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NEUROLOGY REVIEW James K. Rustad, M.D. Copyright © 2010. All Rights Reserved.

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1 NEUROLOGY REVIEW James K. Rustad, M.D. Copyright © 2010. All Rights Reserved.

2 Lecture Outline  Neuroanatomy Review  Neurotransmitters: Dopamine, Norepinephrine, Serotonin  Pain: Peripheral Nerve Fibers, Opiate Receptor Subtypes and Neuropathic Pain Syndromes  Headaches (Tension, Migraine, and Cluster Headaches)  Brain Hemorrhages (Subdural, Epidural and Subarachnoid Hemorrhages)

3 Lecture Outline  Dementias: Alzheimer’s, CJD, Lewy Body, Pick’s, NPH, SSPE  Neuropathology “power review”: Parkinson’s, Huntington’s, Duchenne’s, ALS, Guillian Barre, Myasthenia Gravis, Multiple Sclerosis, Seizure Disorder, and Brown-Sequard Syndrome.  Stroke Lesion Localization  Aphasias

4 Neuroanatomy Review  Frontal Lobe: Remember stimulation drives eyes contralateral and destruction induces ipsilateral eye movement. Prefrontal Region - Frontal lobe anterior to motor strip contains executive and inhibitory centers.  Parietal Lobe: Stereognosis, two-point discrimination, visual association area (perception of visual symbols). Angular Gyrus (Gerstmann’s syndrome).  Occipital lobe: Visual cortex.  Temporal lobe: Wernicke’s area. Mesial surface is origin of most partial complex seizures.

5 Neuroanatomy Review (continued)  Basal Ganglia = Caudate nucleus, Putamen, Globus Pallidus, Subthalamic Nucleus, Substantia Nigra.  Striatum = caudate nucleus and putamen.  Lenticular Nuclei = globus pallidus and putamen.  Nigrostriatal tract (substantia nigra to striatum).

6 Dopamine Metabolism Steps Phenylalanine Tyrosine DOPA Dopamine Norepinephrine Phenylalanine Hydroxylase Tyrosine Hydroxylase (rate- limiting) DOPA Decarboxylase Dopamine Beta Hydroxylase Phenylamine N- Methyltransferase Epinephrine

7 Serotonin Synthesis Serotonin Breaks down to 5-HIAA(5-Hydroxyindoleacetic acid) 5-hydroxytryptophan Converted by:Amino acid Decarboxylase Tryptophan Converted by:Tryptophan Hydroxylase

8 Peripheral Nerve Fibers, Opiate Receptor Subtypes and Neuropathic Pain Syndromes Pain

9 Peripheral Nerve Fibers TypeC fibersA-delta fibers SizeSmallLarge MyelinNoYes Conduction VelocitySlowFast CharacterDull, achingSharp, brief ExamplePancreatic cancerBurn

10 Opiate Receptor Subtypes ReceptorAction MuAnalgesia, respiratory depression, constipation, miosis (pupillary constriction), euphoria KappaAnalgesia, dysphoria, disorientation/depersonalization Deltaanalgesia

11 Neuropathic Pain Syndromes  Mononeuropathy: Carpal tunnel syndrome  Polyneuropathy: Diabetic, Alcoholic  Post-herpetic neuralgia

12 Neuropathic Pain Syndromes  Complex Regional Pain Syndrome Type I (Reflex Sympathetic Dystrophy)  Initial noxious event involving distal extremity, pain disproportionate to injury, not limited to single nerve distribution.  Associated with edema, changes in skin blood flow.

13 Neuropathic Pain Syndromes  Sequela of injury to nerve or major branch (esp. median, sciatic, tibial, and ulnar nerves).  Due to thalamic infarct or other lesion.  Hemi-anesthesia followed by gradual return of sensory function and pain.  Pain contra-lateral to lesion. Complex Regional Pain Syndrome Type 2 (Causalgia) Thalamic Pain: Dejerine-Roussy Syndrome

14 Headaches

15 Tension headache  Dull, bilateral, band like, chronic in course and usually related to stress.  Tx: NSAID, rule out Major Depression

16 Migraine: at least five episodes of Episodic Headache lasting four to seventy two hours  Unilateral  Throbbing  Worsened by movement  Moderate or Severe  Nausea or vomiting  Photophobia and phonophobia Any two of:Any one of:

