101: PERIPHERAL NEUROPATHIES

APPROACH Symmetric vs Asymmetric Proximal vs Distal vs Mixed Sensory vs Motor vs Mixed TYPES OF PERIPHERAL NEUROPATHIES Myelinopathy = damage to the myelin sheath Axonopathy = primarily damage to the axon with or without myelin sheath damage Neuronopathy = damage to the cell body of the neuron itself INVESTIGATIONS Nerve conduction studies and Electromyography (EMG) can distinguish between symmetric, asymmetric, proximal, distal, sensory, motor, mixed, They can identify the level of the problem within the nerve Can determine myelinopathy, vs axonopathy, vs neuronopathy TYPE 1: DEMYELINATING POLYNEUROPATHIES Pattern Symmetric Proximal and distal weakness Motor > sensory loss Guillain-Barre Syndrome General MCC or rapidly progressive weakness MCC of demylinating polyneuropathies Associations: campylobacter jejuni Clinical Features and Presentation Progressive symmetrical weakness of proximal and distal muscles Often starts 1-3 weeks after viral illness Symptoms are usually worse in the lower extremities Absent or decreased DTRs Down going plantars Hypotonia Variable sensory loss May look like cauda equina or conus as B/B dysfunction can occur from autonomic dysfunction 1/3 will require ventilatory support May present with cranial nerve findings (Millar Fisher) Autonomic dysfunction is reflected by fluctuating BPs Diagnosis Elevated CSF protein with near-normal wbc count is specific for GBS in a suspected case MRI enhancement of nerve roots is suggestive Managment Check FEV1 and PEFR to r/o resp insufficiency Intubation for severe resp weakness or fatigue, very poor FEV1, hypoventilation

Plasmapharesis IVIG Both work and combination is not better Do not treat blood pressure

TYPE 2: DISTAL SYMMETRIC POLYNEUROPATHIES Pattern Distal and symmetric Sensory and motor loss Wose int eh lower extremities Stocking and Glove distribution Sensory loss occur before motor loss and loss of DTRs Diabetic DSPN MC type of peripheral neuropathy in ED Tingling and burning are the MC initial complaints Numbness, weakness, sensory loss as it progresses Plantar surface of the feet lose sensation before the dorsal aspect Weakness of great toe dorsiflexion is first motor sign Foot drop, loss of ankle jerks, then steppage gate Sensory loss moves proximally and starts in the fingers before it reaches the knees; progressive reflex loss is seen Sensory gait ataxia may occur b/c of loss of JPS Mx: Amitriptyline 10 - 25 mg qhs for neuropathic symptoms Alcoholic DSPN Unknown if its due to alcohol or nutritional deficiencies Similar to diabetic polyneuropathy Ataxia compounded by cerebellar vermis degeneration Toxic DSPN: many (Arsenic, mercury, gold) Drug DSPN: many (inh, dilantin, flagyl, dapsone) Nutritional DSPN: many (B1, B6, B12) Porphyrias Other

TYPE 3: ASYMMETRIC PROXIMAL AND DISTAL PERIPHERAL NEUROPATHIES (RADICULOPATHIES AND PLEXOPATHIES) Pattern Asymmetric Proximal and distal Sensory and motor loss Brachial Plexopathy Lumbar Plexopathy Thoracic Outlet Syndrome upper extremity pain and paresthesia secondary to vascular or neurological compression of the subclavian vessels or the brachial plexus Osseous: cervical rib, thoracic rib abnormality, large C7 transverse process, clavicle anomaly, clavicle # Non-Osseous: hypertrophic muscle, tumor, , vascular anomaly, inflam Mechanical : hyperabduction (pec minor), dislocated humeral head pain, numbness, and tingling are the MC complaints, motor wkness rare Adson maneuver: pt sits w/ hands on knees, inhales deeply, extends the head and turns the chin to the affected side. This produces diminution or obliteration of the radial pulse as the scalenus muscle compresses the subclavian artery Shoulder Depression Hyperabduction Dx: CXR, CT chest, EMGs and NCS Radiation Plexopathy Note this can develop up to 20+ years after radiation TYPE 4: ISOLATED MONONEUROPATHIES Pattern Asymmetric Distal Sensory and motor loss Radial neuropathy Median neuropathy Ulnar neuropathy Sciatic neuropathy Lateral Femoral Cutaneous neuropathy Common Peroneal Neuropathy Carpal Tunnel Syndrome

