Neurology Peripheral Neurology

Alison Ruiz PA-C

Case Presentation 50 y/o M presents with c/o rt facial droop. Woke this

morning and his wife noticed it. Pt denies other symptoms. No slurred speech, no weakness, no dizziness. Pt states his upper lip feels heavy and he can’t close his rt eye very well.

No previous episode

No F/C/N/V/D. But states had a fever and cough approx 2 weeks ago

No other complaints

History

• HTN • Hyperlipidemia

PMH

• None

PSH

• Stroke (mother 76 y/o) • MI (father 79 y/o)

FH

• Nonsmoker • 2 drinks per week

SH

PE Vitals: T99.0, HR 86, BP 140/80, R 14, Pulse ox 99%

HEENT: PERRL, EOMi, rt sided facial droop, asymmetry in smile. Loss of nasolabial fold Unable to lift rt eyebrow

Neck supple full rom. No carotid bruits

Lungs: CTA bilat

CV RRR S1S2

Abd: soft NT ND NABS

Ext: no C/C/E

PE cont Neuro Speech clear. No focal deficits. Muscle strength 5/5 and equal bilat throughout- UE and

LE. Blow out cheeks test: Buccinator function is disrupted Unable to raise eyebrow. Loss of nasolabial fold and

nasal flaring Negative for expressive or receptive aphasia Patellar tendon, brachioradialis, triceps, achilles tendon

2+ bilat. Pronator Drift WNL, Rhomberg WNL, finger to nose WNL

Diagnostic Tests ?????

Motor Neurons

Bell’s Palsy The pathophysiology is not clear Theories Inflammation and edema of the nerve due to infectious

processes, leading to nerve compression Ischemic mononeuropathy due to disturbance in the

circulation in the vasa nervorum (the arterial branches supplying the nerve), leading to edema from the subsequent ischemic neuritis

The most common cause is idiopathic

What causes Bell’s and Who is at risk?

Common triggers include

Stress, trauma, fever, tooth extraction and “chilling” episode from exposure to the drafts and cold

Common diseases associated with the Bell’s

DM, HTN, HIV, Sarcoidosis, Sjogren’s, parotid nerve tumors, eclampsia, amyloidosis and recipients of intranasal influenza vaccine

Bells Palsy Peripheral VII nerve palsy (Facial Nerve) • Symptoms include • Ipsilateral tongue numbness, • Loss of taste, • Ear pain, • Overt paralysis preceded by a sensation of numbness or

weakness on affected side, Tinnitus, Drooling, Inability to keep liquids in the mouth, Occipital headache

Physical findings • Loss of ability to wrinkle nose • raise eyebrow, blow out cheeks • unable to purse lips allowing food, liquids and air to

escape • loss of nasolabial fold and nasal flaring

Differential Diagnosis Stroke

Shingles

Algorithm Acute Facial Weakness

Central or Peripheral?

Peripheral Herpes zoster oticus

Bells Palsy

Ramsay Hunt Syndrome

Central

MRI Evaluate ischemia,

inflammatory, infectious disease

Consider

CSF

ESR

Serologic studies Syphilis, HIV, Vasculitis Admit patient

Treatment for Bell’s Palsy

Prednisone • Start within 2-14 days of onset • 1mg/kg/day • Usually 7 days

Valacyclovir 1000mg bid for 7 days or Famvir 750mg tid for 7

days

Wear an eyelid patch at night to prevent drying out the

cornea

Trigeminal Nerve (V)

3 Branches • Ophthalmic nerve V1 • Maxillary nerve V2 • Mandibular nerve V3

Mixed motor and sensory nerve. • Motor innervation for the muscles of

mastication • Sensation from the face, scalp, conjunctiva,

globe, mucous membranes of the sinuses, tongue, teeth and part of the external TM

Trigeminal Nerve Dysfunction Presentation Pain, paraesthesias, dysesthesias, and anesthesias

Motor dysfunction typically presents as difficulty chewing or difficulty swallowing

Peripheral Lesions

Cause loss of sensation or pain in only one division

Central Lesions

Consider if positive findings in 2 or more divisions

Trigeminal Neuralgia

Tic Douloureaux • Slight predilection in women • Increased incidence >60 years of age

Mechanism • Compression of the trigeminal nerve root

within millimeters of the entry into the pons

Presentation

Sudden onset of pain • Paroxysms of severe unilateral pain

in the distribution of the trigeminal nerve

Last only seconds, with normal findings on neurologic exam • There is no pain between paroxysms

Causes Vascular compression by artery or vein

Saccular aneurysm

AV malformation

Vestibular Schwannomas

Meningioma

Epidermoid cyst

Tumor

Primary demyelinating disorders •MS •Charcot-Marie Tooth (rare)

Trigeminal Amyloidoma

Small infarct or angioma in the brainstem

Famillial

Diagnosing

Consider in all patients with unilateral facial pain

Look for “red flags” of other diseases

Abnormal Neuro exam • Think MS, Tumor,

hemmorhage, stroke

Abnormal oral, dental, or ear exam

Age < 40 yrs • unusual Bilateral SXs

Dizziness or vertigo • BPV, Stroke, Dehydration,

Labrinythitis

Hearing loss Numbness

Pain lasting > 2 minutes

Pain outside of trigeminal distribution

Visual changes

Treatment for Trigeminal Neuralgia

Carbamazepine 100mg PO bid and then increased in dosage as needed Treatment is very effective If fails, then the pt is unlikely to have trigeminal neuralgia

Case Presentation 30 y/o M presents to the ER with weakness in his lower

extremities at the ankle area bilat. States started 3 days ago but he is having trouble walking now. Today developed tingling in thighs bilat. He was recently diagnosed with mononucleosis and has been home in bed for 2 weeks.

