Neurology Notes for Boards Dyanne P. Westerberg, DO

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Neurology Review

Dyanne P. Westerberg, DO FAAFP

Associate Professor and Chair, Department of Family and Community Medicine

Cooper Medical School of Rowan University

Headache• Primary

– Migraine– Cluster– Tension

• Secondary– Hemorrhage– Encephalopathy– Meningitis– Temporal arteritis– Neoplasm– Sinus– Exertional– Trauma

Cranial Nerve Type Function

Olfactory 1 S Smell

Optic 2 S Sight

Oculomotor 3 M Medial, superior and inferior rectus , inferior oblique, ciliary muscle, sphincter muscle of the eye

Trochlear 4 M Superior Oblique

Trigeminal 5 B Sensation of face, muscles of mastication

Abducens 6 M Lateral Rectus

Facial 7 B Taste ( anterior 2/3 tongue) muscle of facial expression, stapediusmuscle, stylohyoid muscle, digastric muscle, lacrimal, submandibular and sublingual glands

Vestibulococular(Auditory)

8 S Hearing and balance

Glossopharyngeal 9 B Taste( posterior 2/3 tongue) Pharyngeal sensation, parotid gland, styrlopharyngeus muscle

Vagus 10 B Sensation of trachea, esophagus,viscera,laryngeal,pharyngeal muscles, visceral autonomics

Accessory 11 M Sternocleidomastoid and trapezius muscle

Hypoglossal 12 M Tongue

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Overview of the CNS arterial supply.Nolte, John, PhD - Essentials of The Human Brain, 37-42© 2009 Copyright © 2010 by Mosby, Inc., an affiliate of Elsevier Inc.

Lateral ( A ), medial ( B ), and cross-sectional ( C ) views of the hemisphere showing the regions served by the anterior cerebral ( green ), middle cerebral ( blue ), and posterior cerebral ( pink ) arteries. The distal territories of these vessels overlap at their peripheries and create border zones. These zones are susceptible to infarcts ( C ) in cases of hypoperfusion of the vascular bed. Small border zones also exist ( A ) between superior ( green ) and inferior ( blue ) cerebellar arteries.

Haines, D.E.,Lancon, J.A. - Fundamental Neuroscience for Basic and Clinical Applications, 109-123.e1© 2013 Copyright © 2013 by Saunders, an imprint of Elsevier Inc.

Which headache is the most common?

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Which headache is the most common?

Tension accounting for 40%

Migraine Cluster Tension

Patient 10 to 30 yearsF>M

Young Men F>M

Types Without auraWith aura

Precipitating factors Stress, BCP, menstruation, exertion, food containing tyramine or nitrates, chocolate, cheese, processed meats

ETOH, vasodilators Stress, fatigue

Associated symptoms photophobia, visualabnormalities, aura

Horner’s syndrome, lacrimation nasal congestionsPain radiates to the jaw and teeth

Anxiety

Duration 4 to 72 hours 30 Min to 3 hours Variable

Treatment NSAID, ergots,triptans, Antiemetics, prophylaxis: tricyclics, BblockersCalcium channel blockersErgots, OMT

Also, 100% oxygen7 L/minute for 15 minutes, OMT

Also relaxation exercises. OMT

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Characteristics of the headaches

Migraines Cluster Tension

NauseaPhotophobia/phonophobiaIncrease with activityP-pulsatile qualityO- onset 4 to 72 hoursU-UnilateralN- N/VD- DisablingAura- flickering lights, spotsFully reversible neurological symptoms

Can be bilateralSeveral per day ( 1 to 8)Between 15 and 180 minutesEpisodes 6 to 12 weeksRemission for 12 months

Bilateral Like a tightening band around the headNon pulsatingNo increase with physical activityNo N/VNo Photophobia/Phonophobia

Characteristics of the headaches

Migraines Cluster Tension

NauseaPhotophobia/phonophobiaIncrease with activityP-pulsatile qualityO- onset 4 to 72 hoursU-UnilateralN- N/VD- DisablingAura- flickering lights, spotsFully reversible neurological symptoms

