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Neuromuscular diseases leading to respiratory failure
Jiann-Horng Yeh, M.D.
Department of Neurology
Shin Kong WHS Memorial Hospital
Respiratory muscles
Muscle groups AHC level
Diaphragm C3-5
Intercostal muscles T1-12
Scalene
Sternocleidomastoid
Trapezoid
C4-8
Cranial XI, C2-3
Cranial XI, C2-4
Abdominal muscles T7-L1
Neurological signs for D/D
Level DTR Bulbar EOM Sensation ANS
UMN +/— +/— +/— +/—
AHC +/— — — —#
Nerve + +/— + +
NMJ N + + — +/—
Muscle N +/— — —* —
* Pain in polymyositis# ANS s/s in Bulbar poliomyelitis
Common disorders of NMD
UMN NMJ Muscle
BS/cord lesion Myasthenia gravis Dystrophy
LMN LE syndrome Polymyositis
Poliomyelitis Botulism Trichinosis
ALS OP poisoning Endocrine myopathy
Nerve Tick paralysis
GB syndrome
Brainstem lesions Stroke Extrinsic compression Intrinsic tumor Encephalitis MS, CPM Motor neuron disease
Spinal cord lesions Cord compression Motor neuron Dz, Polio Intrinsic tumor MS, Myelitis Rabies
Sedative drugsMetabolic disorderCentral transtentorial herniation
CNS disorders causing RF
Neuropathy with RF - IGBS NCV PE, IVIG
CIDP NCV PE, Steroid, IVIG
Critical illness — —
Lymphoma N biopsy Cytotoxic
Vasculitis-LE N biopsy Steroid, Endoxan
Porphyria U/porphobilinogen IV hematin
Diphtheria Throat swab Antitoxin
H.tyrosinemia U/d-ALA High calorie intake
Liver transplant
Toxic neuropathy with RF
O-phosphate RBC C-esterase
P/pseudoC-esteraseAtropine
Pralidoxime
Thallium Blood level Berline blue
Arsenic 24h Urine level Dimercaprol, DMSA
Lead Blood level Na-Ca edetate, above
Gold — Na-Ca edetate, above
Lithium Plasma level Hemodialysis
Vincristine — Withdrawal
General hints in initial symptomsVomitingAltered level of consciousness
ThalliumProminent cutaneous & muscular pain, esp. feetPreserved DTR in the early stage
Clues for toxic neuropathy
NM disorders with RF
Myasthenia gravis Tensilon test, AchRAb PP, Steroid
AC overdose Tensilon test — Withdrawal
Antibiotics — Withdrawal
Hypermagnesemia Plasma level, RNS IV calcium
Botulism Antibody, RNS Antitoxin
Poisoning * Identification Antitoxin
Tick paralysis Find the tick Removal
LE syndrome Increment on RNS PP, steroid
* snake, scorpion, spider, fish, shellfish, crab
Muscle disorders with RF Hypokalemia Plasma level K+
Polymyositis CPK, EMG, biopsy Steroid
Rhabdomyolysis CPK, EMG, biopsy Urine alkalinization
Hypophosphatemia Plasma level Phosphate
Acid maltase def. PAS stain (PB film)
—
Barium intoxication Plasma K+ IV K+
Mg sulfate, po
Hemodialysis
Differential tests
Physical and neurological examinationsLaboratory tests
Electrophysiology: NCV, RNS, EMG, SFEMGCPK, electrolyte, thyroid functionAntibody titerCSFBiopsy: nerve, muscle
Provocative test: Tensilon test
General Management in ICU
Orthopnea, interrupted speechShallow & rapid respirationParadoxical respirationBreathing sound
Reduced BS, sputum, crackle
Arterial blood gasHypoxemia, CO2 narcosis, respiratory acidosis
AIRWAY MANAGEMENT Evaluate s/s of impending respiratory
failure
Criteria for intubationVC<15 mL/kg; Pimax < -25 cmH2O
Paired VC test – supine & sitting positionNormal: Supine VC > 80% Sitting VCWeakness: Supine VC < 40% Sitting VC
Digit count at one breathCount <25: VC < 20 mL/kg
AIRWAY MANAGEMENTMonitor the changes of pulmonary
function
