MOVEMENT SYSTEM DIAGNOSES NEUROMUSCULAR CONDITIONS

Description of Categories

Patricia L Scheets, PT DPT NCS [email protected]

Collaborators from Washington University Nancy J Bloom, PT DPT MSOT Beth Crowner, PT DPT NCS MPPA Patricia N McGee, PT DPT PCS Barbara J Norton, PT PhD FAPTA Shirley A Sahrmann, PT PhD FAPTA Jennifer S Stith, PT PhD LCSW Susan K Strecker, PT DPT

INTRODUCTION

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The following pages contain descriptions of the movement system problems we have identified among people with neuromuscular conditions. While many of the people in the categories described in this set of diagnoses may have a central nervous system pathology, many will have non-specific and/or varied health conditions. What the patients have in common is one or more limitations in their ability to move within the environment, manipulate objects, and /or balance in a given position for reasons other than pain. In this set of movement impairment diagnoses, rather than being sorted by traditional health conditions or diseases, patients are categorized by their type of movement system problem. This allows for the grouping of patients along parameters that physical therapists both examine and treat.

We believe that the movement system problems described apply to both the adult and pediatric population. There are differences between adults and children in the health conditions associated with a given movement impairment diagnosis and the tasks examined. We have used the following symbols to designate characteristics of the diagnosis applicable for all age groups and those associated predominantly with pediatrics:

KEY  Observed in all age groups Observed in pediatrics

We have described the clinical examination required to identify these movement system diagnoses in an accompanying document. The examination consists of tests of body structures and functions and observational analysis of specific tasks. The key findings that relate to each specific diagnosis are outlined in the pages that follow.

In addition to the examination, we have outlined sample treatments for each of the diagnoses. The treatment ideas are derived from external evidence where possible and clinical practice experience. Because much of the literature related to rehabilitation intervention is sorted by health condition rather than movement system problem, the supporting external evidence for our treatment ideas is based on our understanding of the subjects in a given study, the study results, and how they may be related to the movement system problem described.

To request a copy of the examination and/or sample treatment ideas, please contact Patty Scheets at [email protected] or [email protected].

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DIAGNOSIS: MOVEMENT PATTERN COORDINATION DEFICIT The primary movement dysfunction is the inability to coordinate an intersegmental task because of a deficit in timing and sequencing of one segment in relationship to another. The movement dysfunction in the lower extremity is primarily observed during postural control tasks and in the upper extremity during in hand manipulation and grasp and release of different objects coupled with reach. Motor performance typically improves with practice and instruction. Differential Movement Subjective/Medical History Key Tests and Signs Associated Signs Expected Outcome System Dx Associated Conditions: Task Analysis: Movement:  Force Production Deficit  Stable with standing ADL  Stroke (mild) Sit to Stand:  Generally fractionated  Sensory Selection and tasks  Multiple Sclerosis  Altered sequence of movement against gravity Weighting Deficit  Independent ambulation (remitting) movement components throughout  Sensory Detection Deficit in home and community  Parkinson’s Disease during execution (usually  Dysmetria (at least in familiar (mild) insufficient DF of leg over Muscle Tone: environments)  Generalized debilitation foot)  Normal or mild  Ambulate without device  Multi-sensory  Posterior sway at ankle hyperexcitability, mild or with cane at most; may gait/balance disturbance and may step at hypotonicity or mild need an AFO but unlikely  s/p LE surgery termination rigidity  Ascend/descend stairs  BPPV with postural  Unlikely to require  Grades of 0-2 on reciprocally instability significant physical modified Ashworth  Gait speed at least 75% Down Syndrome assistance of normal for age Mental Retardation Sensation: Prenatal Drug/Alcohol Additional Transitional Mvts:  Normal or no more than Exposure Altered sequence, mild loss of JPS at great Developmental instability, and lack of toe or ankle in LE Coordination Disorder fluidity when executing  Normal or no more than Autism Spectrum transitional movements mild loss of sharp/dull or Disorder appropriate to age (or numbness in UE Prematurity adjusted age) Developmental Delay Non-equilibrium Fragile X Gait: Coordination: Idiopathic Toe Walker  Variable foot placement or  Normal or mild (to Motor Apraxia line of progression or may moderate) ataxia with be guarded with slow, reciprocal and synergistic Pt / Caregiver May Report: small steps movement  Feels unsteady; possible  Assistance for balance  Normal or mild ataxia with fall tests of accuracy  Fear of falling Clumsiness Occasional falls Delay in fine motor tasks Overly messy when