Can be bilateralSeveral per day ( 1 to 8)Between 15 and 180 minutesEpisodes 6 to 12 weeksRemission for 12 months

Bilateral Like a tightening band around the headNon pulsatingNo increase with physical activityNo N/VNo Photophobia/Phonophobia

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Medical Treatments

Acute

• Acetaminophen/Aspirin/Caffeine

• NSAID

• Triptans

• Antiemetics

• Dexamethasone

• Ergotamine

• Intranasal Lidocaine

• Isometheptene

Chronic – First Line

• Propanolol

• Amitriptyline

• Sodium Valproate

• Topiramate

• Divalproex

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Exertional Headache• Headache after physical activity

• Running

• Coughing

• Sexual Intercourse

• Bowel Movement

• Evaluation i.e. MRI if• >40

• Vomiting

• Prolonged duration

• Cardiac evaluation if risk factors

• A literature review from the early 1990s, of 219 a structural lesion was found in 22 percent

Meningitis

• Infection of the covering of brain and spinal cord- meninges

• Risk Factors: ear infections, sinusitis, immunocompromise, neurosurgery, maternal group B infection during childbirth

• H and P: headache, neck pain, photophobia, nausea, vomiting, confusion, fever, seizure, Kernig, Brudzinski, rash

Meningitis by AgeAge Most common

organismOther organism Emperic Treatment

Newborn Group B strep E.Coli, ListeriaH.influenza

AmpicillinCefotaximine

1 m to 2 y S.pneumoniaN.meningitis

Group B StrepListeria, H.influenza

VancomycinCeftriaxone

2-18 y N.meningitis S.pneumoniaListeria

VancomycinCeftriaxone

18 to 50 y S.pneumonia N.meningitisListeria

VancomycinCeftriaxone

50+yAltered cellularimmunityAlcoholic

S. pneumonia N. Meningitis, Listeria , gram-negative rods

VancomycinCeftriaxoneAmpicillin

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Viruses

• Viral Meningitis:

– Numerous viruses -Enteroviruses most common

– Nausea, vomiting, headache stiffness

– LP helps in diagnosis, more specific- PCR testing

• Encephalitis:

– Numerous viruses i.e flavivirus- West Nile*

– Headache, vomiting, change in mental status

* Increased incidence since 1999 (< 1% )

LP findings

Patient WBC Pressure Glucose Protein

Healthy <5 50-180mmH20

40-70 mg/dl 20-45mg/dl

Bacterial IncreasedEsp. PMN

increased decreased increased

viral IncreasedEsp. Lymphocytes

increased normal normal

Fungal or TB IncreasedEsp. Lymphocytes

Increased decreased increased

Prevention

• Vaccines for general population and aspleenicpatients

– Hib Vaccine

– Pneumococcal Vaccine

– Meningococcal Vaccine

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Prevention

• The Advisory Committee on Immunization Practices (ACIP) today stopped short of recommending routine use of serogroup B meningococcal vaccine in young people, instead leaving the decision to doctors and their patients.

• The committee voted 14-1 for individual choice on use of the vaccine in adolescents and young adults ages 16 through 23, with 16- to 18-year-olds as the preferred age-group. The move came in the wake of several meningitis B outbreaks and a number of sporadic cases on college campuses in the past 2 years.

• INSURANCE may not cover this.

Trigeminal Neuralgia

• Recurrent- Head and Facial Pain in the trigeminal area-made worse by palpation with radiation to the maxillary and mandibular areas.

• Stabbing and electrical• F> M age >50• The mechanism by which compression of the nerve

leads to symptoms appears to be related to demyelination in a circumscribed area around the compression

• MRI to r/o lesions such as tumor or MS• Treatment: carbamazepine 200 to 1,200 mg/day

recommended

Trigeminal Neuralgia

• Recurrent- Head and Facial Pain in the trigeminal area-made worse by palpation with radiation to the maxillary and mandibular areas.

