Neurological DisordersPaul Kelner, M.D.

Neurological DisordersOverview and Organization of the Nervous SystemCentral Nervous System brain and spinal cordPeripheral nervous system cranial nerves and spinal nerves

Divisions of the Nervous System

Neurological Disorders:Organization of the Nervous SystemThe Central Nervous SystemThe Brain The Spinal CordMotor pathways (in spinal cord) - efferentSensory pathways (in spinal cord) - afferent

Central Nervous System

Afferent and Efferent Pathways

Neurological Disorders:Organization of the Nervous SystemPeripheral Nervous SystemCranial nervesSpinal nervesSomatic nervesAutonomic nervous systemSympatheticParasympathetic

Neurological disordersCranial Nerves

Cranial Nerves

Cranial Nerves Reviewed

Autonomic Nervous System

Neurological disordersCells of the Nervous SystemThe NeuronNeuroglia and Schwann cellsThe Nerve ImpulseSynapsesNeurotransmittersMyelin Formation:Peripheral NS Schwann cellsCentral NS Oligodendrocytes

Neuron

Neuroglia

Saltatory Conduction

http://s3.amazonaws.com/ppt-download/nerve-impulse-28770.ppt#256,1,NERVE IMPULSE/ACTION POTENTIAL

Web Site with AP transmission animation

The Synapse

The Brain

The Brain (Functional Areas)

Neurological disordersProtective structuresCraniumMeningesCerebrospinal fluid and the ventricular systemVertebral columnBlood supply

Neurological disorders

The Meninges

Cerebral Ventricular System

MRI of Spinal Cord within Vertebrae

Cerebral Blood Supply

Circle of Willis

PainA Summary of Important ConceptsPart II

Pain

Pain (definitions)Pain Threshold point at which a stimulus is perceived as painPain Tolerance amount of pain a person will tolerate before outwardly responding to itNociceptive pain pain resulting from direct tissue injuryNon-nociceptive pain neuropathic pain

Neuropathic (non-nociceptive pain)

Neurological disordersPain (continued)Acute versus chronic painAcute pain is a protective mechanismChronic pain is persistent, lasting > 6 months

Neurological disordersClinical manifestations of painAcute painSomatic painVisceral painReferred painChronic painNeuropathic painHyperesthesiasPhantom limb painCancerReflex sympathetic dystrophy(RSD)

Referred Pain

Alterations in Neurological FunctionPart III

Neurological DisordersAlterations in Cognitive SystemsAlterations in arousal Coma Structural vs. metabolic vs. psychogenic causesGrouped according pathologic processInfectiousVascularNeoplasticTraumaticCongenitalDegenerativePolygenicMetabolic

ComaBy definition, coma (decreased arousal) is produced by:Bilateral hemispheric damageSuppression by hypoxia,hypoglycemia, drugs or toxinsBrain stem lesion or metabolic derangement that suppresses Reticular Activating System (RAS)

Neurological disordersComaClinical manifestationsLevel of consciousnessPattern of breathing (cheyne-stokes)Pupillary changesOculomotor responses (ie. Dolls eyes)Motor responses

PosturingDecorticateFlexion of arms, wrists, fingersAdduction of upper extremitiesExtension of lower extremitiesDecerebrateExtremities in extensionPronation of forearms and plantar extension of feet

Decorticate ->

Glasgow Coma Scale (GCS)

Neurological disordersOutcomesMortalityBrain death brain stem death no potential for recovery no control of homeostasisCerebral death death of cerebral hemispheres not including the brain stem vegetative stateMorbidity Recovery of consciousnessResidual cognitive dysfunctionPsychosocial domainVocational domain

Neurological disordersSeizures a sudden, explosive disorderly discharge of cerebral neuronsCharacterized by sudden, transient alterations in brain functionClinical manifestations Motor, Sensory, Autonomic, Psychic, Level of arousalEpilepsy term applied to seizures in which no underlying cause is foundGeneral term for primary condition causing seizures

Neurological disordersConditions associated with seizure disordersAny disorder that alters neuronal environmentMetabolic defectsCongenital conditionsGenetic predispositionPeri- or post-natal injuryInfectionsTumorsDrugs or alcohol

Neurological disordersTerminologyAuraProdromaTonic phaseClonic phasePostictal state