Chest physical therapyPercussion, postural drainage
Education for effective respiration/coughingElective intubation
Impaired swallowingSigns of aspiration pneumoniaHypoxemiaCritical level of lung function
AIRWAY MANAGEMENTAppropriate chest care
Prevention of complications
Careful posturing: entrapment neuropathyFrequent turn: bedsoresPassive exercise: disuse atrophyNG feeding: aspirationHeparin or pneumatic leg compression: DVTVital sign monitoring: ANS instabilityEmotional support: anxiety, depression
Guillain-Barré syndrome
Guillain-Barré Syndrome(Acute inflammatory demyelinating polyneuropathy)
Acute/subacute motor paralysis for days/wksArefelexia or hyporeflexiaMild sensory symptoms or signsCSF: albuminocytological dissociation
NCV: conduction slowing or block
Pathology Focal segmental demyelination Inflammatory cells infiltration
Clinical courseCourse
Progression: 4 wk (90%)Plateau: 4 wk (85%)Recovery: 4-6 months (80%)
OutcomePermanent residua: 15%Permanently disabled: 5%Mortality: 2-5%Relapse: 3%
Clinical features - I
Spectrum: mild ataxia to total paralysis
Limb involvement Leg onset: arm & face are possible Proximal > distal involvement Symmetric pattern Absence of DTR even in minimally involved m.
External urethral sphnicter 10-20% retention > incontinence
Clinical features - IICranial N involvement
VII: asymmetric: 50% (esp. upper lip/perioral)EOM: 10% Isolated cranial N: 5%Oropharngeal involvement: 40% (herald of
impending respiratory failure)Respiratory muscle involvement
Major cranial N involvement frequently associatedWeakness of shoulder elevation & neck flexion
parallels diaphragmatic weakness & resp. failure
Clinical features -IIIAutonomic involvement: 65%
Sinus tachycardia: > 50%SIADH, (DI)Orthostatic hypotension (20%) & hypertensionSweating disturbanceCardiac dysautonomia may correlate with
sensory dysfunction ( Raphael JC, 1986 )Muscular of neuropathic pain: 30-55%
follow vigorous exercise (chaley horse)distributed in thigh, buttock & low back
73 F AIDP onset: May 27,1996
0
1
2
3
4
5
6
7
1 2 3 4 5 6 7 8 9 10 11 12 14 16 18 20 22 24 26 28
GradeMRC-sum score *10
ANSANS
IntubationIntubation
GBS 之處置呼吸道 : 插管
肺活量監控 , VC<15mL/Kg, Pimax < -20mmHg輸液 : N/S 2L/ 天營養 : 腸道營養。如有腸堵塞才改靜脈營養特殊處置
IVIG ( 免疫球蛋白 ), 0.4g/Kg/ 天 x 5 天 PE ( 血漿交換 ) 隔日一次 x 5 次 如使用呼吸器或同時使用 aspirin/NSAID 病人 ,
投予 Sucralfate 10mL bid. 肌肉疼痛可投予肌肉注射類固醇
Plasmaphresis in Neurology
Disease Definition Class
GBS Established I
CIDP Established I
MS - acute; refractory to steroid Established I
MGUS - IgG/A Established I
MG – preop & crisis Established III
MGUS - IgM Investigational I
Lambert-Eaton syndrome Possibly useful II/IIIInvestigational: Refsum disease, acquired neuromyotonia,
Stiff-man syndrome, Cryoglobulinemic neuropathy, CNS lupus, ADEM
GBS Study Group : PE vs No TxNeurology 1985,35,1094-1104
245 patients; 40-50 cc/kg for 3-5 PE
Parameters PE No Tx p
Improve > 1 grade at 4 wks 59% 39% *
Mean grade change at 4 wks 1.1 G 0.4 G **
Median time to improve 1 G 19 D 40 D **
Median time to walk unaided 53 D 85 D **
Median time on ventilator 24 D 48 D *
Failed to improve 1 G at 6M 3% 13% *
Plasmapheresis appears to be of benefit in patients with GBS of recent onset (within 7 days).
Change of MRC-sum score during plasmapheresis in GBS
Chen et al; J Clin Apheresis 1999;14:126-9.
