
m. Aran Duchenne T (poliomyelitis ant. chronica)
m. Werdnig Hoffmann L myatonia congenita Oppenheim1. + 2. ALS
Classification of Disorders Affecting Motor Neurons
Primary– idiopathic (ALS)– inherited (SMA)
Secondary– infective :acute poliomyelitis, HIV, syphilis, prions– metabolic: hyper/hypo thyr, hyperparathyr…– immune. paraproteinemia– Environmental/toxic: Pb, Sb, Cd…neurolathyrism– vascular– paraneoplastic: nHML, MLH
Multisystem neurodeg. diseases affecting motor neurons– Western Pacific ALS /Parkinson/dementia complex– spinocerebellar deg– Huntington´s disease– prionoses
β-ODAP = 3-N-oxalyl-L-2,3diaminopropionic acid
The level of this compound in the dry seeds varies depending on genetic
factors and environmental conditions.
Amyotrophic Lateral Sclerosis
Def.
motor neuron disease affecting
both 1st and 2nd neuron of pyramidal
tract
Amyotrophic Lateral Sclerosis
Clinical featuresstart: 10 – 60 yrspalsies spastic/ feebleneurogenous hand muscle atrophy
„simian hand“bulbar disturbancesdeath in several years (aspir. bpn.)
Amyotrophic Lateral Sclerosis
Morphologymacro:
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)
Paralysis progressiva bulbaris
Clinical featuresfonation and deglution disturbances tachycardia, dyspnoe (insuff. n. X)
Morphology neuronal atrophia nn. IX, X, XI, XII.
chewing muscles, tonguePrognosis fatal
Case Report ALSman 52 yrs (driver) *1943 †1999
July 1991 physical exercise (mountain bike trip)
first symptomsDisturbance 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.
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 (2)
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
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 CNSVII. 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 CNSIX. 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