• Stabbing and electrical• F> M ,age >50• The mechanism by which compression of the nerve

leads to symptoms appears to be related to demyelination in a circumscribed area around the compression

• MRI to r/o lesions such as tumor or MS• Treatment: carbamazepine 200 to 1,200 mg/day

recommended

Most Common Cause

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Temporal (giant cell) Arteritis

• The diagnosis of giant cell arteritis (GCA) should be considered in a patient over the age of 50 who complains of or is found to have:

●New headaches●Abrupt onset of visual disturbances ●Symptoms of polymyalgia rheumatica●Jaw claudication●Unexplained fever or anemia●High erythrocyte sedimentation rate (ESR) and/or high serum C-reactive protein (CRP)

Temporal( giant cell) Arteritis

• The diagnosis of giant cell arteritis (GCA) should be considered in a patient over the age of 50 who complains of or is found to have:

●New headaches

●Abrupt onset of visual disturbances 15 to 20% blindness

●Symptoms of polymyalgia rheumatica

●Jaw claudication

●Unexplained fever or anemia

●High erythrocyte sedimentation rate (ESR) and/or high serum C-reactive protein (CRP)

Trigeminal Neuralgia Temporal Arteritis

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TIA• American Heart Association/American Stroke

Association 2009 definition of TIA –Transient episode of neurological dysfunction caused by focal brain, spinal cord, or retinal ischemia, without acute infarction

• Underreported

• Sudden onset of unilateral paresis, speech disturbance, transient monocular vision loss

• NOT tinnitus, dizziness, vertigo, memory loss

( mimics)

Evaluation

• Thorough History – Is it recurrent?

• Physical exam esp. neuro

• Imaging- within 24 hours– Diffusion weighted MRI

– CT scan often completed in the ER

• Cardiac Assessment:– EKG

– Transthorasic echo/TEE• Patent foramen ovale, thrombus, valvular disease

– Telemetry

– Labs

Treatment- prevent future strokes10 to 20 % risk at 90 days often within 48 hours

• Modify risks: – hypertension, smoking, obesity, inactivity etc.

• Statins regardless of LDL:– Reduce by 50 % or less than 70

• Antiplatelets if non cardiac– 81 mg aspirin

– Dipyridamole/aspirin

– Clopidogrel

• Carotid endarterectomy or angioplasty – If 70 to 99% blocked and risk <6%

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Treatment- prevent future strokes10 to 20 % risk at 90 days often within 48 hours

• Modify risks: – hypertension, smoking, obesity, inactivity etc.

• Statins regardless of LDL:– Reduce by 50 % or less than 70

• Antiplatelets if non cardiac– 81 mg aspirin

– Dipyridamole/aspirin

– Clopidogrel

• Carotid endarterectomy or angioplasty – If 70 to 99% blocked and risk <6%

Not ASA and Plavix

Atrial Fibrillation ( AHA)

• Warfarin with INR between 2 and 3• Control group 4.5 % stroke

• Warfarin group 1.4% stroke

• Other agents: ( Do NOT have AHA approval)– Dabigatron- (Pradaxa)

– Rivaroxaban (Xarelto)

– Apixaban (Eiquis)

– Edoxaban ( Savaysa)

Who needs anticogulation?

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Stroke

• symptomatic cerebral ischemic events of > 24 hour duration

– 80%-87% ischemic ( thrombus or emboli)

– 13%-20% hemorrhagic

• Intracerebral

• subarachnoid

• Risk Factors: Age, FMH, obesity, DM, HTN, tobacco, AFIB, Stress, High Alcohol

Acute Treatment• EKG• Labs• Imaging to r/o Bleed• Thrombolytic Therapy if within 3 hours if acute and

clinically meaningful defecit• No bleed or AV malformation• Normal platelets• No anticoagulants• No trauma• BP < 185/110

• oxygen if hypoxic• Aspirin within 48 hours ( if no thrombolytics)• Do not lower BP unless extreme i.e. 220/120 or patient

has CAD to maintain cerebral perfusion• Monitor for complications i.e seizures, edema, bleed