32.6
45
0
50
Pre-PP Post-PP
Score
Author Y Country No G at entry
Time to Tx
Osterman 84 Sweden 18 4.6 6.9GBS study 85 USA 122 4.3 11.1
French 87 France 109 ND 6.3Van der Meche 92 Neth 73 3.9 5.6
Bril 96 Canada 24 4.1 4.7PES/GBS 97 UK 121 3.9 6.9
SKH 98 Taiwan 16 3.6 8.1
Plasmapheresis in GBSChen et al; J Clin Apheresis 1999;14:126-9.
Plasmapheresis in GBSChen et al; J Clin Apheresis 1999;14:126-9.
Author 1G at W4(%)
G at W4
Time to G2
Fail to G2 at M6
OFF respirator
Osterman 2.1 GBS study 59 1.1 53 21 9
French 70 18 Van der Meche 34 0.4 69 22.6
Bril 61 1 PES/GBS 1.1 40 13.7
SKH 81 1.5 19 13 11.2
GBS 病情惡化之成因
病情持續惡化病情穩定後再復發 (relapse)自律神經異常 (dysautonomia)
好發於急速癱瘓且合併眼肌麻痺者血壓不穩心律不整
呼吸器相關之併發症
Myasthenic Crisis
Onset - MG
Presenting symptoms Ocular (50%): ptosis; diplopia Weakness (35%): bulbar; leg; arm Fatigue (10%)
Progression: generally insidious over wks to months Aggravating factors
Systemic disease: infection, thyroid Emotional stress Pregnancy Medications
Clinical pattern - MGOcular
Ptosis & ophthalmoplegia
Usually asymmetric & bilateral
Bulbar Dysarthria, dysphagia,
weak mastication Complicated with
aspiration pneumonia Facial: > 95%
Respiratory failure Life-threatening Etiology
diaphragmatic & intercostal muscle weakness
vocal cord paralysis
Systemic Typical: symmetric
Proximal > distal Arms > legs
Selective weakness Posterior neck Occasional distal
Severity classification of MG
Osserman/71 Drachman/82
Grade 1 Ocular Focal
Grade 2 a: Mild generalized
b: Severe generalized
Mild generalized
Grade 3 Acute fulminating Severe generalized
Grade 4 Late severe Crisis
Diagnosis - MG
3 mainstays of diagnostic testing Pharmacological (Tensilon test)Serological (acetylcholine receptor antibody)Electrodiagnostic (RNS & SFEMG)
Diagnosis a characteristic history /PE two positive diagnostic tests, preferably
serological and electrodiagnostic.
2 mg IV observation for 2 min 8 mg IVA positive test requires objective improvement in
muscle strength. Most myasthenic muscles respond in 30 to 45
seconds after injection Sensitivity: 60%False positive results in patients with LES, ALS or
even localized, intracranial mass lesions
Edrophonium (Tensilon) test
Repetitive Nerve Stimulation
Electric stimulation 6-10 times at 2 or 3 Hz. Positive: (R1-R5) /R1>10% Sensitivity:
75% (generalized MG) 50% (ocular MG)
False positive Lambert-Eaton syndrome Motor neuron diseases
Single Fiber EMG (SFEMG)
Rationale increased variability of the latencies at which the muscle fibers
innervated by an individual axon
Sensitivity: 95% in both generalized and ocular MG the test site includes facial muscles.
