320 Parkinson’s Disease CLINICAL FEATURES (CONT’D) DISTINGUISHING FEATURES BETWEEN CEREBELLAR DISORDER AND TABES DORSALIS (see p 244) Cerebellar ataxia Tabes dorsalis Sensory Normal # vibration and proprioception Gait Truncal ataxia Slap foot gait Wide based gait Wide based gait Romberg Positive with eyes closed and open Positive with eyes closed only CLINICAL FEATURES (CONT’D) HISTORY characterize ataxia (truncal or limb, tim ing, progressive), speech changes, vision changes, incoordination, falls, headaches, nausea and vomit ing, weight loss, past medical history (alcohol use, stroke, multiple sclerosis, malignancy, Wilson’s dis ease), medications, family history PHYSICAL nystagmus, ataxic speech (‘‘British constitution,’’ explosive in volume, scanning), hypo tonia, dysdiadochokinesia, finger to nose test (dysmetria), heel shin test, pendular reflex, wide based stance, ataxic gait (wide based and CLINICAL FEATURES (CONT’D) staggering), rebound (outstretched arms swing easily when pushed), pronator drift (upward), trun cal ataxia (Romberg’s test shows unsteadiness with eyes both open and closed) INVESTIGATIONS IMAGING CT/MR head MANAGEMENT TREAT UNDERLYING CAUSE Subacute Combined Degeneration See VITAMIN B12 DEFICIENCY (p 405) Parkinson’s Disease CLASSIFICATION OF MOVEMENT DISORDERS CLASSIFICATION OF MOVEMENT DISORDERS (CONT’D) HYPOKINETIC PERIODIC LEG MOVEMENT OF SLEEP BRADYKINESIA RIGIDITY POSTURAL INSTABILITY PARKINSONIAN SYNDROMES constellation of rest tremor, rigidity, bradykinesia, and loss of pos tural reflexes HYPERKINETIC DYSTONIA/ATHETOSIS sustained muscle contrac tion, causing twisting and repetitive move ments/posture TREMOR oscillations produced by alternating contractions of reciprocally innervated muscles, e.g physiological, essential, intention, rest MYOCLONUS sudden shock like muscle contrac tions, e.g focal, multifocal, generalized CHOREA/BALLISM arrhythmic, rapid, jerky, pur poseless movements Ballismus is large ampli tude, proximal chorea, e.g Huntington’s chorea PSEUDOATHETOSIS chorea type movements sec ondary to sensory loss PAINFUL LEGS AND MOVING TOES continuous, stereotyped, flexion extension, or adduction abduction movements of toe nocturnal myoclonus, with repetitive stereotyped exten sion of big toe RESTLESS LEG SYNDROME abnormal sensation in legs, especially at night ALIEN LIMB complex non volitional movements (reaching, grasping) TICS rapid, non rhythmic movement or sound on background of normal activity STEREOTYPY tardive dyskinesia AKATHISIA motor activity from voluntary effort to relieve uncomfortable sensation, mainly in daytime PHANTOM DYSKINESIA amputees HEMIFACIAL SPASM unilateral contraction of facial muscles involving eyelids, cheek, and corner of mouth , - STARTLE DISEASE OR HYPEREKPLEXIA STIFF PERSON continuous isometric contractions of somatic muscles SYNDROME PATHOPHYSIOLOGY PARKINSONISM wTRAPw any of Tremor, Rigidity, Akinesia/bradykinesia, and Postural instability 321 Parkinson’s Disease PATHOPHYSIOLOGY (CONT’D) Parkinson’s disease is primary or idiopathic parkin sonism Secondary or acquired parkinsonism may be due to head trauma, cerebrovascular disease, drugs, or hydrocephalus PARKINSONISM PLUS SYNDROMES progressive supranuclear palsy, multiple system atrophy, Lewy body dementia, cortico basal ganglionic degeneration CLINICAL FEATURES PHYSICAL EXAMINATION FOR PARKINSON’S DIS EASE resting tremor (4 6/s), rigidity, bradykinesia, micrographia, dementia, stare (reduced blink rate), mask face (hypomimia), glabellar tap, drooling, dys arthria, difficulty getting up from chair, postural instability, difficult with heel to toe walking, shuffling gait, and en bloc turn Associated with disordered sleep, constipation, pain, and depression CLINICAL FEATURES (CONT’D) DISTINGUISHING FEATURES BETWEEN PHYSIOLOGIC AND PSYCHOGENIC MOVEMENT DISORDERS HISTORY abrupt onset, static course, spontaneous remissions (inconsistency over time), obvious psychiatric disturbance, multiple somatizations, healthcare works, pending litigation or compensa tion, secondary gain PHYSICAL inconsistent character of movement (amplitude, frequency, distribution, selective disabil ity), paroxysmal, movements increase with attention or decrease with distraction, ability to trigger or relieve the abnormal movements with unusual or non physiological interventions, false weakness, false sensory complaints, self inflicted injuries, deliberate slowness of movements, functional disability out of proportion to exam findings THERAPEUTICS unresponsiveness, response to pla cebo, remission with psychotherapy RATIONAL CLINICAL EXAMINATION SERIES: DOES THIS PATIENT HAVE PARKINSON’S DISEASE? LR+ LR History Tremor 1.3 17 0.24 0.60 Rigidity 1.3 4.5 0.12 0.93 Difficulty rising from chair 1.9 5.2 0.39 0.58 Loss of balance 1.6 6.6 0.29 0.35 Shuffling gait 3.3 15 0.32 0.50 Difficulty opening jars 6.1 0.26 Difficulty turning in bed 13 0.56 Micrographia 2.8 5.9 0.30 0.44 Physical Tremor 1.3 1.5 0.47 0.61 Rigidity 0.5 2.8 0.38 1.6 Bradykinesia 0.4 0.9 1.67 3.7 Heel to toe difficulties 2.9 0.32 Glabellar tap 4.5 0.13 TESTING glabellar tap (percussion of forehead for $20 times Normally blinking stops after 10 times Persistent blinking suggests positive Myerson sign), bradykinesia maneuvers (tapping finger, twiddling like motor, pinching and circling, tapping with heel) APPROACH ‘‘a combination of tremor, rigidity, bradykinesia, loss of balance, shuffling gait, micrographia, difficulty with turning in bed, opening jars, and rising from a chair should raise suspicion of Parkinson’s disease On examination, the diagnostic value of the classic combination of tremor, rigidity, bradykinesia is limited Useful signs include the glabellar tap, difficulty walking heel to toe and rigidity’’ JAMA 2003 289:3 DISTINGUISHING FEATURES BETWEEN VARIOUS TREMORS Parkinson Essential Tremor Resting Postural (action) Hertz Head direction Up down (‘‘yes’’) Side to side (‘‘no ’) Legs involved Yes Rare Effect of alcohol No change Improved Cerebellar Intention (action) None Yes No change 322 Parkinson’s Disease CLINICAL FEATURES (CONT’D) MANAGEMENT (CONT’D) CHARACTERIZING MOVEMENT DISORDERS SPEED slow (dystonia, athetosis, dystonic tics), moderate (chorea, tremor, asterixis), quick (myo clonus, myoclonic tics) SUPPRESSIBILITY volitional in tics, sensory tricks in dystonia, activity in rest tremor AGGRAVATING FACTORS stress, anxiety Improves with rest and sleep PRECIPITATING FACTORS alcohol, caffeine, stress, fatigue, cold, quick movements, prolonged exercises INVESTIGATIONS SPECIAL IMAGING CT/MR head, particularly if atypical features INVESTIGATIONS FOR HYPERKINETIC MOVEMENT DISORDERS BASIC CBCD, lytes, urea, Cr, glucose, AST, ALT, ALP, bilirubin, LDH, CK, INR, PTT, urinalysis IMAGING CT head, MRI head SPECIAL FURTHER NEUROLOGIC WORKUP EMG/NCS, mus cle/nerve biopsy, lumbar puncture, genetic test ing (CAG