CARPAL TUNNEL SYNDROME MEDIAN NEUROPATHY Epidemiology - Most common nerve entrapment syndrome - 2:1 females; 50% b/w 40-60 - associated conditions: RA, hypothyroidisms, DM, local wrist trauma, pregnancy, acromegaly, multiple myeloma, amylioidosis (Look for theses b/f Sx!!!!!!!) Etiology - carpal tunnel is a bony trough covered by flexor retinaculum and contains 9 tendons each covered w/ 2 synovial layers :. NO room for expansion - causes fluid retension is MCC (pregnancy is mcc) overuse of tendons is MCC referred to orthropods synovial thickening (RA, Colles #, etc) History - parathesia in front of thumb, index + middle + of ring finger - palm is SPARED b/c the palmar branch of the median n arises above the wrist (Sparing of palmar cutaneous br of median n. which supplies thenar eminence) - s/s worse at night, and aggravated by driving, reading, and holding a telephone, mopping, sweeping - parathesia replaced w/ pain proximally as far up as the shoulder - eventually numbness occurs in the median distribution - motor symptoms devp late (thenar atrophy, clumpsy thumb and 1st finger) Physical - Most sensitive sign: abnormal sensation of the index finger tip - Most specific sign: splitting of the ring finger sensation - Tinels sign: tap over median n. produces irritation - Phalens test: wrist flexion for 1min causes hands to go to sleep - Tourniquet test: cuff > SBP below elbow produces tingling w/i 60sec - 2 point discrimination > 4mm - thenar atrophy - motor testing: LOAF muscles L: lumbricals: flexion of MCP of digits 1,2,3 O: opposition: Abductor Oppones Pollicis (thumb towards pinky) A: abduction: Abductor Pollicis Brevis (BOOM) F: flexion of the thubm - dx confirmed w/ nerve conduction studies if necessary -*Be suspicious if all fingers involved b/c median doesnt supply any of ring f** Ddx - peripheral neuropathy, mononeuritis, cervical spondylosis, thoracici inlet tumors involving the brachial plexus - how to differentiate? Treatment - Night splint in extension - surgical decompression - surgical indications: persistence a/f 3 injections; NCS grossly abnormal

PRONATOR TUNNEL SYNDROME MEDIAN NEUROPATHY Median nerve compression in the forearm as it passes b/w the heads of pronator teres

History Physical Treatment paresthesia (tingling) and numbness in thumb, index, middle, and radial of ring finger pain and fullness in the proximal forearm on the palmar surface pain increased by resistance to pronation occassional radiation of pain into axilla, neck frequently a subjective feeling of weakness of grip tenderness, firmness, enlargement of pronator muscle tinels sign: tingling/numbness a/f percussion or compression of pronator muscle (percuss a few cm.s below the antecubital fossa b/w the radius and ulna) pronation against resistance produces paresthesia NO wkness of the median nerve innervated intrinsic or extrinsic mm NSAIDs, graded and garded activity, immobilization surgical decompression

CUBITAL TUNNEL SYNDROME ULNAR NEUROPATHY Compression in the cubital tunnel or condylar groove at elbow Most Common entrapment of the ulnar nerve Trauma, tumors, congenital, leaning on elbows (Tardy ulnar palsy) Anatomy ulnar nerve passes thru the cubital tunnel as it goes from arm to forearm cubital tunnel can be palpated behind and beneath the medial epicondyle of the humerus History sharp or aching pain in proximal forearm tingling or numbness of ring and little fingers especially w/ repetitive activities dorsal hand sensory loss on the ulnar side tells you that the dorsal cutaneous branch is involved and the lesion is proximal to the canal of guyon wkness and loss of dexterity Physical ulnar claw and atrophy of intrinsics and hypothenars weekness of flexor carpi ulnaris and flexor digitorum profundus to the little finger tells you that the neuropathy is at the elbow dinosaurs and froments sign tinels sign: percussion test over the ulnar nerve at the elbow elbow flexion test: flexion increases pressure on nerve and produces s/s Cubital tunnel vs compression of the ulnar nerve at the wrist Splitting of the ring finger sensation Ulnar cutaneous innervation branches before the Guyons canal thus a lesion at the wrist should not produce sensory abnormalities while one at the elbow should Treatment Avoid repetitive flexion/extension of elbow, dont rest elbows on objects Surgical decompression if needed ULNAR COMPRESSION at the WRIST Anatomy

ulnar nerve travels thru the canal of guyon (not the carpal tunnel) the dorsal branch of the ulnar nerve is not a component of the canal of guyon it arises proximal to canal it supplies sensory innervation to dorsal ulnar aspect of hand sparing -------> lesion w/i canal of guyon involvement ------> lesion proximal to canal of guyon

Etiology

ganglia (MCC), lipoma, anomolous muscles, thick ligaments, #s, repetitive trauma, following burns, arthritis History/Physical wrist pain that radiates to little and ring fingers numbness, burning, tingling of little and ring fingers wkness, wasting of hypothenars, interossei, 4th and 5th lumbricals, and adductor pollicis longus longstanding compression may lead to the ulnar claw (extension of MCP, and flexion of the PIP) Dinosaur sign inability to abduct or adduct fingers b/c of weak interossei which are all supplied by the ulnar nerve Froments sign weakness of adductor pollicus (ulnar) thus the pt uses flexor pollicus longus (median) to pinch a piece of paper pinch paper test Tinels and Phalans sign Allans test of ulnar artery