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eating and dressing Jump, Run, Skip and other Started walking later than advanced motor skills: other children Altered sequence, Awkward compared to instability, and/or lack of peers fluidity Poor performance in May need assistance for sports activities balance

Reach and grasp:  Slowed or awkward  Difficulty adjusting grip during transport of objects  Difficulty controlling force relative to task demands Lack of age appropriate grasp

Postural Control:  Increased latency in postural movement patterns and/or  Inappropriate amplitude of postural adjustments or responses  Increased posterior sway during stance activities

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DIAGNOSIS: FORCE PRODUCTION DEFICIT The primary movement fault is weakness. The origin of the weakness may be muscle, neuromuscular junction, peripheral nerve, or central nervous system dysfunction. The presentation may be focal (one joint), segmental (generalized to an extremity or body region), or related to fatigue (of skeletal muscle rather than cardiopulmonary capacity). Differential Movement Subjective/Medical History Key Tests and Signs Associated Signs Expected Outcome System Dx Associated Conditions: Strength: Movement:  Movement Pattern Good potential for  Less than 3+/5 to 4/5  Fractionated if present Coordination Deficit Impairment Recovery: Good potential for muscle strength  Hypokinesia  Stable with standing ADL Impairment Recovery: throughout a limb or limbs Muscle Tone: tasks  Generalized debilitation or  Normal or mild  Independent ambulation  Disuse atrophy  Difficulty moving through hyperexcitability, mild in home and community  Peripheral nerve full range against gravity hypotonicity or flaccid, (at least in familiar contusion or mild rigidity environments)  Guillian Barre Syndrome  Focal weakness at one  Grades 0-2 on the  Ambulate without device  Stroke (mild) primary joint or modified Ashworth or with cane at most (may  Brain injury  Deterioration in range of need AFO)  Multiple Sclerosis motion/speed of Sensation:  Ascend/descend stairs (remitting) movement with repetition  Normal or no more than reciprocally; may need Prematurity mild loss of joint position railing for balance/support Developmental Delay Task Analysis: sense at ankle, loss of  Gait speed at least 60- Down Syndrome Prone on elbows: sharp/dull, or numbness 80% of normal for age Fragile X Unable to maintain head  Able to use hand in all CP (mild) at 90 degrees when age Non-equilibrium functional tasks Transverse Myelitis appropriate Coordination:  With nervous system Hypotonia Pull to sit:  In more severe forms problems, may reach a Maintains head lag after may be unable to test due plateau in improvement in Poor potential for age appropriate to weakness strength and notice Impairment Recovery: Floor to stand  In milder forms, likely to ongoing difficulties with  Stroke Exhibits a + Gower’s sign be slow but accurate musculoskeletal fatigue,  Brain Injury or requires use of UEs via power, and speed  Multiple Sclerosis ½ kneel after age Postural Control: (primary or secondary appropriate  In early stages of Poor potential for progressive) Sit to Stand: recovery, unable to sit or Impairment Recovery:  Chronic Inflammatory  Failure during initiation perhaps stand  Varies with degree of Diffuse Polyneuropathy phase typically requiring unsupported; would fall involvement  Myopathies assistance or without support  In all but most severe accommodation  Muscular Dystrophies forms, improved  Extension of knees before  Spinal Cord Injury (low independence with hips during first half of ASIA A or B paraplegia) functional activities using

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 Polio/post-polio execution compensatory movement syndrome ? Gait: strategies but may still Cerebral Palsy  May need manual require assistance Myelomeningocele assistance or a device to  Use of wheelchair at least Hypotonia bear weight and maintain for distances likely; upright degree of independence Pt / Caregiver May Report:  Deviations are often with wheelchair mobility  Increased need for significant relative to involved caregiver assistance  In severe forms will be extremities  Fatigue unable to attempt  In less severe form, History of prematurity ambulation ambulate short distances Delay in acquisition of Reach and Grasp: with device and/or motor milestones  Difficulty or failure with bracing and/or physical appropriate for age reach above 60° shoulder assistance at very slow flexion and/or with speeds sustaining reach position  Able to use hand as an  Unable to maintain force assist with activity in less for gripping objects involved forms especially during transport  In more severe forms Unable to bring arms to requires 24 hour care midline in supine when age appropriate Unable to hold bottle in sitting

Postural Control:  Unable to stand unsupported or loss of support moment at hip and knee during single limb support  Limited improvement in performance with practice; may worsen with repeated trials  In more severe forms may be unable to sit unsupported

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DIAGNOSIS: FRACTIONATED MOVEMENT DEFICIT The primary movement dysfunction is the inability to fractionate movement associated with moderate or greater hyperexcitability. May describe the upper or lower extremity or both. Always associated with central neurological deficit.

