Case Study #2

1. Case Study #2 ALS (Amyotrophic Lateral Sclerosis) Lou Gehrig’s Disease Elis Conejo Stacy Murphy October 7, 2008

2. Examination… • Mr. Richards • Speech therapist • Lives with wife and 1 y/o son • Lives in 2-story home • MOI: choked on piece of meat at school, then noted weakness later (picking up son off floor, going up stairs, and getting up from sofa)

3. Examination continued… • Cranial Nerve Testing: • All CN’s N except: CN XII – Hypoglossal: Tongue Fasciculation's • Deep Tendon Reflexes: • UE – increased • LE – increased • Cognition/Language/Memory: • A&O x 3 • Language: receptive, but slurs occasionally, which is abnormal for this speech therapist • Memory - intact

4. Examination continued… • UE and LE Motor Testing: • Extreme weakness at distal mm’s (feet and hands) • Pt is stronger at core, but pt states that there is “still decreased STR than N.” • MMT of UE and LE = 3/5 • UE and LE Sensory Testing: • All sensory intact = discriminative touch, light touch, crude touch, temperature, pin prick

5. Examination Continued… • MM Extremity Tone: N • Posture: WFL • Coordination: • Due to recent loss of motor STR, pt has difficulty with coordination tests • Balance: • Due to weak LE, unable to maintain balance, but proprioception and kinesthesia still intact lying supine

6. Follow-up Exam (2 weeks later): • Cranial Nerve Testing: N except for continued tongue fasciculation's • Deep Tendon Reflexes: Increased (Hypereflexia) • + Babinski Reflex • Cognition/Language/Memory: N • UE and LE Motor Testing: decreased (2/5)

7. Follow-up Exam (continued): • Posture: Due to drooping head, beginning to become lordotic in order to keep eyes level • Coordination: Continuing to regress • Balance: Continuing to regress • Tone: Spastic Extremities • UE and LE Sensory Testing: N

8. Tract Problem • Corticospinal- Demyelinaton in the lateral uncrossed ventral tract. Related to axonal degeneration of large myelinated fibers • Rubrospinaland Corticobulbar- Similar to Corticospinal • Vestibulospinal- loss of brain stem control of the tract • Spinocerebellar- 80% of Familial ALS cases have degeneration of the tract

9. Pathology continued… • Adult onset, progressive motor neuron disease affecting the anterior horn cells and the corticospinal tracts • Eventually leads to paralysis and death • No known cause or cure • LMN and UMN involvement • Lou Gehrig: • Yankee • 36 y/o • 2 year battle

10. Pathology continued… • Pt is demonstrating more UMN deficits (UE extensor and LE flexor weakness, spasticity, increased reflexes, + Babinski) than LMN (tongue fasciculations) • Regardless of whether the initial dz involves upper or lower motor neurons, both categories are eventually implicated

11. Pathology continued… • LOWER MOTOR NEURON EFFECTS: • Asymmetric weakness of the distal aspect of one limb progressing to weakness of the muscles • Extensor muscles become weaker than flexor muscles, especially the hands • Flaccid facial muscles • Fasciculation's in the tongue • Dysarthria, and difficulty with swallowing

12. Pathology continued… • UPPER MOTOR NEURON EFFECTS: • Loss of dexterity; Spascisity • Extensor muscles of the upper extremity and flexor muscles of the lower extremity are weakened • + Babinski • Clonus • Hyperactive tendon and muscle stretch reflexes

13. Evaluation: Disablement Model • Pathology- Adult onset progressive motor neuron disease affecting the anterior horn cells and corticospinal tracts • Impairment- Degeneration- scaring of the motor neurons in the lateral aspect of the spinal cord, brain stem, and cerebral cortex • Functional Limitation- Pt is unable to lift son, climb stairs, get up from sitting • Disability- Pt can’t play with son, get to bedroom, or work as speech therapist

14. PT Dx: • APTA Practice Pattern: • 5E- Impaired motor function and sensory integrity associated with progressive disorders of the central nervous system. • 6B- Impaired aerobic capacity/endurance associated with deconditioning • 6E- Impaired ventilation and respiration/gas exchange associated with ventilatory pump dysfunction or failure (acute pneumonia) • Pt is referred to a PCP for a definitive dx

15. Dx of ALS: • Electrodiagnostic tests including electomyography (EMG) and nerve conduction velocity (NCV) • Blood and urine studies including high resolution serum protein electrophoresis, thyroid and parathyroid hormone levels and 24 hour urine collection for heavy metals • Spinal tap • X-rays, including magnetic resonance imaging (MRI) • Myelogram of cervical spine • Muscle and/or nerve biopsy • Thorough neurological examination

16. PT Goals • LTG 1: Pt will be able to walk up 12 stairs with ankle braces in 2 wks in order to get to his bedroom STG: Pt will walk up 6 stairs with ankle braces in 1 wk in order to have more control of his ankles (dorsiflexion)

17. PT Goals • LTG 2: Pt will be able to transfer from his walker to his couch with no assistance in 1 wk in order to maintain full independence in his home environment • STG: Pt will be able to transfer from walker to couch Min assist in 2 days in order to be able to sit down and get up from couch

18. PT Prognosis • The course of ALS is relentlessly progressive. Death from the adult-onset type occurs within 2 to 5 years. Resulting mainly from pneumonia caused by respiratory compromise. The lack of ability to eat is another part of the final stages of ALS. • The prognosis is NOT good.

19. PT Intervention • Phase 1 (Independent)- ROM exercises (stretching, yoga, tai chi). Strengthening program with gentle resistance. Deep breathing exercises. Use of adaptive equipment • Phase 2 (Practically Independent)- Active assisted PROM. Isometric contractions. Motorized chair fitting • Phase 3 (Dependent)- Palatal lifts. • Transfer training, Pt and caregiver education