Generalized SeizuresGeneralized Seizures (Produced by the entire brain) Symptoms 1. "Grand Mal" or Generalized tonic-clonic Unconsciousness, convulsions, muscle rigidity 2. Absence Brief loss of consciousness 3. Myoclonic Sporadic (isolated), jerking movements 4. Clonic Repetitive, jerking movements 5. Tonic Muscle stiffness, rigidity 6. Atonic Loss of muscle tone

Partial SeizuresPartial Seizures (Produced by a small area of the brain) Symptoms 1. Simple (awareness is retained)a. Simple Motorb. Simple Sensoryc. Simple Psychological a. Jerking, muscle rigidity, spasms, head-turningb. Unusual sensations affecting either the vision, hearing, smell taste or touchc. Memory or emotional disturbances 2. Complex (Impairment of awareness) Automatisms such as lip smacking, chewing, fidgeting, walking and other repetitive, involuntary but coordinated movements 3. Partial seizure with secondary generalization Symptoms that are initially associated with a preservation of consciousness that then evolves into a loss of consciousness and convulsions.

Neurological disordersTypes of seizure disordersGeneralized seizuresPartial seizuresStatus epilepticus AbsencePseudo-seizuresTreatment of seizure disordersMedicationsPatient educationsurgery

Neurological DisordersData processing defectsAgnosia failure to recognize form and nature of objectsDysphasia impairment in understanding or production of languageExpressiveEcholaliaAphasia loss of ability to understand or produce language

Neurological disordersDementia Progressive failure of multiple cerebral functionsSyndrome with many causesLoss of intellect with impaired mental abilitiesDisorientedMemory problems (recent and remote)Language problems Attentional focusAlterations in behaviors

Neurological DisordersEvaluation of causeNeuropsychological testingLaboratory and diagnostic testingTreat underlying causeInfectionsNutritional issuesProgressive dementiasGoal is to maintain current function and prevent continued deterioration

Neurological disordersAlzheimer disease (AD)Most common cause of severe cognitive dysfunction in older personsFamilial, early-onset occurs in persons before age 65Familial, late-onset known as FADNon-hereditary, late-onset AD occurs in 70% of casesExact cause is not known several theoriesLoss of neurotransmitter stimulation by choline acetyltransferaseMutations in genes that code amyloid proteinsAlterations in apolipoprotein E (binds beta amyloid)Neurofibrillary tangleSenile plaques diagnostic of Alzheimers DiseaseDiagnosisTreatment

Alzheimers Atrophy

Pet Scan and AD

Alzheimers Disease Microscopic Pathology

Neurological disorders:Trauma and BleedsHematomasExtradural hematomasSubdural hematomasSubarachnoid hemorrhageIntracerebral hemorrhage

Epidural Hematoma

Subdural Hematoma

Subarachnoid Hemorrhage

Intracerebral Hemorrhage

Neurological disordersCerebrovascular accidents (Stroke)Occurs in 600,000 persons per yearThird leading cause of death in USMost common in persons > 65 yearsMore common in womenMore common in African-Americans and AsiansHeredity component

CVA on CT

Neurological disordersRisk factors for CVAHypertensionSmokingDiabetesInsulin resistancePolycythemia and thrombocythemiaElevated lipoprotein-aImpaired cardiac functionHyperhomocysteinemiaAtrial fibrillationEstrogen deficiency

Carotid Artery Disease

Carotid Artery Disease

Neurological disorderThrombotic strokesDue to arterial occlusion caused by thrombiClassified secondary to clinical manifestationsTransient ischemic attacks (TIA)Caused by thromboembolic particles Abrupt onset of symptomsStrokes-in-evolution (Sometimes called RIND reversible ischemic neurologic deficit)Intermittent progression of neuro deficits over hours to daysCompleted strokesMaximum amount of destruction has occurred

Neurological disordersEmbolic strokesFragment of clot breaks off from thrombi outside of brainMost common from heart, aorta, carotid artery or thoraxRisk factors atrial fibrillation, MI, endocarditis, valve replacementsTumors, fat and air can also cause strokes

Hemorrhagic strokesThird most common cause of CVARisk factorsHypertensionAneurysms Bleeding disordersTumors Trauma Drug use

Neurological disordersSymptoms of CVADepend upon which artery is obstructedWeaknessFacial droopingLoss of or trouble with speechLoss of function of limbs hemiparesisLoss of or changes in visionHeadacheInability to recognize objects or personsChanges in level of consciousness

