in service - tirr gh
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Neuromuscular Diseases
Differential Diagnosis and Treatment Ideas
Ashley Spiegel, SPTTexas Woman’s University
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ObjectivesClinicians will be able to...
● Understand pathology and symptoms of various neuromuscular diseases○ GBS○ AIDP○ CIDP○ TM○ ALS
● Prescribe treatments while regarding diagnosis-specific considerations ● Differentially diagnose based on two case studies
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Guillain Barre SyndromeDefinition: rapidly evolving, immune-mediated peripheral neuropathy preceded by a triggering event (infection)1
Symptoms:
● Proximal weakness accompanied by tingling dysesthesias in U/LEs○ LEs more affected than UE
● Pain○ Shoulder girdle, back, posterior thighs○ Most severe at night
● Hypo/areflexic
Diagnosis:
● Electrodiagnostic examination ● Absent DTRs● Elevated protein in CSF● Progressive weakness
● Progression of symptoms over days - 4 wks● Cranial nerve involvement● Autonomic dysfunction
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Guillain Barre Syndrome: Phases● Progressive: worsening symptoms; days - 4 wks
○ 73% reach lowest point of clinical function at 1 wk, 98% at 4 wks○ 30% require ventilatory assistance○ 20% mortality rate
● Plateau: persistent, unchanging symptoms; days
● Recovery: improvement in symptoms and functioning○ 85% achieve full and functional recovery within 6-12 months○ Maximal by 18 months past onset○ Relapse occurs in 3-5% of pts
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Guillain Barre Syndrome: Subtypes1. Acute inflammatory demyelinating polyradiculopathy (AIDP)
2. Acute motor axonal neuropathy (AMAN)
3. Acute motor sensory axonal neuropathy (AMSAN)
4. Miller Fisher Syndrome
5. Acute panautonomic neuropathy
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Chronic Inflammatory Demyelinating Polyradiculopathy Definition: GBS/AIDP, but longer...2
● Sensory loss and weakness progress beyond 4 wks● Not self-limiting or spontaneous - if untreated, 30% will progress to w/c
dependence
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Guillain Barre Syndrome: TreatmentSpecific treatment:
● Intravenous immunoglobulin (IVIg) or plasmapheresis● Gabapentin for pain management
Supportive treatment:
● PT/OT/Speech3,4,5
○ Progressive phase: PROM and positioning, upright tolerance, functional training○ Plateau: energy conservation, A-AROM and AROM exercises○ Recovery phase: aerobic training, RROM
● Psychological stress
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Transverse MyelitisDefinition: neurological disorder caused by inflammation across both sides of one level of the spinal cord; often caused by viral infections, abnormal immune reactions6,7
Symptoms:
● Weakness of U/LEs depending on the level of the lesion● Pain/sensory alteration● Spasticity● Hyperreflexia ● Bowel and bladder dysfunction
Diagnosis:
● MRI/CT● Blood test (to rule out HIV, SLE, B12 deficiency)● CSF (elevated protein and WBCs)
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Transverse Myelitis: Treatments Specific treatment:
● Corticosteroids● Plasma exchange● Pain medications ● Baclofen● Gabapentin
Supportive treatment:
● PT/OT/Speech○ Strength, endurance, coordination, reduce spasticity, equipment training, pressure relief
● Psychological
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Transverse Myelitis: Prognosis● Recovery begins 2 - 12 wks of onset, up to 2 years
○ No improvement within 3-6 months, complete recovery unlikely
● ⅓ good or full recovery ○ Normal gait pattern, minimal urinary or bowel effects and paresthesias
● ⅓ fair recovery ○ Spastic gait, sensory dysfunction, incontinence
● ⅓ no recovery at all ○ Bedridden
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Amyotrophic Lateral SclerosisDefinition: progressive neurodegenerative disease affecting cells in the brain and spinal cord8,9
● Amyotrophy: “no muscle nourishment”● Lateral sclerosis: hardness, or scarring, in the lateral columns of the spinal cord
Symptoms: gradual onset, painless, progressive muscle weakness
● Classic, spinal ALS: muscle cramps/fasciculations (initially), asymmetric muscle weakness and atrophy
● Bulbar onset ALS: dysarthria, dysphagia, sialorrhea● Respiratory onset ALS: respiratory failure
Diagnosis:
● Electrodiagnostic tests● Blood and urine studies
● Spinal tap● MRI
● Muscle/nerve biopsy● Thorough neurological exam
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Amyotrophic Lateral Sclerosis: Diagnostic Criteria4