False positive Lambert-Eaton syndrome Motor neuron diseases Polymyositis
Clinical significance of AchRAb
Diagnostic aidMonitor the clinical statusEvaluate the efficacy of immune therapiesAssess the functional capacity of
plasmapheresis column quantitatively
Adults with generalized MG: 85 to 90% Childhood MG: 50% Ocular MG: 50% to 70%MG with thymoma: nearly 100% Some patients taking penicillamine +/- MG Thymoma without MG Immune liver disordersLambert-Eaton syndrome (13%)Primary lung cancer: 3%Older patients (> 70 years): 1% to 3% Neuromyotonia
AchRAbPositive
AchRAbFalse +
AchRAb titers in Osserman stages (n=699)
56.980.2 88.8 96.3 100
0%
20%
40%
60%
80%
100%
I-348 IIa-243 IIb-80 III-27 IV-1
<0.2
<0.5
>0.5
Thymoma: 10% to 15% Mostly in MG patients > 30 years AChRAb 95% to 100%
Hyperplasia:60% to 80% Younger age groups Female HLA: B8 & DR3
Atrophy: 20% Usually > 50 years
Thymus relationship - MG
重症肌無力危象之處置呼吸道 : 插管
吞嚥困難 , 咳嗽乏力 , 吸入性肺炎 , VC<15mL/Kg
疑有肺炎時 , 先投予第 3 代 Cephalosporin
輸液 : N/S, 發燒時加量營養 : 嚴重吞嚥障礙時 , NG 餵食特殊處置 :
使用呼吸器時 , Pyridostigmine 停用 血漿交換 , 隔日一次 x 5 次 IVIG, 0.4g/Kg/ 天 x 5 天 大量類固醇 1mg/Kg, 視情況而定
Characteristic % no
Pneumonia/pneumonitis 40 8
Bronchitis 30 6
URI 5 1
Sepsis 15 3
Surgery 10 2
No obvious precipitant 15 3
Precipitants (n=20)Yeh et al; Acta Neurol Scand 2001; in
press
Drugs interfere NM transmission
Variety Drug
Antibiotic Aminoglycoside, Fluoroquinolone, Tetracycline, Sulfonamide, Penicillin, Macrolide, Lincomycin, Colistin, Polymyxin, Quinocrine, Chloroquine
CNS Transquillizer, Barbiturate, Anticonvulsant, Lithium, Mg salt, TCA, Haloperidol
Anesthesic Halothane, Ether, Trichloroethylene
CV B-blocker, Verapamil, Quinidine, Procainamide
Others Narcotic, Penicillamine, Iodinated contrast
0.3
0.5
4
6
10
12
18
24
Plasmapheresis
PP+steroid
Steroid
CTX+steroid
Cyclophosphamide
Azathioprine
AZA+steroid
Thymectomy
Time to produce a 50% AchRAb (m)
Tindall RSA/1982
Plasma Exchange - MGDose: 5 exchanges over 9 to 10 days Indications:
Acutely ill MG patient Pre-thymectomy (respiratory/bulbar involvement)
Advantages Very short onset of action (3 to 10 days) Probably more effective in crisis than IVIG
Disadvantages Requires specialized equipment & personnel Complications more frequent in elderly High cost with short-term effects (weeks)
Double-filtration plasmapheresis
PlasmaBlood Purified P
Clinical responseYeh et al, Acta Neurol Scand 1999;100:305-9
Poor16%
Fair62%
Good22%
Poor Fair Good0: 2 2:12 5:31: 5 3:8 6:2
4:8 >:5
Clinical response: plasmapheresis
Dau-81 60 PE 74%
Fornasari-85 33 PE 61%
Mantegazza-87 37 PE 87%
Antozzi-91 70 PE 70%
Kornfeld-92 43 PE 91%
Author-year no Method Response
Shibuya-94 20 IAP 55%Yeh-99 45 DFP 84%
Change of MG score during DFP
9.9
4.2
0
5
10
Pre-PP Post-PP
Score
Change of AchRAb titer during DFP
1st2nd
3rd4th
Filtrate
Blood
0.670.56
0.440
0.2
0.4
0.6
0.8
1
Session of plasmapheresis
0.780.71
0.61
Pulmonary function tests during DFP
1st2nd
3rd4th
Vital capacity
Pimax1.37 1.55 1.85
0
0.5
1
1.5
2
Session of plasmapheresis
1.40 1.49 1.86
Favorable prognostic parameters
Yeh et al, Acta Neurol Scand 1999;100:305-9
High MG scorePathology of non-thymoma typeYoung age at onsetDaily apheresisHigh removal rate for IgG
Clinical response of DFPYeh et al; Acta Neurol Sin 1995;4:107-12.