repeats), and smear for acanthocytes if suspect Huntington’s disease INFLAMMATORY WORKUP ESR, CRP, ANA, ENA, RF, ANCA, C3, C4, lupus anticoagulant, antiphospho lipid antibody, antistreptolysin O MALIGNANCY WORKUP quantitative immunoglo bulin, serum protein electrophoresis ENDOCRINE WORKUP TSH, PTH METABOLIC WORKUP copper, 24 h urinary cop per, vitamin B12, ceruloplasmin, RBC folate, lac tate pyruvate LABS COMT and MAO B inhibitors entacapone 200 mg with each dose of levodopa, rasagiline 0.5 mg PO daily ANTICHOLINERGICS benztropine 0.5 mg PO BID AMANTADINE amantadine 200 300 mg PO daily APPROACH Sinemet should be first line therapy for most patients because of its effectiveness COMT/MAO B inhibitors or dopamine agonists may be used in combination with Sinemet or as first line agent alone for young patients Anticho linergics have limited activity but can help with tremor and dyskinesia Amantadine may be useful for mild disease and dyskinesia DYSKINESIA a classic complication of Sinemet Consider lowering dose of levodopa, changing its timing/frequency, and replacing part of the levodopa dose with a dopamine agonist Amantadine may be added to counteract dyskinesia SYMPTOM MANAGEMENT GENERAL education, support, exercise, speech therapy NAUSEA domperidone is safe as it does not cross the blood brain barrier Avoid antidopaminergic medications such as metoclopramide and phe nothiazines (prochlorperazine, chlorpromazine) PSYCHOSIS AND HALLUCINATIONS consider stopping anti Parkinsonian drugs in sequence May need to start atypical neuroleptic antipsychotics such as quetiapine or clozapine Avoid older neuroleptic antipsychotics such as haloperidol DEPRESSION antidepressants such as TCAs and SSRIs may be used with caution SPECIFIC ENTITIES GAIT ASSESSMENT MANAGEMENT TREAT UNDERLYING CAUSE SINEMET carbidopa/levodopa 25/100 25/250 mg PO TID Combined use with entacapone can lead to more sustained levodopa levels See NEJM 2008 359:23 for details DOPAMINE AGONISTS bromocriptine 10 mg PO BID, pramipexole, ropinirole, pergolide Ineffective in patients unresponsive to levodopa Type Spastic gait Scissor gait Apraxic/magnetic gait inspect pelvis, knees, ankles, and feet for asymmetry, deformity Ask the patient to walk normally, then heel to toe, walk on heels, walk on toes, and squat FOOT MOVEMENTS heel strike, foot flat (mid stance), heel off (lift off), toes off (swing) GAIT MOVEMENTS comment on pace length, width, coordination, and stability (see table below for specific pathologies) NEUROLOGICAL EXAMINATION lower limb motor and sensory examination Also include Romberg test GENERAL INSPECTION Pathology Upper motor neuron lesion (stroke) Bilateral upper motor neuron disease Frontal lobe (NPH, stroke) 323 Radiculopathy SPECIFIC ENTITIES (CONT’D) Type Shuffling gait Broad based gait Ataxic gait Unsteady, sensory ataxia gait Trendelenburg gait (waddling) Steppage gait Pathology Extrapyramidal lesion (Parkinson’s) Cerebellar vermis Cerebellar anterior (alcohol) Posterior column (Tabes dorsalis, B12 deficiency, Friedreich’s ataxia) Hip adductor muscle weakness (gluteus medius) Foot drop (peroneal nerve palsy) Related Topics Dementia (p 378) Orthostatic Hypotension (p 312) Radiculopathy PATHOPHYSIOLOGY FORAMINAL ENCROACHMENT OF THE SPINAL NERVE usually due to a combination of decreased disc height and degenerative changes of the unco vertebral joints anteriorly and zygapophyseal joints posteriorly COMMONLY