Neurological disordersTreatment of CVATime is Brain treatment within 6 hours of onset of symptomsInterventional and drug therapyClot busters thrombolytics TPA Improve blood flow - vasodilatorsStenting of vesselsPrevention of thrombus anti-platelet drugsPhysical, emotional and mental rehabilitationEducation of patient and family

AneurysmsMany etiologies (can be inherited)Dilation or outpouching of vesselsUsually go undiagnosed until they bleedTreated surgically

Aneurysm

Aneurysm Clip

Neurological disordersHeadaches Most common neurological disorderCan be a symptom of serious illnessCan be a symptom of being a nursing studentMigraines - Benign recurring headache provoked by a triggerAffects 11 million person in the U.S.Prevalent in women ages 15-55 years and can occur in childrenAuras can occurMost common is migraine without aura

Tension Vs. Migraine HeadachesSymptomATensionBMigraineIntensity, Duration and Quality of PainMild or moderate pain intensitySevere Duration of headache 30 min 7 days 4-72 hours Intense pounding, throbbing and/or debilitating

SymptomA TensionB MigraineIntensity, Duration and Quality of PainMild or moderate pain intensitySevereDuration of headache 30 min 7 days 4-72 hoursIntense pounding, throbbing and/or debilitatingDistracting but not debilitatingSteady acheLocation of PainOne side of headBoth sides of headAssociated SymptomsNausea/vomitingSensitivity to light and/or soundsAura before onset of headache such as visual symptoms

Neurological disordersBasis of migraines is multifactorialSerotoninVasoactive substancesInflammatory processesTreatment of migraineAvoidance of triggersRest or sleep in a dark, quiet roomCompresses, cold or warmMedicationsSerotonin antagonists (Imitrex)Beta or calcium channel blockersAspirin, caffeine, NSAIDSMagnesium supplements

Migraine On MRI

Neurological disordersMeningitis infection & inflammation of meningesCaused by bacteria, viruses, fungi, parasites or toxinsAcute, subacute or chronicBacterial vs. aseptic meningitisSymptomsFever, chills, petechial rashHeadache, photophobia, otophobia, neck stiffnessNuchal rigidity, decrease consciousness, seizures, hemiparesis, hemiplegia

Meningitis

Neurological disordersTreatment Supportive measures - Quiet, dark roomAntibiotics or anti-viral medicationsVaccinations are available for bacterial formChemoprophylaxis for exposed persons

Neurological disordersParkinsons diseaseCommon degenerative disease of basal ganglia involving the dopamine-secreting cellsOnset after age 40, most common in menPrimary vs. secondary

Degeneration of the Substantia NigraNORMALParkinsons Disease

Neurological disordersSymptomsResting tremorRigidityAkinesia hypokinesia and bradykinesiaStooped postureShuffling gait, equilibrium disordersOrthostatic hypotension, gastric/urinary retention and constipationDepression

Neurological disordersTreatmentAdministration of dopaminergic drugs -> L-DopaAntihistamines, amantadine reduce akinesiaDrugs lose effects over timeStem cell researchSlow, progressive diseaseTotal loss of functionDeath is commonly due to pneumonia

Neurological disordersMultiple sclerosis (MS)Common immune disorder involving CNSDemyelinating disorderOnset between 20 and 50 yearsFemales affected twice as often as malesMost prevalent in northern countriesGenetic susceptibilityPrevious viral insult in a genetically susceptible person T cells reactive to myelinDestruction of myelin leads to slowing and eventual blockage of conduction

MS on MRI

Neurological disordersMS (continued)Different types of MSMixed, spinal, cerebellar, amaurotic formsDifferent clinical coursesRelapsing-remittingPrimary progressiveSecondary progressiveProgressive relapsing

Neurological disordersMS (continued)SymptomsOptic neuritisVisual changesDizzinessNystagmusWeaknessNumbness, tinglingAtaxia, tremorBladder and bowel changes

Neurological disorderMS (continued)Diagnosis with CT scan or MRICSF examTreatmentAcute management of exacerbationsReducing frequency of relapses or disease progressionSteroidsInterferonImmunosuppressive agentsSymptom managementPhysical and occupational therapyPatient educationSupport of patient and family

Intracranial Neoplasms50 60% of all adult intracranial neoplasms are malignant gliomas and/or astrocytomasApproximately 25% of patients with primary tumors outside of the CNS will develop intracranial metastases. Most of these develop secondary to lung cancer.