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Amyotrophic Lateral Sclerosis: TreatmentSpecific treatment4:
● Riluzole (Rilutek)● Drugs for spasticity, fatigue, muscle cramps
Supportive treatment3,4:
● PT/OT/Speech○ Low impact aerobic exercise, ROM, stretching○ Avoid vigorous exercise in later stages
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Case StudiesDifferential Diagnosis
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Patient #1● Female● Mid 50s● Symptoms:
○ LE weakness● Eval:
○ Tone: 0○ Clonus: 0 ○ Bed mobility: max A○ Transfers: max A○ Gait/stairs: does not occur
● D/C: after 10 day stent in rehab○ Bed mobility, transfers: mod I○ Gait: 1000’ RW; ramps, curbs, unlevel surfaces○ Stairs: 3 flights CGA
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Patient #2● Male● Mid 60s● Symptoms:
○ LE weakness → UE weakness○ Bulbar symptoms ○ B ptosis○ Neurogenic bladder
● Eval:○ Strength: 2-○ Tone: 0○ Clonus: 0○ Bed mobility: total A○ Transfers: total A with mechanical lift○ Gait/stairs: does not occur
● D/C: not yet (6 wks since hospital admisison, 19 days in rehab)
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Differential Diagnosis: ALS
Patient #1 Presentation Typical ALS Presentation Patient #2 Presentation
Sudden onset Gradual onset Sudden onset
✓ No pain Severe pain
✓ Progressive muscle weakness
✓
LMN only LMN + UMN signs LMN only
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Differential Diagnosis: TM
Patient #1 Presentation Typical TM Presentation Patient #2 Presentation
✓ LE weakness U/LE weakness ✓
No pain Pain/abnormal sensation ✓
Flaccid Spasticity/hyperreflexia Flaccid
No bowel and bladder dysfunction
Bowel and bladder dysfunction
✓ Neurogenic bladder
Decreased signals MRI Decreased signals
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Differential Diagnosis: GBS
Patient #1 Presentation Typical GBS Presentation Patient #2 Presentation
Stronger proximally Weaker proximally ✓
Inconsistent hypersensitivity/ no pain
Pain/hypersensitivity at shoulders, back, and thighs (severe night pain)
✓
Rapid improvements (1wk), no plateau
Progressive (4 wks), plateau, recovery (6-12 mos)
✓ 6 wks since dx; currently in plateau/recovery
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GBS/AIDP CIDP TM ALS
Preceding infection Preceding infection Autoimmune, preceding infection
Unknown cause
Proximal > distal weakness Proximal > distal weakness Weakness in U/LEs below level of lesion
Asymmetric muscle weakness, fasciculations, atrophy
Pain/hypersensitivity, esp. at night (shoulders, back, thighs)
Pain/hypersensitivity, esp. at night (shoulders, back, thighs)
Sensory alteration below level of lesion
Hypotonicity and/or spasticity
Hypo and/or hyperreflexia
Bulbar signs (dysarthria, dysphagia, sialorrhea)
Bulbar signs (dysarthria, dysphagia, sialorrhea)
Bowel and bladder dysfunction
Bulbar signs (dysarthria, dysphagia, sialorrhea)
Progressive up to 4 wks followed by plateau; maximal at 18 months
Continues to progress beyond 4 wks, not self-limiting
Recovery within 2-12 wks; maximal at 2 years
No recovery, no cure
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References1. Newswanger DL, Warren CR. Guillain-Barré Syndrome. American Family Physician. 2004;69(10):2405-2410.
http://www.aafp.org/afp/2004/0515/p2405.html. Accessed October 4, 2016.2. All About CIDP - GBS/CIDP Foundation International. GBSCIDP Foundation International.
http://www.gbs-cidp.org/cidp/all-about-cidp/. Accessed October 4, 2016.3. Danto A. PT Management of Other Neurological Conditions: MS, ALS, GBS, CNS Tumors. October 2016.4. Das R. Neuromuscular Diseases. June 2015.5. GBS/CIDP Foundation International. Guillain-Barre Syndrome, CIDP and Variants: Guidelines for Physical and
Occupational Therapy. https://www.gbs-cidp.org/wp-content/uploads/2012/01/PTOTGuidelines.pdf. Accessed October 4, 2016.
6. Transverse Myelitis Fact Sheet. U.S National Library of Medicine. http://www.ninds.nih.gov/disorders/transversemyelitis/detail_transversemyelitis.htm. Accessed October 4, 2016.
7. Disease Information - The Transverse Myelitis Association. The Transverse Myelitis Association. https://myelitis.org/living-with-myelitis/disease-information/. Accessed October 4, 2016.
8. Amyotrophic Lateral Sclerosis (ALS) Fact Sheet. U.S National Library of Medicine. http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_als.htm. Accessed October 4, 2016.
9. ALS Association. ALSA.org. http://www.alsa.org/. Accessed October 4, 2016.
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Questions or comments?