Nicola Sutton

Motor Neurone Disease(MND) includes a spectrum of clinical disorders of the upper and lower motor neurons. Characterised by progressive, degeneration and loss of motor neurons in spinal cord, brain stem or motor cortex
(Rowland 1982, Swash 2000)

4/5 in 100,000 in UK have MND Men: Women, 2:1

(MNDA, 2009)

1.

Amyotrophic Lateral Sclerosis (ALS 65% (ALS) Progressive Bulbar Palsy (PBP) 25% Progressive Muscular Atrophy (PMA)10% Primary Lateral Sclerosis (PLS) very rare
(NHS, 2009)

2.

3.

4.

Involves both Upper and Lower Motor Neurons

Muscle Weakness

Spasticity and contractures

Hyperactive reflexes Emotional Lability Fasciculation Weight Loss (Walton et al, 1993)

ALS Anterior horn cells degenerate Anterior & lateral columns of Motor neurons degenerate in spinal cord harden this area Motor neurons degenerate in this area Replaced by fibrous astrocytes (gliosis)

Muscle fibres denervated

Atrophy of muscle fibres

Amyotrophic Lateral Sclerosis

(adapted from Sheehan et al, 2002)

Electromyography (EMG) Nerve conduction tests Laboratory screening tests Muscle or nerve biopsy Transcranial magnetic stimulation (TMS) MRI
(MNDA, 2010)

(Brooks 1994)

(ACTS, 1996)

 Mobility & dexterity, ADLs

Function

Pain

Muscle shortening, pain, postural effects Decrease spasms, orthotic wearing Care & hygiene Positioning, dressing

Carer burden

(Sheehan, 2002)

(BMJ, 2009)

Modified Ashworth (MAS)

Brashear et al (2002) Naghdi et al (2008)

Tardieu Scale

Haugh et al (2006) Mehrholz et al (2005)

Ordinal scale of tone intensity 0-5 Proven reliable @ elbow

Ordinal scale of tone that measures the intensity of the muscle reaction @ specific velocities V1,2,3

Watanabe (2004) reported clinicians have used massage, cooling, FES but stretching is the most researched Stretching found to be extensively used in practice as its safe, convenient and economical (Tsai et al, 2001).

De Jong et al, 2006 Pilot RCT PEDro = 8 19 post stroke pts (sub acute, 12 wks) 36-63 yrs Intervention: All pts. had conventional rehab 9 pts. had additional positioning (x2) 30 mins p/day, 5/7, 5/52 Measures: PROM Resistance to PROM elbow using Ashworth Scale Secondary outcomes pain, independence in ADLs Outcome: Additional positioning slowed down development of shoulder abduction contracture STRENGTH standardisation of PROM Interrater reliability of measurement protocol explored Therapists used checklist after each session LIMITATION Sample size was small Sampling method of convenience Positioning carried out by several diff nurses and physios

Ada et al, Prospective RCT PEDro = 7 36 post stroke pts (sub acute), ~68 yrs Intervention: Exp.gp (x2) 30min sessions, 5/7, 4/52 in 2 positions Measures: Contracture measured as max.passive ext shoulder rotation and flexion of affected side vs.intact side Measured at 2, 6 wks Outcome: Exp gp. reduction in development of contractures, Ext.rotation range improved STRENGTH Measurements and positioning carried out at diff times, reduces short term effects of intervention First study to show significant effect of positioning on reduction in contractures LIMITATION 8 patients were discharged during the intervention and while it was noted in the discussion, they were included in the results Slings and supports provided, interference?

Pizzi et al, 2005 Pretest Posttest trial 40 post stroke pts (4 months) Intervention: Patients wore immobilising hand splint custom fitted in func.position for 90mins day, 3/12 Measures: Spasticity at elbow- MAS PROM at wrist and elbow Pain at shoulder, elbow, wrist using VAS Comfort and time worn Outcome: Wrist PROM improved, reduction in elbow spasticity, well tolerated STRENGTH Increased internal validity Written instructions given to pts LIMITATION Sacrificed external validiy Results cant be generalised Pts self reported wearing time

Gracies et al, 2000 Crossover trial 16 pts, subacute stroke (>3wks) 36-85 yrs Intervention: Assessment at start and end of 3 hr period during standard rehab day on, day off Measures: Comfort, Spasticity at shoulder, elbow and wrist using Tardieu scale, AROM,PROM Outcome: Garments were comfortable, improved wrist posture and reduced wrist and finger flexor spasticity, Improved PROM at shoulder STRENGTH Used Tardieu Scale Splint fitted by same trained investigator each time Took into account effects of time and learning LIMITATION Convenience sample of pts Small sample size Plastic boning only in the hand splint

Studies De Jong Ada Exp (9) vs Control (8) p=0.042 23 degrees vs -5.3 degrees Exp, development of contractures p = 0.03, no significance of the shoulder being in 90flexion Shoulder ext.rotation range sig dif in exp (p=0.05) PROM wrist improved (p=.001) Reduction in elbow spasticity (p=.002) Wrist pain (p=0.04) RIS was well tolerated Spasticity in wrist and finger extensors reduced (p<0.05) PROM improved at shoulder (p<0.01) Only one to use Tardieu scale