17 Five Phases of Migraine Attack  Phase I: Prodrome  Phase II: Aura  Phase III: Headache  Phase IV: Headache Resolution  Phase V: Postdrome

18 Migraine Treatments  Non-specific: NSAIDS, Combination analgesics, Opioids, Antiemetics, Neuroleptics.  Specific: Ergotamine, Dihydroergotamine, Triptans.  Anticonvulsants (Divalproex, topirimate)  Tricyclics  Beta-Blockers  Calcium Channel Blockers AcutePreventive

19 Cluster Headache  M/F 6:1  Unilateral stabbing headache  Ipsilateral lacrimation and nasal congestion with rhinorrhea  Usually triggered by alcohol use.  Treatment: 100% Oxygen (“Air Supply”)

20 Cluster Headache  Rare Disorder (0.1-0.4% of population)  Age of onset 20’s-30’s  Clinical features: Excruciatingly severe, retro-orbital pain. Radiates to temple, teeth, neck. Last 15-180 minutes.  Ipsilateral autonomic features, pacing, nocturnal attacks often awaken them.  Triggers: Alcohol, Nitroglycerin.  Cycles usually occur spring and fall and last 4-12 weeks.

21 Cluster Headaches  Oxygen 5-10 Liters  Sumatriptan injection (FDA approved)  Ergotamine  Prednisone  Verapamil  Lithium (for chronic cluster headaches)  Divalproex AcutePreventive

22 Subdural, Epidural and Subarachnoid Hemorrhages Brain Hemorrhages

23 Subdural Hematoma  Suspect in older patient with new-onset headache  Follows head trauma  Tearing of bridging veins  Extra-axial mass causes focal findings  Waxing and Waning mental status  “Concave on CT”

24 Epidural Hematoma  Between bone and dura  Associated skull fracture in 85% of adults  Middle meningeal artery tear  Brief LOC, initial lucid period then acute deterioration  “Convex on CT”

25 Subarachnoid Hemorrhage  Cause: Rupture of aneurysm or AV malformation. Clinical feature: sudden onset of “worst headache of my life”  Studies: CT scan. LP will show blood and Xanthochromia (yellow heme pigment)  Tx: Clip aneurysm  “Athena and Zeus”

26 Subarachnoid Hemorrhage  Occipito-nuchal  Sentinel bleeds  Most common cause?  Trauma

27 Dementias

28 Alzheimer’s Disease  Extracellular deposition of amyloid-beta protein, intracellular neurofibrillary tangles, and loss of neurons.  CT: Atrophy, ventricular dilatation  Treatment:

29 CJD vs. AD CJDAD CourseSix months (rapid)Insidious (years to decades) Physical findingsMyoclonus EEGPeriodic complexes, burst- suppression Slowing of background CSF14-3-3 protein (>90%), some false + PathologySpongiform changesPlaques and tangles TransmissibilityPerson to person and person to animal Non-transmissable

30 Huey, Dewey and…

31 …..Lewy Body Dementia  2 nd commonest cause of degenerative dementia  Progressive cognitive impairment  Spontaneous onset Parkinson’s symptoms  Extreme neuroleptic sensitivity, Sleep problems, V/H

32 Pick’s disease  Personality change  Decline in function  Poor social judgment  Inappropriate  Pathology: Prominent fronto-temporal atrophy, neuron cell inclusions, clustered cytoskeletal elements

33 “NPH wouldn’t do that!”  Triad: an imbalanced, wide-based walk or “shuffle,” urinary incontinence and MEMORY PROBLEMS.  Dementia predominantly frontal lobe in nature, with apathy, dullness in thinking, and slight inattention. Memory problems are usually main problem, which can lead to the misdiagnosis of AD.  Neil Patrick Harris, aka “Doogie Howser, MD”

34 Normal Pressure Hydrocephalus  Dementia  Incontinence  Gait apraxia (“magnetic gait”)

35 Subacute Sclerosing Pancephalitis (SSPE)  Dementia and myoclonus with onset in childhood  Course usually rapid and fatal, may be aborted with antiviral drugs  CSF has anti-measles antibodies, and EEG shows periodic or burst-suppression  Cause probably measles or measles-like virus

