Assessment of Motor Neuron Disease PDF

Assessment of Motor Neuron Disease Prof.Nahed Ahmed Salem Professor of Physical Therapy for Neurology Cairo University Presented by Mohamed Nagy Elshafey Mohamed Abdallah Elkaffas Mohamed Bayomy Mohamed Definiton: It...

Assessment of Motor Neuron Disease Prof.Nahed Ahmed Salem Professor of Physical Therapy for Neurology Cairo University Presented by Mohamed Nagy Elshafey Mohamed Abdallah Elkaffas Mohamed Bayomy Mohamed Definiton: It is a degenerative disease of a gradual onset and progressive course, affecting the motor system only (systemic disease). Clinical picture : Age of onset: usually the middle and old ages Sex: male are more affected the female Onset and course: gradual onset and progressive course Signs and symptoms: are usually bilateral, depend on the type of affection  Types : 1. Upper motor neuron affection (U.M.N) 2. Lower motor neuron affection(L.M.N) 3. Combined UMN and LMN affection.. Upper motor neuron affection: In the spinal cord : Called syndrome of (lateral sclerosis), symptoms are bilateral can be : i. Below the cervical region : results in spastic paraplegia ii. At the level of cervical region : results in spastic quadriplegia In the brain stem or the cerebral hemisphere : Called syndrome of (pseudo-bulbar palsy) manifested by: I. Bulbar symtoms II. Quadriplegia III. Exaggerated palatal and pharyngeal reflexes IV. Emotional and mood changes may be present V. Exaggerated jaw reflex if the lesion above pons Lower Motor Neuron affection It can affect (A.H.C) in the spinal cord or the cranial nerve nuclei in the brain stem I. In A.H.C : Syndrome of (progressive muscular atrophy) Resulting in signs of L.M.N.L: I. Wasting II. Hypotonia III. Weakness IV. Hyporeflexia and fasciculations V. Symptoms start in upper limbs then lower limbs II. In cranial nerve nuclei : Syndrome of (true bulbar palsy) manifested by: I. Bulbar symptoms II. Absent palatal and pharyngeal reflex III. Tounge is wasted and show fasciculations Combined U.M.N and L.M.N affection Syndrome of (Amyotrophic lateral sclerosis ) There are combind signs and symptoms of U.M.n.L and L.M.N.L In the upper limb: I. weakness, wasting and fasciculations (L.M.N) II. hypertonia and hyperreflexia (U.M.N) This is known as Tonic atrophy In the lower limb : Weakness with signs of U.M.N.L Diagnosis of MND 1. Nerve conduction studies: preservation of sensory responses, normal or reduced motor amplitudes 2. Needle EMG (Intramuscular): Will demonstrate Active denervation: Fibrillation potentials and chronic sharp waves Chronic denervation in multiple myotomes 3. MRI, Brain/Spine -T2 weighted imaging- hyperintensity of corticospinal tract- Posterior limb of internal capsule, centrum semiovale Assessment of M.N.D o History : personal history : Name , Age (50-70),sex(male >female ), special habits present history : onset (gradual ) course (progressive ) Duration Medications Past history : Trauma (to exclude SCI), other medical problems Family history : not important Chief complain : in patient own words o Examination : General: ass of respiration Neurological: I. Non essential II. Essential Non essential : Mentality : emotional liability ,changes in mood and pseudo bulbar palsy. Cranial nerves : all are affected except (1,2,8) , ocular nerves and sensory part of 5,7 Speech : Dysarthria which characterized by slurred speech , dysphonia (bulbar symptoms ). Essential : Sensory examination : is normal Motor examination : Inspection : -wasting of muscles -fasciculations Muscle tone : -according to the type U.M.N affection (hypertonia) L.M.N affection (hypotonia)  Muscle test : -patient easily fatigued so in U.M.N affection ( do group ms test if mild cases , functional ms test if moderate -in L.M.N affection (do group ms test ) Reflexes ass : -according to the type Coordination : -due to ms weakness, (equilibrium is only affected) ADL ass :affected most commonly in (progressive muscle atrophy ) Gait ass : according to type , with disease progression (crutches , walker , wheel chair )  Screening for multisystem involvement should include checking vital signs at rest, skin integrity, bony abnormalities, sensory integrity, communication ability, and ability to follow multistep commands. 1-Baseline testing of muscle strength (manual muscle testing [MMT] or electronic handheld dynamometer testing if standards are clear and can be replicated), ROM, spasticity, and endurance, documentation of any areas of atrophy. 