diseases of the nervous system - indiana university
TRANSCRIPT
1
Diseases of the Nervous SystemDiseases of the Nervous System
Central nervous systemCentral nervous systemBrain is a prisonerBrain is a prisonerBasic cellular elementsBasic cellular elements
Neurons, location means everythingNeurons, location means everythingNeuronal reaction to injury, very limitedNeuronal reaction to injury, very limited
Axonal growthAxonal growthNo regeneration of lost cellsNo regeneration of lost cellsAccumulation of junk within the cells can be harmful.Accumulation of junk within the cells can be harmful.
Glial component, supportiveGlial component, supportiveMicroglia, the police force of the CNSMicroglia, the police force of the CNSAstrocytes, structural like fibroblasts elsewhereAstrocytes, structural like fibroblasts elsewhere
Gemistocytes are reactive astrocytesGemistocytes are reactive astrocytesOligodendrocytes, make myelin (the insulation)Oligodendrocytes, make myelin (the insulation)
MeningesMeninges
Cerebral EdemaCerebral Edema
Injury to brainInjury to brainTumorTumorRubor……Rubor……
Swelling can’t go Swelling can’t go anywhereanywhereCompression of vital Compression of vital structuresstructuresHerniationHerniation
SublaxSublaxTranstentorialTranstentorialCerebellar tonsilsCerebellar tonsils
Brainstem HemorrhagesBrainstem Hemorrhages CSF FlowCSF Flow
Made in the ventriclesMade in the ventriclesFlows down aqueductFlows down aqueductInto 4Into 4thth ventricleventricleOut into the Out into the subarachnoid spacesubarachnoid spaceUp to the arachnoid Up to the arachnoid granulationsgranulationsBack into the bloodBack into the bloodObstructions in Obstructions in movement will lead to movement will lead to hydrocehpalushydrocehpalus
2
HydrocephalusHydrocephalus
Obstruction to flow of CSFObstruction to flow of CSFOver production of CSFOver production of CSFInability of arachnoid Inability of arachnoid granulations to restore granulations to restore water of CSF back into water of CSF back into circulationcirculation
HydrocephalusHydrocephalusNoncommunicating: Can’t get out of ventriclesNoncommunicating: Can’t get out of ventriclesCommunicating: CSF can’t get to arachnoid granulationsCommunicating: CSF can’t get to arachnoid granulations
TraumaTrauma
Birth traumaBirth traumaHemorrhageHemorrhagePermanent lossPermanent loss
TraumaTrauma
Closed headClosed headCoupCoupContraContra--coupcoup
PenetratingPenetratingHemorrhageHemorrhageContusionContusionLacerationLaceration
ContusionsContusions
3
Subdural HemorrhageSubdural HemorrhageRotational injury tears little veinsRotational injury tears little veinsSlow venous bleedingSlow venous bleeding
Epidural HemorrhageEpidural Hemorrhage
Trauma with skull fxTrauma with skull fxMiddle meningeal a.Middle meningeal a.Hemorrhage Hemorrhage compresses braincompresses brain
Subarachnoid Subarachnoid HemorrhageHemorrhage
Not as commonly due Not as commonly due to trauma, but maybe.to trauma, but maybe.Arterial bleedingArterial bleedingTypically from Circle of Typically from Circle of WillisWillisBlood in subarachnoid Blood in subarachnoid spacespace
Vascular DiseaseVascular Disease
HypoxicHypoxicTIATIAStrokeStroke
InfarctionInfarction
HemorrhagicHemorrhagicVascular blowoutVascular blowoutTraumaTrauma
4
Ischemic InfarctsIschemic Infarcts Hypertensive Hypertensive HemorrhagesHemorrhages
Berry aneurysmBerry aneurysmSubarachnoidSubarachnoid
ParenchymalParenchymal
Berry AneurysmBerry Aneurysm Subarachnoid Subarachnoid HemorrhageHemorrhage
Lacunar InfarctsLacunar InfarctsHypertensive vascular diseaseHypertensive vascular disease‘Watershed’ infarcts‘Watershed’ infarcts
Chronic IschemiaChronic Ischemia
Chronic vascular Chronic vascular insufficiencyinsufficiencyAtherosclerosisAtherosclerosisMarked cerebral atrophyMarked cerebral atrophy
5
InfectionsInfections
Brain properBrain properMinengesMinengesBugBug
BacteriaBacteriaVirusVirusSpirochtesSpirochtesParasitesParasitesPrionsPrions
Bacterial MeningitisBacterial Meningitis
ExudateExudate over cerebral over cerebral hemisphereshemispheresBacteria grow in CSFBacteria grow in CSFCSFCSF