Glioblastoma on MRI

Glioblastoma on MRIGLIOBLASTOMA

Cerebral Edema

Elevated Intracranial Pressure (ICP)Medical EmergencySymptoms include headache, vomiting and changes in LOCMedically treated with mannitol and other agentsDefinitive treatment involves correction of underlying pathologyComplications include herniation

*Afferent pathways move sensory impulses towards CNSEfferent pathways innervate effector organs such as skeleltal muscle, cardiac and smooth muscle as wel as glands transmits impulses away from CNSSomatic NS motor and sensory pathways regulating voluntary motor control of skeletal muscleAutonomic NS motor and sensory components and regulates bodys internal environment through involuntary control of organ systems*Neurons vary in size, shape and processes; contain cell body (AKA soma in groups called ganglia or plexuses), dendrites (extensions carrying nerve impulses toward cell body) and axons (projections that carry impules away from cell body)Typical neurons have one axon covered in myelin (insulating substance)Schwann cells forms and maintains the myelin myelin acts as an insulator allowing ions to flow between segments thereby increasing velocityNeuroglia are classifications of cell supporting the neurons of the CNSNerve injury leads to swelling, hypertrophy of the neurofilaments, myelin shealth shrinks and disintigrates and axon degenerates and disappearsNerve regeneration depneds on location of the injury, type of injury, iflammatory response and scarring; the closer to the cell body, the greater the chance the nerve cell will die; a crushing injury allows recovery than a cutting injurySynapses region between adjacent neurons impulses are carried across by chemical and electrical conductionChemical conduction uses neurotransmittersMore than 30 neurotransmitters norepinephrine, acetylcholine, dopamine, serotonin, histamine and various amino acidsNeurotransmitters are responsible for regulation of sleep, mood, dreams, maintenance of arousal, perception and integration of pain, emotional experiences

*Cranium*Acute alerts a person to a condition or experience that is immediately harmful; acute pain is sudden in onset and is relieved when chemical mediators which produced the pain are gone; mobilizes the person to take actionChronic cause is unknown and if known, the pain does not response to usual treatment; develops slowly, insidiously; individuals adapt and try to modify the pain; leads to hopelessness and depression, disability and unemployment

*Neuralgias result from infection or disease which damages a peripheral nerve diabetic neuropathyHyperesthesias increased sensitivity and decreased pain threshold to touch and painful stimuliPhantom limb pain pain felt in an amputated limb after the stump is completely healed; occurs if the neuronal pathway from the amputated limb is stimulated at any point along the pathwayCancer pain pain attributed to the advance of the disease, associated with the treatment of the disease, pain attributed to comorbidities *Level of consciousness confusion, disorientation, lethargic, obtunded, stuporous, coma light (purposeful movement with stimulation), coma (nonpurposeful movement with stimulation only), deep (unresponsiveness to stimuli)Pattern of breathing Cheyne-Stokes respirations smooth increase (crescendo) in rate and depth of breathing which peaks and is followed by a gradual smooth decrease (decrescendo) in the rate and depth of breathing to the point of apneaPupillary changes used to guide to eval the presence and level of brain stem dysfunction b/c brain stem areas control pupils also control level of arousal; must consider drugs pt is taking in evaluationSee figure 15-1 page 442Oculomotor responses resting, spontaneous and reflexive eye movements under changes with different levels of brain dysfunction see table 15-5 page 443 figure 15-3 pages 443 and 444Motor responses help evaluate level of brain dysfunction as well as determine the side of brain that is maximally damaged. Purposeful defensive or withdrawal movement of limbs to noxious stimuli requires an intact corticospinal systems. Inappropriate or not purposeful generalized motor movement, posturing, grimacing or groaning. Not present unresponsive severe dysfunction of corticospinal system