AFFECTED NERVE ROOTS CERVICAL REGION C7 (70%) and C6 (20%) are the most commonly affected nerve roots LUMBOSACRAL REGION L5 and S1 (>90% com bined) are the most commonly affected nerve roots Related Topics Back Pain (p 284) Peripheral Neuropathy (p 327) Spinal Cord Compression (p 228) CLINICAL FEATURES HISTORY characterize neck or back pain Paresthe sia, radiation of pain, and weakness over specific nerve root distribution, any associated neurological symptoms Ask about red flags (fever, chills, unex plained weight loss, unremitting night pain, previous cancer, immunosuppression, and IDU) which may suggest tumor or infections SPURLING’S SIGN reproduction of symptoms (e.g pain radiating down arm) with extension and lateral rotation of neck toward affected side followed by compressive force to the top of the head suggests cervical radiculopathy and may facilitate localization Despite popularization in physical examination books NEJM 2005 353:4 CLINICAL FEATURES (CONT’D) and modest diagnostic utility (LR+ 3.6), Spurling’s test should probably not be performed In patients with rheumatoid arthritis, cervical malformations, or metastatic diseases, this test risks serious injury to the spine INVESTIGATIONS IMAGING spine X ray (low sens), CT spine, MR spine (especially if suspect myelopathy, red flags, progressive neurologic deficits, no improvement for weeks) EMG AND NERVE CONDUCTION STUDY TREATMENT OF CERVICAL RADICULOPATHY NON SURGICAL acetaminophen, NSAIDs, opioids, corticosteroid injections, cervical traction, exercise SURGICAL indicated if myelopathy or a combina tion of definite cervical root compression by CT/MRI, radiculopathy symptoms/signs, and persistent pain despite non surgical treatment of 12 weeks or progressive motor weakness SPECIFIC ENTITIES CERVICAL MYELOPATHY diffuse hand numbness and clumsiness (often attributed to peripheral neuro pathy), imbalance, sphincter disturbances (late find ing, urinary urgency/frequency initially, then reten tion or incontinence) Physical findings include hypertonia, hyperreflexia/clonus, positive Babinski, Hoffmann’s (flexion and adduction of the thumb when the examiner flexes the terminal phalanx of the long finger), and Lhermitte’s sign 324 Radiculopathy DERMATOMES Reprinted with permission from Springer Medizin Verlag MYOTOMES Root C3,4,5 C5 C6 C7 C8 T1 T2 12 L2 L3 L4 Muscles Diaphragm Deltoid (shoulder abduction) Biceps and brachioradialis (elbow flexion), radial wrist extensors (wrist extension) Triceps (elbow extension), ulnar wrist extensors (wrist extension), wrist flexors, finger extensors Intrinsic muscles of hand Intrinsic muscles of hand Chest wall and abdominal muscles Iliopsoas (hip flexion) Quadriceps (knee extension), adductor longus (hip adduction) Quadriceps (knee extension), tibialis anterior (dorsiflexion and inversion) ... deformity Ask the patient to walk normally, then heel to toe, walk on heels, walk on toes, and squat FOOT MOVEMENTS heel strike, foot flat (mid stance), heel off (lift off), toes off (swing) GAIT... SPEED slow (dystonia, athetosis, dystonic tics), moderate (chorea, tremor, asterixis), quick (myo clonus, myoclonic tics) SUPPRESSIBILITY volitional in tics, sensory tricks in dystonia, activity... SPURLING’S SIGN reproduction of symptoms (e.g pain radiating down arm) with extension and lateral rotation of neck toward affected side followed by compressive force to the top of the head suggests cervical