Pizzi

Gracies

Ada L, Goddard E, McCully J, Stavrinos T, Bampton J (2005) Thirty minutes of positioning reduces the development of shoulder external rotation contracture after stroke: A Randomised Controlled Trial, American Congress of Rehabiliation Medicine 86 Adams RD, Victor M, Ropper AH, eds.(1997) Principles of neurology. 6th ed. New York: McGrawHill, 1089-94. Anand P, Parrett A, Martin J, Zeman S, Foley P, Swash M, et al. (1995) Regional changes in ciliary neurotrophic factor and nerve growth factor levels in the post mortem spinal cord and cerebral cortex from patients with motor disease. Nature Med 1:168-78. Belsh JM, Schiffman PL, eds. (1996) Amyotrophic lateral sclerosis: diagnosis and management for the clinician. Armonk, N.Y.: Futura, 41-3. Belsh JM, Schiffman PL. (1996) Amyotrophic lateral sclerosis: diagnosis and management for the clinician. Armonk, N.Y.: Futura, 286-90. Bensimon G, Lacomblez L, Meininger V. (1994) A controlled trial of riluzole in amyotrophic lateral sclerosis. New England Journal of Medicine 330:585-91. Brasher et al (2002) Inter- and intrarater reliability of the Ashworth Scale and the Disability Assessment Scale in patients with upper-limb poststroke spasticity, Archives of Physical Medicine and Rehabilitation 83(10) 1349-1354 Brooks BR (1994) El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/ Amyotrophic Lateral Sclerosis of the World Federation of Research Groups on Neuromuscular Diseases and the El Escorial Clinical Limits of amyotrophic lateral sclerosis workshop contributors. Journal of the Neurological Sciences 124:96-107

Brooks BR (1996) Clinical epidemiology of amyotrophic lateral sclerosis. Neurology Clinics 14:399-420. Brooks BR. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. J Neurol Sci 1994;124(suppl):96-107. Costigan DA. Motor neuron disorders. In: Hurst JW, ed. Medicine for the practicing physician. 3d ed. Boston: Butterworth-Heinemann, 1992:1642-3. De Jong LD, Nieuwboer A, Aufdemkampe G (2006) Contracture preventative positioning of the hemiplegic arm in subacute stroke patients: a pilot randomised controlled trial, Clinical Rehabilitaion 20: 656-667 Drachman DB, Kundel RW.(1989) Amyotrophic lateral sclerosis: an unconventional autoimmune disease? Ann Neurol 26:269-74. Gracies JM, Marosszeky JE, Renton R, Sandanam J, Gandevia SC, Burke D (2000) Short Term Effects of a Dynamic Lycra Splint on Upper Limb in Hemiplegic Patients, Archives of Physical and Medical Rehabilitation 81: 1547-1555 Haugh A.B, Pandyan A.D, Johnson G.R (2006) A systematic review of the Tardieu Scale for the measurement of spasticity, Disability & Rehabilitation 28 (15) 899-907 Kolde G, Bachus R, Ludolph AC. (1996) Skin involvement in amyotrophic lateral sclerosis. Lancet;347: 1226-7. Kurtzke JF.(1991) Risk factors in amyotrophic lateral sclerosis. Advances in Neurology 56:245-70.

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Mehrholz J, Wagner K, Meisner D, Grundmann K, Zange C (2005) Reliability of the Modified Tardieu Scale and the Modified Ashworth Scale in adults with sever brain injury : A comparison study , Clinical Rehabilitation 19(7) 751-759 Mitsumoto H, Chad DA, Pioro EP. (1998) Amyotrophic lateral sclerosis. Philadelphia: Davis, Naghdi S, Ansari N.N, Azarnia S (2008) Interrater reliabiltity of the Modified Ashworth Scale (MMAS) for patients with wrist flexor muscle spasticity, Physiotherapy Theory and Practice 24(5) 372-379 Naghdi S, Ansari NN, Azarnia S, Kazemneiad A (2008) Interrater reliability of the Modified Ashworth Scale for patients with wrist flexor muscle spasticity, Physiotherapy Theory Practice 24(5): 372-379 Oliver D. Motor neurone disease. 2d ed.(1994) London: Royal College of General Practitioners, 1-16. Physician's desk reference. (1997) Riluzole. 51st ed. Montvale, N.J.: Medical Economics, 2200. Pierson S.H (1997) Outcome measures in spasticity management, Muscle & Nerve 6 S36 Pizzi A, Carlucci G, Falsini C, Verdesca S, Grippo A (2005) Application of a Volar Static Splint in Poststroke Spasticity of the Upper Limb, Archives of Physical Medicine and Rehabilitation 86 Rowland LP (1982) Diverse forms of motor neuron disease. Advances in Neurology 36:1-13 Rowland LP (1991) Ten central themes in a decade of ALS research. Advances in Neurology 56:3-23.

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Rowland LP (1994) Riluzole for the treatment of amyotrophic lateral sclerosis--too soon to tell? New England Journal of Medicine ;330:636-7. Sheehan G (2002) The pathophysiology of spasticity' European Journal of Neurology. 9 (1):3-9 Swash M, Desai J (2000) Motor neuron disease : Classification and nomenclature. ALS and other motor neuron disorders 1(2) 105-12 Swash M, Schwartz MS.(1992) What do we really know about amyotrophic lateral sclerosis? Journal Neurology Sci 113:4-16. The ALS Ciliary Neurotrophic Factor Treatment Study (ACTS) Phase I-II Study Group. (1996)The amyotrophic lateral sclerosis functional rating scale: assessment of activities of daily living in patients with amyotrophic lateral sclerosis Archives of Neurology 53:141-147 Walton JN, ed.(1993) Brain's Diseases of the nervous system. 10th ed. New York: Oxford University Press, 443-9. Watanabe T (2004) The Role of therapy in spasticity management, American Journal of Physical Medicine and Rehabilitation 83(suppl):S45-S49. Williams DB, Windebank AJ. Motor neuron disease (amyotrophic lateral sclerosis). Mayo Clinic Protocol Wokke JH.(1996) Diseases that masquerade as motor neuron disease. Lancet 347:1347-8.