36 Parkinson’s, Huntington’s, Duchenne’s, ALS, Guillian Barre, Myasthenia Gravis, Multiple Sclerosis, Seizure Disorder, and Brown- Sequard Syndrome. Neuropathology “power-review”

37 Parkinson’s Disease  Tremor: resting, “pill- rolling,” coarse, 3-5 Hz.  Rigidity: “cogwheel”  Bradykinesia or akinesia  Festinating gait: small accelerating steps with decreased arm swing.  Postural reflex abnormalities including a positive “pull test”  Hypophonia, Micrographia  Sleep Disturbances (partly iatrogenic from dopamine agonists) Cardinal FeaturesMinor Features

38 Huntington’s Disease  Atrophy of cerebral cortex and head of caudate nuclei leads to compensatory enlargement of lateral ventricles (“bat-wing”).  Excessive Cytosine-Adenine-Guanine (CAG) trinucleotide repeats (other illnesses with excessive trinucleotide repeats include: Myotonic dystrophy, Fragile X syndrome, many forms of Spinocerebellar degeneration).

39 Duchenne’s Type Muscular Dystrophy  Sex-linked recessive inheritance with onset in early childhood  Caused by loss of muscle membrane protein, dystrophin  Pseudohypertophy (especially in calves)  Proximal weakness: requires arms to arise from chair or floor  DTRs decreased except for Achilles reflexes (ankle)  Elevated muscle enzymes: CPK, aldolase

40 UMN vs. LMN Sign and SymptomsUpper Motor NeuronLower Motor Neuron ReflexesHyperactiveHypoactive Babinski signPositive (dorsiflexion)Negative Muscle atrophyAbsentPresent Muscle toneSpasticityFlaccidity FasciculationAbsentPresent

41 Amyotrophic Lateral Sclerosis  Combines UMN and LMN symptoms  Asymmetric limb weakness = most common presentation. Limb fatigability, twitching, wasting, and stiffness.  Bulbar onset (2nd most common type) features dysarthria and dysphagia.  “Lou Gehrig’s disease”

42 Clinical Case Scenario A patient develops progressive weakness two weeks after a viral infection. What is possible diagnosis and what tests would you order?

43 Acute Idiopathic Polyneuropathy  “Guillain Barre Syndrome”

44 Guillain-Barre  Symmetric weakness, usually beginning in the legs, and more marked proximally than distally.  Some sensory complaints.  Typical absence of deep tendon reflexes. May be marked autonomic dysfunction.  Slow conduction velocity  CSF shows increased protein concentration but normal cell count. Electrophysiology shows marked slowing of motor/sensory nerve conduction velocity (denervation and axonal loss)

45 Guillain-Barre Treatment: Plasmapheresis or IV IG

46 Myasthenia Gravis  Insidious onset. Slowly progressive course.  Diplopia, dysarthria, ptosis, extremity and generalized weakness not conforming to distribution of any single nerve.  Pupillary responses not affected.  Persistent activity of a muscle group leads to temporary increase of weakness > restoration of strength after brief rest.

47 Myasthenia Gravis  Edrophonium challenge test (screening)  EMG  Confirmatory: Ach receptor antibody test  Tx: Neostigmine, Pyridostigmine (if poor response: Corticosteroid).  Consider Thymectomy in all patients < 60 yr unless only extraocular weakness.

48 Multiple Sclerosis  Relapsing remitting symptoms  Separated in space and time  MRI: Multiple periventricular plaques in white matter.  Oligoclonal bands in CSF.

49 Internuclear ophthalmoplegia  When the patient attempts to look to left, left eye turns with nystagmus (abducted eye) and right eye cannot turn to left (impaired adduction).  Location of lesion: Medial longitudinal fasciculus.  Young adults: MS most common cause  Older patients: vascular diseases.