1 0.70.53 0.47
0
0.2
0.4
0.6
0.8
1
Response rate Effective duration > 2Months
Dyspnea groupNon-dyspnea group
Botulism
Botulinum Toxin
Clostridium botulinum Gram positive bacilli Spore producingAnaerobic: obligate
Botulinum Toxin Sequence homology (30% to 40%) to tetanus toxinProduced as a protoxin withMW 150 kDa7 types of neurotoxin: A-G
Clinical Features - Botulism
Type A Most common outbreaks in Rocky Mountains & West Ca++ level in synaptosomes overcomes blockade More severe & long-lasting paralysis: 67% intubation
Type B Most common outbreaks in East, especially Allegheny Has most structural homology to tetanus toxin Require assembled intracellular microtubule for action Somewhat less severe paralysis than Type A
Time course Incubation period: average: 18 to 38 hoursExtremes: 2 hours to 1 week
Weakness Diffuse; Usually symmetric; Proximal > distal Bulbar: dysphagia; dysarthria Extraocular: ptosis; EOM weakness
Sensory loss: never prominent
Tendon reflexes: reduced
Clinical Features - Botulism
Cholinergic ANS involvement
Pupils: dilated, blurred vision
Bradycardia; hypotension Skin: Hypohydrosis
Urinary retention Gastrointestinal Nausea & vomiting with contaminated food Constipation: first sign, especially in infants Diarrhea may occur early
Diagnosis - Botulism
Analysis of serum, feces & implicated food Passive transfer of serum/body fluid to mice
Toxicity to miceSelectively prevented by anti-toxin
Stool or wound culture
Foodborne Botulism
FoodContaminated with spores in anaerobic conditionsHome canned vegetable/potato & preserved sea food
ToxinResistant to proteolysis in stomach
AbsorptionAlkaline pH of intestine dissociates toxin from
proteins Absorption into circulation
Usually adults
Foodborne Botulism in Taiwan
1986; 9 cases (2 fatalities) in Chang-Hwa cityType A foodborne botulismCanned peanuts from a unlicensed canneryMalaise, ptosis, diplopia, dysphagia, dysarthria and
proximal weakness Implications
Poor governmental supervision of canned food Inadequate quantities of orphan drug stored Inefficient system for recalling the problem products Delayed broadcasting of warnings to the public
Wound Botulism
Toxin is produced locallyDrug abuse: most common cause IM or SC heroin for subcutaneous abscesses (50%)
Incubation: 4-14 D, longer when wound is debrided Onset: blurred vision & bulbar weakness
Progression Generalized weakness Dysarthria; dysphagia Pupillary reactivity
Botulinum types A > B
Treatment - Botulism
Supportive care: respiratory; wound debridement Early Emetics: avoid magnesium containing Lavage Enemas: not when paralytic ileus
Antitoxin Most useful in 1st 24 hours Use on clinical diagnosis Lowers fatality rate & shortens illness (Type A) Complications (immune): 9%
? 3,4-diaminopyridine
Prognosis - Botulism
Ventilator dependence frequency Type A > B > E
Slow improvement in strength over weeks to months
1 year: Most near normal ± fatigue
ANS function may improve later than weakness
Mortality: 5% to 10%
Prevention - Botulism
Canning or preserving foods with appropriate heat, pressure, & low pH Spores
Survive 2 hr at 100 °C; inactivated at 120 °C
Factors favoring spore germination: Low acidity (pH > 5.0); Low O2; High water content
Toxin: inactivated 1 min at 85 °C, or 5 min at 80 °C Avoid exposure of infants to honey (may contain
Clostridium botulinum spores)
Periodic Paralysis
HyperkalemicHereditary (AD)
Na+ channel (SCN4A)17q35
Hypokalemic Hereditary
Ca++ channel (CACNA1S)1q31