SATURDAY NIGHT PALSY: HUMERAL RADIAL NEUROPATHY Compression of the radial nerve as it winds around the humerus in the spiral groove on medial side in axilla Etiology - axillary crutches, sleeping in chair, arm along back of chair, humeral #, sleep with arm underneath you Presentation - DROP WRIST b/c of wkness of wrist extensors (all supplied by radial) - weak finger extensors - may be sensory loss of back of hand at base of thumb - NOTE: triceps is spared b/c its innervation comes off the radial b/f compressn - Triceps involvement = axillary radial neuropathy (uncommon) instead of humeral Treatment/Prognosis - hold wrist up in a sling - remyelination @ 2mm/d, axonal regeneration can take a long time if damaged POSTERIOR INTEROSSEOUS Radial nerve bifurcates in antecubital fossa

- posterior interosseuos: pure motor - superficial radial nerve: pure sensory radial nerve vulnerable as it winds around radial neck does NOT run b/w radius and ulna, runs on interosseous membrane MCC is trauma wkness of wrist and finger extension (sensation loss rare) Treatment - Sx only reqd for mechanical entrapment or irritation by bone fragments

SCIATIC MONONEUROPATHY Sciatic nerve includes L4-S3 spinal nerve roots Sciatic nerve divides into the common peroneal and the tibial nerve at the knee Traumatic lesions: penetrating buttock injury, buttock hematoma, deep im injection Complete sciatic neuropathy is devasatating: total loss of sensory motor below the knee and loss of hamstring function

LATERAL FEMORAL CUTANEOUS MONONEUROPATHY Meralgia Paresthetica Sensory nerve Compression as the nerve passes through the inguinal ligament Numbness and dyesthesia over the upper lateral thigh Very common: obesity, diabetes, HIV Inguinal ligament release can be done if very severe COMMON PERONEAL NERVE Anatomy sciatic nerve splits into tibial and common peroneal curves around fibular neck then splits into superficial and deep only covered by skin and subQ tissue at this vulnerable site superficial peroneal nerve: foot everters and lateral foot sensation deep peroneal nerve: dorsiflexors of foot/toes, 1st webspace sensation Causes Idiopathic: often occurs during sleep position Leg crossing Casts Fractures Penetrating truama Presentation foot drop b/c of weak tibialis anterior (steppage gait) weakness of evertors and toe extensors pain over lateral aspect of legs

Treatment

paresthesias in lateral leg or dorsum of the foot compare to a L5 radiculopathy if the invertors are weak it may be a sciatic neuropathy or L5 radiculopathy depends on cause penetrating trauma should have exploration and repair posterior splint to keep foot at 90 degrees if there is a foot drop observation for incomplete lesions surgical exploration may be necessary

TARSAL TUNNEL SYNDROME Compression of the posterior tibial nerve w/i the tarsal canal Tarsal canal is created by the distal tibia behind the medial malleolus and is covered by the flexor retinaculum Etiology gangilon, cyst, lipoma, tumor, varicosities, tenosynovitis History burning, tingling, numbness on the plantar aspect of the foot often aggravated by activities relievd by mvmt, rubbing the foot or soaking the foot Physical Tinels sign look for lump over medial ankle

TYPE 5: MONONEUROPATHY MULTIPLEX Pattern Asymmetric Distal Sensory and motor DTRs of affected nerves are decreased Diabetes: most common cause, likely vascular, different than DSPN Vasculitis: another common cause, PAN, RA, SLE, sjogrens Lyme dz: early and late stages; begins with radiculitis; spontanesous rsolution over months TYPE 6: AMYOTROPHIC LATERAL SCLEROSIS ALS is a type of Motor Neuron Disease ALS requires the presence of both upper and lower motor neuron findings is in the middle of the specturm between primary lateral sclerosis of the SC and progressive muscular atrophy Neuronopathy of the anterior horn cell is the primary problem Purely motor signs b/c the problem is proximal to where the motor and sensory fibers

merge together Asymmetirc idstal weakness without sensory findings Positive motor findings: fasciculations in almost all but weakness is initial complaint ALL need NCS and EMGs to r/o treatable condidtions

SENSORY NEURONOPATHY (GANGLIONOPATHY) Predominant loss of the dorsal root ganglion Pure sensory syndrome Typically distal and asymmetric but progresses to be symmetric Changes can be seen on MRI Many causes: HSV, shingles, mercuruy, etc