50 Seizure Disorder Partial Seizures: originate in a focal region of a single hemisphere Simple Partial Seizures: no alteration of consciousness Secondary Generalization: spreads to both hemispheres Complex Partial Seizures: alteration of consciousness Brief Duration (i.e. 2-3 minutes); May have motionless staring or automatisms (involuntary motor behaviors)

51 Generalized seizures: involve both cerebral hemispheres from the outset (1). Tonic: sudden muscular rigidity (extension or flexion) (2) Clonic: generalized rhythmic jerking of muscles, no initial tonic phase (3) Generalized tonic clonic (may feature epileptic cry, apnea/cyanosis). Ictal activity 1-2 min; Post-ictal: incontinence, flaccidity, gradually return to normal consciousness. Disoriented, headache, sore mouth (oral trauma, muscular ache) ( 4). Atonic Seizure: brief loss of muscle tone (5). Absence ("petit mal") a few seconds of staring/blank look/unresponsive (6). Myoclonic: localized or widespread: non-rhythmic rapid jerking movements of muscles

52 Brown-Sequard Syndrome  Contra: loss of P and T, Ipsi: loss of vib./joint position, paralysis, hyperreflexia, Babinski

53 CSF in Meningitis DiagnosisCellsGlucose (mg/dl) Protein (mg/dl) Opening Pressure Normal0-5 lymphocyte 45-8515-4570-180 Aseptic,viral25-2000, mostly lymphocytes NORMALHigh > 50Slight elevation Bacterial200-20,000 Polymorphs, neutrophils Low <45High >50Markedly high Tuberculosis100-1000 lymphocytes Low <45High > 50Moderate Elevation


55 The Circle of “whatchu talkin’ bout” Willis?

56 Anterior Cerebral Artery  Profound lower extremity weakness (contralateral)  Stroke may result in paralysis/sensory loss in contralateral leg and foot.

57 Middle Cerebral Artery  Profound upper extremity weakness (contralateral).  Contralateral hemiplegia, hemisensory loss and homonymous hemianopia, eyes deviated to side of lesion. Apraxia/neglect. If left lobe involved: Global aphasia.

58 Posterior Cerebral Artery  Contralateral homonymous hemianopia, with macular sparing.  Prosopagnosia (inability to recognize faces)

59 Vertebrobasilar Artery  Vertigo  Nausea and vomiting  “Drop Attack”  Vertical Nystagmus  Dysarthria/dystonia  Ataxia  Labile Blood Pressure

60 Posterior inferior cerebellar artery  Lateral Medullary or Wallenberg’s syndrome  Sensory loss of the “FACE”  9 and 10 C.N. damage  Limb ataxia  Horner’s syndrome  Spinothalamic sensory loss

61 Pure Motor Stroke  Weakness of face, arm, and leg on one side of body (without cognitive, sensory, visual abnormalities) favors presence of thrombotic stroke involving penetrating arteries or small intracranial hemorrhage.

62 Gerstmann’s Syndrome  Finger agnosia  Left-right disorientation  Agraphia  Acalculia  Dominant parietal lobe angular gyrus lesion (stroke or congenital abnormality).  Associated with learning disabilities, right-sided paresis and hyperactive DTR’s.

63 CategoryNon-fluent AphasiaFluent Aphasia Previous terms (not synonymous) Expressive, Motor, Broca’s, Anterior Receptive, Sensory, Wenicke’s, Posterior Spontaneous SpeechOutput: Sparse < 50 WPM, single words Content: Nouns and Verbs primarily, Dysarthric, Telegraphic Output: Plentiful > 100 WPM, entire phrases, Paraphasias (incorrect words, clang associations, neologisms) Response to TestsComprehension Preserved but Naming and Repeating impaired Comprehension Impaired (variable) and Naming and Repeating impaired Associated DeficitsR-sided hemiparesis (arm > leg) Minimal hemiparesis, hemianopia, hemisensory loss EtiologyCVAs, trauma, other focal lesions Focal lesions, sometimes frontotemporal dementia, AD, cerebral anoxia Location of LesionFrontal lobe, especially L MCATemporal or parietal lobe

64 Variants of Fluent Aphasia ConductionTranscortical (Isolation) AbnormalityCannot repeatCan only repeat (echolalia), other functions variable Nature of InjuryDisconnects arcuate fasciculus Isolates language arc from remainder of cortex EtiologyFocal lesionsAnoxia, CO poisoning, occasionally AD Location of lesionPosterior temporal lobeArea(s) surrounding peri- sylvian arc

65 Any questions?

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