K+ channel (KCNE3)11q13-14
Na+ channel (SCN4A)17q13
Distal RTA (ASLC4A1)17q21-22
ThyrotoxicAcquired: K+ wasting
Periodic Paralysis
Hereditary Hypokalemic PP
l L-type Calcium Channel, a1 subunit (CACNA1S) 1q31: R528H; R1086C; R1086H; R1239G; R1239H
AD inheritance penetrance: M 100%; F 50%
Onset: early childhood to 30's; 60% < 16 years Attacks begin in early morning hours
Weak truncal muscle; spared cranial nerves Duration of attack: hours to days Triggers: carbohydrate-rich meal; cold Permanent weakness: often develops over years
Diagnosis - HOPPLaboratory
Serum CK ; K+ during attacks Electrodiagnostic
CMAP during attacks Amplitude after sustained maximal contraction Progressively (40%) during rest 20-40 min after initial
(80% of patients)Provocative test: Glucose ± insulin Muscle pathology
Vacuoles: clear; central and tubular aggregates Myopathy: varied mf size; split fiber; internal nuclei Angular muscle fibers
K wasting syndrome – urinary loss
Alkaline urine & metabolic acidosis HyperaldosteronismAngiotensin converting enzyme dysfunctionLicorice intoxicationMineralocorticoid excessRenal tubular acidosis
Sjögren's, Fanconi's syndrome
Alkaline urine & azotemia: Amphotericin B
Acidosis Ammonium chloride ingestionUreterocolostomies: bilateralDiabetic coma: recoveryRenal tubular necrosis: recovery Distal renal tubular acidosis
Other Gossypol toxicity (with low K+ diet) Tea: excessive amounts
K wasting syndrome – urinary loss
Non-tropical sprue Laxative abuse Severe diarrhea or vomiting Draining GI fistula
K wasting syndrome – GI loss
Thyrotoxic Periodic Paralysis
Incidence Asians: ~ 2%; North America: 0.1%
Male predominance (83% to 95%) Onset: 20 to 40 years; Proximal weakness Weakness
Duration of episodes: hours to days Distribution: legs > arms; proximal > distal Severe attack may involve resp/bulbar function
Sphincters not involved
Clinical features - TPP
Attacks Often occur in random pattern Precipitating factor: carbohydrate challenge; muscle
cooling; rest after exercise Single or multiple episodes
Abate when thyrotoxicosis resolves Systemic
Thyrotoxicosis: may be subclinical ± Cardiac arrhythmias
Diagnosis & Treatment - TPPLaboratory
Usually hypokalemia; occasionally normal Hypophosphatemia: occasional Renal: retention of Na+ & K+; oliguria
Electrophysiology CMAP reduced during attacks
Muscle pathology Vacuolar dilation of sarcoplasmic reticulum
Treatment Correct thyrotoxicosis b-adrenergic blocking agents
Polymyositis
Polymyositis
Muscle weakness Proximal > distal; symmetric Selective: dysphagia, post.neck; quadriceps
Onset age: usually > 20 years Progression: months Pain
30%; especially with connective tissue disease R/O: polymyalgia; arthritis; fasciitis;
rhabdomyolysis
PM associated disordersCardiac
Arhythmias Inflammatory cardiomyopathy
Pulmonary Respiratory muscle weakness, 4% for initial feature Interstitial lung disease
Esophageal paresis Upper 1/3 with muscle weakness Lower 2/3 with scleroderma
Malignancy: mild increased risk Autoimmune: Lupus; Sjögren's; APAS; thyrotoxicosis
Respiratory involvement in PM
Interstitial lung diseaesAspiration pneumoniaAlveolar hypoventilationVentilatory insufficiency
PM-RF Case 1: 61MSelva-O’Callaghan et al, Spain, Rheumatology
2000;39:914-6
Progressive girdle & neck weakness for 1 MParadoxical dysphagiaCPK: 1494 IU/l; ESR: 48 mm/hEMG & muscle biopsy: confirmedHypercapnic respiratory failure at D3Tx: Prednisone 1mg/kg/d, IVIG, Cyclosporin 150 bid