Imaging

• For diagnosing ischemic stroke in the emergency setting:– CT scans (without contrast enhancements)

• sensitivity= 16% specificity= 96%

– MRI scan• sensitivity= 83%specificity= 98%

• For diagnosing hemorrhagic stroke in the emergency setting:– CT scans (without contrast enhancements)

• sensitivity= 89%specificity= 100%

– MRI scan• sensitivity= 81%specificity= 100%

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Seizure

• Sudden change in neurological activity (e.g. behavior, movement, sensation) causes by excessive synchronized discharge of cortical neurons in a limited (focal) or generalized distribution of the brain.

• Epilepsy: 2or more seizures that are not precipitated by illnesses or other inciting events i.e. alcohol withdrawal

Common Causes of Seizure by Age Group

• Infant– Hypoxic injury

– Metabolic defect

– Genetic or congenital abnormality

– infection

• Children– Idiopathic

– Infection

– Fever

– trauma

• Adult– Idiopathic

– Metabolic defect

– Drug or drug withdrawal

– Trauma

– Neoplasm

– Infection

– CVA or stroke

Types of SeizuresType Involvement Comments

Simple Partial Focal, cortical region of the brain

Focal sensory of motordeficit with no LOC

Complex Partial Focal Region of the temporal lobe

Hallucinations and repeated coordinated movements

Generalized Bilateral cerebral cortex Tonic- clonic repetitive contraction and relaxation

Absence Bilateral Cerebral cortex Mostly in children

Diagnosis: History and EEG

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Status Epilepticus

• Either > 30 minutes of continuous seizure activity or ≥ 2 sequential seizures without recovery of full consciousness between seizures

• Due to : numerous- infections , brain tumor

• Treat with IV benzodiazepines, then start phenytoin or phenobarbitol if refractory

• Mortality > 20 % if untreated.

Febrile Seizures

• Between age of 6 months and 5 years• Prevalence 2 to 5% • Males > female• No evidence of intracranial infection • Simple < 15 minutes, complex > 15 minutes.• Greater risk of developing epilepsy• LP should be performed in patients

• < 12 months• Complex seizure• Symptoms of meningitis

Parkinson Disease• Idiopathic dopamine depletion:

– loss of dopaminergic striated neurons in the substantia nigra and Lewy formation leading to abnormal cholinergic input to the cortex.

• Symptoms:– Resting tremor: pill rolling– Cogwheel rigidity– Bradykinesia/ akinesia- shuffling gait– Mask-like faces– Memory loss– Difficulty initiating movement– Postural instability– Stooped posture, decreased arm swing

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Parkinson Disease• Idiopathic dopamine depletion:

– loss of dopaminergic striated neurons in the substantianigra and Lewy formation leading to abnormal cholinergic input to the cortex.

• Symptoms: – Resting tremor: pill rolling– Cogwheel rigidity– Bradykinesia/ akinesia- shuffling gait– Mask-like faces– Memory loss– Difficulty initiating movement– Postural instability– Stooped posture, decreased arm swing

TRAP

Treatment of Parkinson DiseaseDrug Mechanism indication

Levodopa Dopamine precursor Initial therapy

Carbidopa Dopamine decarboxylaseinhibitor that reduces levodopa metabolism

Combined with levodopa to augment effects

Bromocriptine Dopamine receptor agonist Increases response to levodopain patients with declining response

Selegiline Monamine oxidase type B inhibitor

Early disease – may help delay need to start levodopa

Amantadine Increases synthesis, release or reuptake of dopamine

More effective against rigidity and bradykinesia

Antimuscarinicagents

Block cholinergic transmission

Adjuvant therapy

Drug induced Parkinson Disease

• the older major tranquilizers such as Haloperisol ( Haldol), Trifluoperazine(Stelazine)

• the newer major antipsychotic drugs such as Risperidone (Risperdal), Olanzapine (Zyprexa),

• drugs used for nausea, vomiting, and acid reflux such as Metoclopramide (Reglan)