Cell countCell countGlucoseGlucoseProteinProtein
Age of patientAge of patientComplicationsComplications
ScarringScarringEpilepsyEpilepsyAbscessAbscess
Bacterial MeningitisBacterial Meningitis
Cerebral AbscessCerebral Abscess
Septic Septic endocarditisendocarditisBlood borne pathogensBlood borne pathogensMust surgically drainMust surgically drain
Viral EncephalitisViral Encephalitis
Infection of brain Infection of brain substancesubstanceHerpes Herpes -->>Absent temporal Absent temporal lobeslobesSporadicSporadicImmunsuppressedImmunsuppressedHIVHIV
6
HIV EncephalopathyHIV Encephalopathy
MeningitisMeningitisNeuronalNeuronalBoth cognitive motorBoth cognitive motorDiffuse cortical atrophyDiffuse cortical atrophyMicroglia at site of dead Microglia at site of dead neuronsneuronsGP120 protein is directly GP120 protein is directly toxictoxic
Tertiary SyphilisTertiary Syphilis
Years after initial infectionYears after initial infectionObliterativeObliterative end end arteritisarteritisMeningitisMeningitisBrain properBrain properTabesTabes dorsalisdorsalis
PrionPrion DiseaseDisease
No nucleic acidNo nucleic acidSporadic or geneticSporadic or geneticAccumulation of Accumulation of abnormally folded abnormally folded proteinproteinVariety of Variety of conformations of the conformations of the diseased proteindiseased proteinSpongioformSpongioformencephalopathyencephalopathyKuruKuru
PrionPrion DiseaseDisease
Degenerative DiseasesDegenerative Diseases
Not just aging changesNot just aging changesNeuronal DeathNeuronal DeathGray matterGray matter
White matter changes are secondaryWhite matter changes are secondarySelective or generalized lossSelective or generalized lossAtrophy (local or global)Atrophy (local or global)Histological featuresHistological features
NeurofibrillaryNeurofibrillary tanglestanglesIntracellular or Intracellular or intranuclearintranuclear inclusionsinclusions
7
Alzheimer’s DiseaseAlzheimer’s Disease
True dementiaTrue dementiaMarked atrophyMarked atrophyProtein alterationsProtein alterations
TauTau proteinproteinAmyloidAmyloid related related proteinproteinSenile plaquesSenile plaquesAmyloidAmyloidangiopathyangiopathy
Alzheimer’s DiseaseAlzheimer’s Disease
Progressive lossProgressive lossMemoryMemoryCognitiveCognitive
55--15 years15 yearsEventually loss of Eventually loss of languagelanguageHigher functionsHigher functionsParkinson’s in a fewParkinson’s in a fewPneumonia is often cause Pneumonia is often cause of deathof death
Alzheimer’s DiseaseAlzheimer’s Disease Alzheimer’s DiseaseAlzheimer’s Disease
Senile plaquesSenile plaquesVascular Vascular amyloidamyloid changeschanges
Parkinson’s DiseaseParkinson’s DiseaseParkinsonism, collection of symptomsParkinsonism, collection of symptoms
Rigidity, stooped posture, gait disturbances, pill rolling, faceRigidity, stooped posture, gait disturbances, pill rolling, faceDrug inducedDrug induced
Parkinson’s DiseaseParkinson’s Disease
HuntigntonHuntignton DiseaseDisease
HereditaryHereditaryProgressiveProgressiveExtrapyramidalExtrapyramidal motormotorChoreaformChoreaform movementsmovementsHuntington geneHuntington gene
TrinucleotideTrinucleotide repeatsrepeatsCAGCAGNormal 6Normal 6--34 copies34 copiesHD has 50HD has 50--70 repeats70 repeats
Caudate nucleus Caudate nucleus atrophyatrophySuicide and infectionsSuicide and infections
8
Amyotrophic Lateral Sclerosis (ALS)Amyotrophic Lateral Sclerosis (ALS)
Sporadic loss of Sporadic loss of motor neuronsmotor neuronsSpinalSpinalBulbarBulbarPoor swallowingPoor swallowingPneumoniaPneumonia
DemyelinatingDemyelinating DisordersDisorders
White matterWhite matterDisease of Disease of oligodendrocytesoligodendrocytesAutoimmune most timesAutoimmune most times
Multiple SclerosisMultiple Sclerosis
Lesions dispersed in Lesions dispersed in space and timespace and timeCome and goesCome and goesSymptomsSymptoms
Optic nerveOptic nerveUrinationUrinationHeat makes worseHeat makes worseWeaknessWeakness
Degeneration of white Degeneration of white mattermatterPlaquesPlaques
Multiple SclerosisMultiple Sclerosis
Areas of Areas of demylinizationdemylinizationPlaquesPlaques