Extubation 20 days later
PM-RF Case 2: 43FSelva-O’Callaghan et al, Spain, Rheumatology
2000;39:914-6
18 y/o: diagnosed PM (EMG, biopsy) 34 y/o: wheelchair bound (P+A treatment)39 y/o: acute URI precipitate resp. failure
PO2: 40 mmHg, PCO2: 68 mmHgTracheostomy with home ventilator
Stable status with normal ABG at homePO2: 83 mmHg, PCO2: 45 mmHg
Diagnosis - PM
Serum CK: High (3 to 30 X ) EMG: Irritative myopathy
Small amplitude, brief, polyphasic motor units Fibrillations; positive sharp waves
Antibodies: disease specific & non-specific Muscle biopsy
Variation in size of muscle fibers Necrosis; phagocytosis & regeneration of fibers Mild, patchy increase in endomysial connective tissue Inflammation: endomysial & perivascular Focal invasion of non-necrotic muscle fibers
Classification of PM
Idiopathic Proximal weakness; CK; inflammatory myopathy
Collagen vascular disease Myalgias; scleroderma & MCTD
Anti-t-RNA synthetase antibodies;Jo-1 antibodies Interstitial pneumonitis; Raynauds; arthritis
Signal recognition particle antibody Acute onset; severe weakness
MAS antibody Acute onset; rhabdomyolysis
Drug-induced: D-penicillamine
Familial: Homozygosity at HLA-DQA1 locus Graft-vs-host disease: 7 to 24 months post BMT Granulomatous: sarcoid; immune; infection Malignancy (necrotic)
Rapid onset; older patients; necrotic myopathy Mitochondrial (P-COX)
Quadriceps weakness; steroid resistant; Age Other systemic disorders: HIV; fasciitis
Classification of PM
Treatment - PMCorticosteroid
Oral Prednisone 100 mg q.d.; latency: 1 to 6 months Solumedrol (iv): Fewer side effects than oral prednisone
Azathioprine 2.5 - 3mg/kg/day; for prednisone dose Latency: 6 to 12 months
Methotrexate 7.5 to 22.5 mg/wk; 1 or 2 doses on weekends Latency: 3 to 6 months
Cyclosporine Starting dose: 2.5 mg/kg b.i.d Latency: 2 to 6 months
Case Demonstration
73 MProgressive malaise, acronumbness for 4 daysER: 970814
144/92mmHg, PR 92/min, RR 14/minNo edema or dehydrationQuadriplegia/malaise: UE:3/LE:0Generalized areflexia [Na]: blood 121 mEq/L, urine 155 mEq/LOsmolality: blood 260 mosm/kg, urine 716 mosm/kg
PH: ASD, gout 4565238
Clinical course
970817 in MICU: respiratory failureHIV: negativeComplement & ANA: WNLSerum protein electrophoresis: No M-proteinCEA, AFP, CA 19.9 & CA 125: WNLCXR: no pneumonic patch
Nerve conduction study
D14 DML CMAP NCV F-wave
Median 14.9/16.5 0.7/0.6 37.0/31.3 41.7/—
Ulnar 6.7/6.1 0.4/0.3 17.4/39.8 —/ —
Peroneal 5.8/7.3 0.8*/1.3* 39.3/44.0 —/ —
Tibial 7.0/8.2 0.7/0.3 38.6/42.4 —/ —
*: conduction blockAll SAPs were absent.
0
10
20
30
40
50
60
70
9 12 15 17 19 21 23 25 27 29 1 3 5 8 10
MR
C s
umsc
ore
MRCGrade
2
4
6SIADH
8/ 9/
120
125
130
135
140
15 16 19 21 22 25 28 1 4 5 8
Na
QOD * 5
1000 1500 1000
60 cc/hr 40 cc/hr
Lasix mg/d
N/S cc/hr
Water restriction cc/d
Plasmapheresis
UNa 155 / Uosm 716 UNa 165
40
55 F
Acute worsening of dyspnea on 8/10, 1996Present illness
General weakness, SOB, dysphagia for 2 monthsBW loss 20 kg/2 monthsCathay General Hospital
Severe restrictive lung diseaseGastric erosion (PES)
Past history: DM for 5 years
NE & ABG
Neurological examClear consciousnessNo ptosisEOM: OKMP: 3/3DTR: ++/++
ABG 8/10 8/11
pH 7.366 7.185
PO2 166.3 113.5
PCO2 58.0 88.0
HCO3 33.4 33.4
SaO2 99.4 96.7
Blood gas
Chest PA
Laboratory tests
Glu(pc)
435 T3 54.8 CPK 402
Ketone + T4 6.8 AchRAb 46.01
Cr 0.7 TSH 0.14 ESR 15/hr
Na 145 Hb 16.5 EF 55%
K 3.8 Platelet 263K LA 52mm
Osmol 309 WBC 9200 EKG NSR
Mediatinal CT
Contrast
Recommended