*Aura partial seizure experienced as a peculiar sensation preceding onset of generalized seizure gustatory, visual or auditory experience, dizziness, numbnessProdroma early clinical manifestations such as headache, malaise, depression occurring hours to days before the seizureTonic muscle contraction with excessive muscle toneClonic alterhnating contraction and relaxation of musclesPostictal state period immediately following seizure activity in generalized seizures people are confused, disoriented, complaining of headache, muscle aches and fatigue; may need to sleep after seizure, no memory of the seizure*Generalized seizuresAccount for 30% of seizuresInvolve neurons bilaterallyNot always with a focal onsetOriginate from a subcortical or deeper brain focusConsciousness is impaired or lostPartial seizuresUnilateral neuron involvementHave a focal onset and originate from cortical brain tissueConsciousness is maintain if kept to one hemisphereMay become generalized loss of consciousness (secondary generalization)Status epilepticus subsequent seizures before a person has time to regain consciousness Lasts more than 30 minutesOccurs from abrupt discontinuation of anti-seizure medications or inadequate treatment of seizure disorderMedical emergency due to cerebral hypoxia

*Classification is based upon etiology trauma, tumors, vascular disorders, infectionsDegeneration of nerve tissue from genetics, inflammation, biochemical alterations; atherosclerosis multiple infarcts; trauma, lesions in the frontal and temporal regions of brain; compression or increased intracranial pressures*Neurofib tangle protein on the neurons becomes distorted and twistedSenile plaques groups of nerve cells degenerate and coalesce around an amyloid core; plaques disrupt nerve transmissionDx of AD is made from a CT scan, history and physical examDisease develops over 5 yearsTreatment use of Aricept in early AD; pts are taught compensation techniques to help memory, maintain cognitive functions which are not impaired, maintain general health and nutrition*Extra dural hematomas epidural is another name for them. Occur in all age groups and account for 1-2% of all major head injuries. Most common in persons 20-40 yrs old. Injuries to menigeal vein or dural sinus leads to bleed. Sx include loss of consciousness at time of injury; become lucid for a few hours to a few days; as blood accumulates headache, vomiting, drowsiness, confusion, seizure and hemiparesis with subsequent loss of consciousness; dx with MRI or CT scan; tx is surgical evacuation and ligation of bleeding vesselSubdural hematoma found in 10-20% of persons with TBI; acute form develops rapidly usually w/in 48 hrs; subacute subdural hematomas develop over a few days after initial injury; chronic subdural hematomas most commonly in elderly and chronic alcoholics and occur over days to wks to months; tearing of the bridging veins in acute, torn cortical veins or venous sinuses also the source; the accumulation of blood acts as an expanding mass and increase intracranial pressure; sx of acute SDH include headache, drowsiness, restlessness, agitation, slowed cognition and confusion; as bleed progresses may develop visual changes, loss of consciousness and pupillary dilation; sx of Chronic SDH include chronic headache and tenderness over the hematoma. Tx is surgical evacuation of clot; chronic SDH may require a craniotomy to evacuate the blood and cut away the meninges around the clot.*Thrombi formed in vessels supply the brain; most commonly due to atherosclerosis and inflammatory disease process that damage the vessel walls. TIA sx depend on vessel affected; transient loss of vision, visual changes, speech/cognition changes, transient weakness, hemiparesis, facial drooping; treatment is life long anticoagulation with either anti-platelet medication, ASA or warfarinSx of stroke*Migraine triggers stress, hunger, weather changes, seasonal changes, sunlight, noise, jet lag, foods such as red wine and aged cheeses, chocolate, hormonal changes, oral contraceptivesMigraine auras smells, lights, visual changesMigraine w/o aura - Lasts 4-72 hoursUnilateral throbbing pain moderate to severe and aggravated by activityNausea, vomiting, photophobia, diarrhea

*Bacterial secondary to infection in bloodstream or extension of infected area to subarachnoid space; mortality is 25%; children and adolescents are primarily affected highly contagiousAseptic or viral inflammation limited to meninges; seasonal with summer and autumn most common times of year; *

Primary loss of neurons in substantia nigraSecondary anything other than primary cause virus, trauma, infection, drugs, toxins designer drugs - Ecstasy

*Degeneration of normal myelin sheaths

*Relapsing-remitting acute exacerbations with recovery and lasting disabilty; between attacks there is no disease progressionPrimary progressive steady progression of disease; only occasional minor recoveries (Plateaus); this course is fairly uncommonSecondary progressive pattern of clear cut relapses and recoveries; becomes steadily progressive over time with continued worsening of diseaseProgressive-relapsing rare type that is a steady progressive form but with acute exacerbations