Active repairActive repairQuiescentQuiescent
Multiple SclerosisMultiple Sclerosis
9
Toxic and Vitamin DeficienciesToxic and Vitamin Deficiencies
Thiamine DeficiencyThiamine Deficiency
BeriberiBeriberiAlcohol abuseAlcohol abuseAbrupt psychotic changesAbrupt psychotic changesWernicke’sWernicke’s encephalopathyencephalopathy
Hemorrhages in Hemorrhages in mamillarymamillary bodiesbodiesConfusionConfusionParalysis of Paralysis of extraoccularextraoccular musclesmusclesAtaxiaAtaxia
Korsakoff’sKorsakoff’sInabilbityInabilbity to form new memoriesto form new memoriesConfabulationConfabulation
B12 DeficiencyB12 Deficiency
Inability to maintain myelinInability to maintain myelinPosterior column degenerationPosterior column degeneration
EthanolEthanol
Acutely, neural depressantAcutely, neural depressantInhibitions go firstInhibitions go firstLoss of depth perceptionLoss of depth perception
ChronicChronicDegeneration of granular cell layer of cerebellumDegeneration of granular cell layer of cerebellumLoss of Purkinje cellsLoss of Purkinje cellsBergman’s Bergman’s gliosisgliosis
Fetal alcohol Fetal alcohol syndromsyndromMicrocephalyMicrocephalyGrowth retardationGrowth retardationFacial abnormalitiesFacial abnormalitiesMental retardationMental retardation
Abnormal migration of neurons during developmentAbnormal migration of neurons during development
10
CNS TumorsCNS Tumors
Primary vs. Primary vs. metastaticmetastaticBenign vs. malignantBenign vs. malignantFocal vs. diffuseFocal vs. diffuseAbove or below Above or below tentorumtentorumNot too common in adultsNot too common in adultsAbout 20% of childhood malignanciesAbout 20% of childhood malignanciesLocation is criticalLocation is criticalCell typeCell type
None are of neuronal originNone are of neuronal originAstocytomaAstocytoma, most, mostOligodendrocytomaOligodendrocytomaMicrogliomatosisMicrogliomatosisEpendymomaEpendymoma
AstrocytomaAstrocytoma
AstrocyticAstrocytic originoriginAbove Above tentorumtentorum most times most times in adultsin adults
Multiple gradesMultiple gradesCompresses surrounding Compresses surrounding tissuetissueHemorrhage and necrosisHemorrhage and necrosisWith higher grade With higher grade malignant tumors, malignant tumors,
Look for vascular growthLook for vascular growth
AstrocytomaAstrocytoma AstrocytomaAstrocytoma
EpendymomaEpendymoma MeduloblastomaMeduloblastoma
ChildrenChildrenMidline cerebellumMidline cerebellumSubarachnoidSubarachnoid spreadspread
11
MeduloblastomaMeduloblastoma MeningiomaMeningioma
Arise from Arise from meningesmeningesBenign in a biological Benign in a biological sensesenseConsider where it isConsider where it isFibroblast lookingFibroblast lookingCells in whirls and Cells in whirls and clustersclustersPsammomaPsammoma bodiesbodies
MeningiomaMeningioma
PsammomaPsammoma bodiesbodiesLittle calcificationsLittle calcificationsMicroscopicMicroscopicWithin the tumorWithin the tumorCan spot on XCan spot on X--rayrayConcentric layers Concentric layers -->>
Peripheral NervesPeripheral Nerves
Axon vs. Axon vs. SchwannSchwann cellscellsMotorMotorSensorySensory
Inflammatory, autoimmuneInflammatory, autoimmuneToxicToxicTraumaTraumaVascular, especially diabetesVascular, especially diabetesTumorsTumors
GuillianGuillian--BarrBarréé SyndromeSyndrome
Autoimmune?Autoimmune?Follows Follows
InfectionInfectionviral viral MycoplasmaMycoplasma
Allergic reactionAllergic reaction
DemylinizationDemylinizationAscending paralysisAscending paralysisPhrenicPhrenic nerve involvement is life threateningnerve involvement is life threatening
12
Peripheral Nerve Peripheral Nerve TumorsTumors
Actually nerve Actually nerve sheath tumorssheath tumors
SchwannSchwann cellscells
Cranial nerves tooCranial nerves tooV & VIIIV & VIII
NeurofibromatosisNeurofibromatosis
Two typesTwo typesNo capsuleNo capsuleType 1Type 1
GeneticGeneticAll over the bodyAll over the bodyGliomaGlioma of optic n. (rare)of optic n. (rare)MeningiomaMeningiomaCafCaféé--auau--laitlait spotsspotsPigmented nodules of irisPigmented nodules of iris
NeurofibromatosisNeurofibromatosis