Neurology Peripheral Neurology. Alison Ruiz PA-C

Transcription

1 Neurology Peripheral Neurology Alison Ruiz PA-C

2 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

3 History PMH HTN Hyperlipidemia PSH None FH Stroke (mother 76 y/o) MI (father 79 y/o) SH Nonsmoker 2 drinks per week

4 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

5 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

6 ????? Diagnostic Tests

7 Motor Neurons

8 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

9 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

10 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

11 Stroke Differential Diagnosis Shingles

12 Algorithm Acute Facial Weakness Central or Peripheral? Peripheral Herpes zoster oticus Bells Palsy Ramsay Hunt Syndrome MRI Evaluate ischemia, inflammatory, infectious disease CSF Central Consider ESR Admit patient Serologic studies Syphilis, HIV, Vasculitis

13 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

14 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

15 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

16 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

17 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

18 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

19 Diagnosing Consider in all patients with unilateral facial pain Look for red flags of other diseases

20 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

21 Hearing loss Numbness Pain lasting > 2 minutes Pain outside of trigeminal distribution Visual changes

22 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

23 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 last week, but resolved in the past 3 days. No N/V/D. Denies cough, sob, chest pain or abdominal pain

24 History PMH Mononucleosis PSH Hernia repair 10 years ago SH Nonsmoker Drinks alcohol mostly beer 4-6 drinks per week FH HTN both parents

25 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

26 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

27 ? Differential Diagnosis

28 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

29 Guillan Barre Syndrome Demyelinating disease primarily affecting the peripheral nervous system. Slight male predominance. Affects the elderly and young adults most commonly.

30 Guillain-Barre Syndrome Presentation 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 Usually 1-3 weeks after URI Decreased or absent DTR. Ascending Weakness, paresthesias

31 Disease Progression Progressive Phase Lasts few days to 3-4 weeks Plateau Phase Days to weeks Recovery Phase Weeks to months

32 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

33 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%

34 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

35 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

36 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.

37 Treatment of Transverse Myelitis Corticosteroids Plasma exchange Reserved for severe disease

38 Myasthenia Gravis Pathophysiology Bimodal occurring first in 20s and 30s in women and then in the 6 th and 7 th decades of life in men Many etiologies All lead to formation of autoantibiodies directed against nicotinic acetylcholine receptors (AchRs) at the neuromuscular junction

39 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 1 st two years of the disease.

40 Diagnostic Testing Clinical findings and serologic testing Endrophonium (Tensilon) test Electromyography Receptor antibody testing is positive At this point, you have called neurology!!!!!

41 Treatment Cholinesterase inhibitors Pyridostigmine and neostigmine Consult!!!! Used for outpt chronic therapy Immunosuppressant drugs Used for chronic control Thymectomy Immunomodulatory Therapy Plasmapheresis and IVIg are used for severe exacerbation

42 References Tintanilli 1118 Adams , Medscape