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Myasthenia Gravis

• Autoimmune disorder• Antibodies bind to acetylcholine receptors at neuromuscular

junctions and block normal neuromuscular transmission• Bimodal distribution younger women, older men• H and P

• Fatigue• Ptosis• Diplopia• Dysphagia• Dyspnea

• Lab: + positive Ach receptor antibodies• Tensilon Test: symptoms improve with edrophonium or the Ice pack

test ( ptosis resolves with the ice pack)

Guillain Barre Syndrome

• Inflammatory neuropathy associated with progressive weakness usually symetrical and ascending

• Autoimmune• Can be associated with recent viral infection, surgery ot

immunization• Peak 20’s and 70’s• Self resolving in 1 month- sooner with plasmapheresis or

immunoglobulin• H and P:

• Rapid bilateral weakness in distal extremities in stocking/glove distribution and going proximal

• Decreased sensation• Absent DTR’s• Respiratory Failure

Amyotrophic Lateral Sclerosis

• Progressive neuro degenerative disease of the motor neurons

• Loss of central nervous system – lower motor neurons

» anterior horn cells in spinal cord

» cranial nerve nuclei (most often X, XI, XII)

– upper motor neurons

» corticospinal tract

• Ages 20 to 80

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Amyotrophic Lateral Sclerosis

• H and P– Asymmetrical progressive weakness in the limbs and

face– Possible change in personality and impaired judgment– Increase or decrease in DTR– Flaccid paralysis– Babinski– Fasciculations of the muscles

• EMG: Widespread muscular denervation and motor block

• Treatment: riluzole- supportive therapy

Alzheimer’s Disease

• Most common cause of Dementia• Due to neurofibrillary tangles, neuritic plaques, amyloid

deposits,neuronal atrophy• Cortical atrophy on Imaging• H and P

• Progressive short term memory loss• Depression• Confusion• Inability to perform complex tasks or movements• Personality changes and delusions

• Treatment– Cholinesterase inhibitors– Memantine ( alone or in combination)– Herbals?????

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Multiple Sclerosis

• Possible autoimmune

• Demyelinating disorder of brain and spinal cord

• Most patient women 20 to 40

• Mri with contrast shows white matter lesions

• H+ P: various symptoms, visual changes( Optic Neuritis- 10 year risk 38%), babinski, positional instabilities, spasticity, dysarrthria

• McDonald Criteria for diagnosis

• Treatment: steroids, methotrexate,interferon, glatirmer lacitate

RadiculopathyForm of neuralgia due to irritationof the spinal nerve

NeuropathyNerve Reflex Motor Deficit Sensory Deficit

C5 Biceps Deltoid, biceps Anterior Shoulder

C6 Brachioradialis Biceps, wrist extensor Lateral forearm

C7 Triceps Triceps, wrist flexors, finger extensors

Posterior forearm

C8 None Finger flexors Forth and fifth fingers, medial forearm

T1 None Finger interossei Axilla

L4 Patellar Tibialis anterior(foot dorsiflexiion)

Medial leg

L5 None Extensor hallucis longus(first toe dorsiflexion)

Lateral lower leg, first web space

S1 Achilles Peroneus longus and brevis( foot eversion)Gastrocnemius(foot plantarflexion)

Lateral foot

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Osteopathic Manipulation

• Numerous studies support the use of OMT in the treatment of patients with musculoskeletal complaints.

• No studies on radiculopathy found.

• Many modalities- should not just think of high velocity techniques.

• High velocity should be used with caution in the cervical spine

Carpal Tunnel

• Compression of the median nerve at wrist

• Ages 30 to 55

• F>M

• H and P

– Wrist pain radiates up the arm

– Decreased grasp

– Numbness in thumb middle and index finger

– Thenar atrophy in advanced cases

+ tinel + phalen

flexor digitorum profundus tendons

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Treatment

• Most of questionable benefit

– Wrist splints

– Activity modification

– NSAID

– Steroid injections

– Surgical release of the transverse carpal tunnel ligament