2-Documentation of pain (type, site, and intensity; use body chart and subjective pain scale); identify what makes pain worse or better and should be examined subjectively and objectively, using a Visual Analogue Scale (VAS) for example. Pain is not necessarily a direct impairment of ALS, but rather an indirect (decreased ROM, adhesive capsulitis) or a composite impairment (joint malalignment secondary to spasticity and faulty posture). 3-Assessment of bulbar. (For an in-depth evaluation of bulbar function, the cranial nerves commonly affected by ALS include V, VII, IX, X, and XII. Cranial nerves should be tested to determine the extent of bulbar involvement. -Respiratory Function Determination of respiratory status and function includes examination of respiratory symptoms and muscle function, breathing pattern, chest expansion, respiratory sounds, cough effectiveness, and VC or forced vital capacity (FVC) using a handheld spirometer. Supine FVC may be a better indicator of diaphragm weakness than erect FVC, and maximal inspiratory pressure (MIP) may also be useful in respiratory function monitoring because it can detect early respiratory insufficiency. 5-Integument In general, even in the late stage of ALS skin integrity is rarely a problem. Skin inspection should be used to examine contact points between the body and assistive, adaptive, orthotic, protective, and supportive devices, mobility devices, and the sleeping surface. 6-Environmental assessment with a focus on energy conservation and safety at current and future functional capabilities. In evaluating the results of the examination, the therapist should synthesize data to define which are necessary for developing goals with the patient. -Cognition No ALS-specific cognitive test or measure exists. If dementia or cognitive impairments are suspected, executive function, language comprehension, memory, and abstract reasoning should be examined. As depression and anxiety are common in individuals with ALS, screening is important and referral to a psychologist or psychiatrist for further evaluation may be indicated. 8-Static and dynamic postural alignment and body mechanics during self-care. Postural stability, reactive control, anticipatory control, and adaptive postural control should also be determined. No ALS-specific balance test or measure exists. A variety of balance status measures, originally designed for use with other patient populations, including the Berg Balance Scale, the Timed Up and Go Test (TUG), and the Functional Reach Test, can be used. 9-Gait No ALS-specific gait test or measure exists. Documentation of gait within a particular time period (e.g., within 15 seconds) or over a certain distance (e.g., 10 feet [3 meters]) has been measured in clinical trials. Gait stability, safety, and endurance should be examined. Energy expenditure, alignment, fit, practicality, safety, and ease of use of orthotic and assistive devices should also be examined at regular intervals. 10-Fatigue is very common in individuals with ALS. No ALS-specific measures exist; the Fatigue Severity Scale has been used in clinical trials. 11-Assessment of functional activity level (using a standardized test or assessment tool whenever possible) to include, as appropriate: transfers, gait, upper-extremity function, postural control, and assistive devices, timed walk test, or Purdue Pegboard. The Functional Independence Measure (FIM) has been used to document functional status in clinical trials. The Schwab and England Activities of Daily Living Scale is an 11-point global measure of functioning that asks the rater to report activities of daily living (ADL) function from 100% (normal) to 0% has been used to examine function in individuals with ALS. The suggested tools specific for ALS are the ALS functional rating scale (ALSFRS), the ALS severity scale (ALSSS). Another five-point scale of severity is currently being used in ALS clinical drug trials. Patients in stage 1 (mild disease) have a recent diagnosis and are functionally independent in ambulation, activities of daily living (ADLs), and speech. Stage 2 (moderate) identifies patients with mild deficits in function in three regions or a moderate to severe deficit in one region and mild or normal function in two other regions. Stage 3 (severe) defines patients who need assistance because of deficits in two or three regions; for example, the patient needs assistance to walk or transfer, needs help with upper-extremity activities, and/or is dysarthric or dysphasic. Stage 4 identifies patients with nonfunctional movement of at least two regions and moderate or nonfunctional movement of a third area. Stage 5 is death.