Fevers 102.0 last week, but resolved in the past 3 days. No N/V/D.

Denies cough, sob, chest pain or abdominal pain

History PMH Mononucleosis

PSH Hernia repair 10 years ago

SH Nonsmoker Drinks alcohol mostly beer 4-6 drinks per week

FH HTN both parents

Physical Exam Vitals: T 99.5 po, R 18, Pulse 89, Pulse ox 100%, BP

120/68

HEENT: PERRL EOMi

Neck supple full rom. No nuccal rigidity

Lungs: CTA bilat. NO W/R/R

CV RRR S1S2

Abd: soft slight RUQ tenderness

Ext: no C/C/E

Neuro Physical exam Neuro Motor weakness bilat LE 3/5 plantar and dorsiflexion, flexion

and extension. Motor weakness bilat UE 4/5 with hand grasp, flexion and

extension of biceps bilat. Otherwise normal 5/5 strength Difficulty getting out of chair. Gait is limited to standing, unable to step without

assistance. Sensory with light and sharp touch equal and intact bilat LE and

UE. CN II-XII grossly intact Finger to nose WNL Rhomberg difficult to assess because patient has trouble

standing

Differential Diagnosis ?

Diagnostic Testing in ER CBC

14,000 WBC Otherwise normal

BMG WNL

LFTs Elevated Alk phos

ESR and CPK Sed Rate elevated Normal CPK

Urinalysis- WNL

LP Protein 500

Guillan Barre Syndrome Demyelinating disease

primarily affecting the peripheral nervous system.

Slight male predominance.

Affects the elderly and young adults most commonly.

Guillain-Barre Syndrome Presentation

Usually 1-3 weeks after URI

Ascending Weakness, paresthesias

Decreased or absent DTR.

Distribution • Distal involvement with

symmetric paresthesias • Spreads proximally with

weakness presenting a few days later moving to the UE. • Weakness is most prominent

in the LE with proximal muscle involvement

• Sensory symptoms are minimal

Disease Progression Progressive

Phase • Lasts few

days to 3-4 weeks

Plateau Phase

• Days to weeks

Recovery Phase

• Weeks to months

Work up LP will show elevated protein levels >400 Normal protein levels do not rule out the GBS

ESR and CPK may be elevated with myopathies

LFTs are elevated in some patient with demyelinating disease

Nerve Conduction studies are common

MRI is sensitive, but nonspecific, for diagnosis. However, it can reveal nerve root enhancement and may be an effective diagnostic adjunct

GB Management Largely Supportive

Should have a respiratory evaluation of FVC

Admitted to the ICU if the FVC < 20mL/kg or intubated if it is less than 15mL/kg.

Plasmapheresis and IV immunoglobin can reduce recovery time by 50%

A Few Points about Transverse Myelitis

May present by itself Or as symptomatology of

Multiple Sclerosis

Paraparesis, which is initially flaccid and then spastic Preceded by back pain

The thoracic cord is most often affected

Transverse Myelitis Resembles Guillain-Barre,

But it is asymmetric involvement

Has definite sensory level Complete lack of UE

involvement Urinary incontinence

symptoms CSF pleocystosis make the

dx of GB less likely

Transverse Myelitis Diagnostics

The spinal cord MRI will almost always confirm the presence of a lesion within the spinal cord, whereas the brain MRI may provide clues to other underlying causes, especially MS.

Treatment of Transverse

Myelitis Corticosteroids

Plasma exchange Reserved for severe disease

Myasthenia Gravis Pathophysiology • Bimodal occurring first in

20s and 30s in women and then in the 6th and 7th decades of life in men

• Many etiologies • All lead to formation of

autoantibiodies directed against nicotinic acetylcholine receptors (AchRs) at the neuromuscular junction

Myasthenia Gravis Muscular weakness and

fatigability are the hallmarks

Ocular muscle weakness is one of the first signs In up to 40% of patients Ptosis, diplopia and blurred vision

If bulbar muscle involvement then may have dysarthria and dysphagia

Acute crisis occurs in 15-20% of patients and presents as respiratory failure. Usually occurs in the 1st two years of the disease.

Diagnostic Testing Clinical findings and serologic testing

Endrophonium (Tensilon) test

Electromyography

Receptor antibody testing is positive

At this point, you have called neurology!!!!!

Treatment Cholinesterase inhibitors

Pyridostigmine and neostigmine Used for outpt chronic

therapy

Immunosuppressant drugs Used for chronic control

Thymectomy

Immunomodulatory Therapy Plasmapheresis and IVIg are

used for severe exacerbation

Consult!!!!

References Tintanilli 1118

Adams 995-997, 1043-1050

Medscape http://emedicine.medscape.com/article/315632-overview