Differential Movement Subjective/Medical History Key Tests and Signs Associated Signs Expected Outcome System Dx Associated Conditions: Movement: Task Analysis:  Force Production Deficit  Related to degree of  Stroke  Unable to fractionate Pull to sit: antigravity movement  Brain Injury/hypoxia movement Neck hyperextension with  In forms with more  Spinal Cord Injury (ASIA  Slow; unable to make shoulder elevation antigravity movement, C or D) rapid reversals in May exhibit LE extension, stability in sitting and with  Multiple Sclerosis movement adduction, and hip medial reaching; ambulation in Intraventricular  Unable to generate force rotation (LE extensor home and community Hemorrhage (IVH) rapidly pattern) with significant deviations Periventricular and significant reduction Luekomalacia (PVL) Muscle Tone: Prone on elbows: in speed with or without a Brain Tumor  Moderate or greater Neck hyperextension with cane; ascend/descend Meningitis hyperexcitability shoulder elevation stairs step-to with or CP/static encephalopathy  Grade 3 or 4 on the without a railing modified Ashworth Floor to stand:  In forms with 4 limb Pt / Caregivers May Report: Pulls up with UEs with involvement wheelchair  Stiffness of the limbs Reflex Testing: LEs extended; unable to for locomotion (probably and/or pain  May exhibit +ATNR, fractionate or dissociate electric) Complicated perinatal +STNR LE movements  Able to use UE as “assist” history, ie documented with ADL PVL, IVH, hypoxic Task Analysis: Sit to Stand: ischemic event  Consistent non-  Stiffness of involved limbs fractionated movement  Slow pattern across multiple  No dissociation of tasks movement at one joint from movement at another  May see associated reactions with increased effort  In forms with less anti- gravity movement, unable to stand

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Creeping: May exhibit bunny hopping or commando crawling rather than assuming a 4-point position Gait:  Compensatory movement strategy of hip hiking, vaulting, or circumduction to initiate swing of involved extremities  Stiffness of hip/knee flexion during swing  Scissoring  “toe walking” or “equinus gait”  Hip and knee often flexed during stance of involved extremities  Likely to require AFO to control foot position for weight bearing  Likely to require assistive device at least early in course  In forms with less anti- gravity movement, unable to stand Reach and Grasp:  Able to reach in very limited range(< 60-90°)  Hand closure with minimal relaxation or minimal opening  Finger flexion associated with wrist flexion and pronation

Postural Control:

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 In forms with more antigravity movement, able to sit unsupported but asymmetrically or with posterior pelvic tilt (sacral sitting) and hip medial rotation with compensatory thoracic flexion  Stability may improve with practice but symmetry will not

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DIAGNOSIS: POSTURAL VERTICAL DEFICIT

The primary movement dysfunction is inaccurate perception of vertical orientation resulting in postural control deficits and the tendency to resist correction of center of mass alignment. The condition may be in the medial/lateral or anterior/posterior direction.

Differential Movement Subjective/Medical History Key Tests and Signs Associated Signs Expected Outcome System Dx Associated Conditions: Postural Control: Movement:  Sensory Selection and  Related to severity of  Stroke (med/lat)  Shifts center of mass  Presentation is variable Weighting Deficit behavioral/cognitive  Brain Injury (med/lat) beyond limits of stability to although movement in at  Sensory Detection Deficit deficits, motor function,  Psychomotor side or backward without least 60% of muscle  Fractionated Movement and natural recovery of Disadaptation Syndrome weight acceptance groups in the LE is Deficit perceptual deficit (ant/post)  Resists correction or expected  Force Production Deficit  Assisted ambulation with  Backward Disequilibrium becomes  Movement may not be uncomplicated (ant/post) fearful/agitated when fractionated devices such as wheeled Rhett’s Syndrome center of mass walker or along a wall alignment is corrected Motor Planning:  Many are non-ambulatory Pt / Caregiver May Report:  Deficits may present in  May have difficulty and require significant  Backward falls sitting, standing, or with planning or organizing assistance with transfers walking depending on movement patterns into  Fear of falling severity purposeful actions  Visual or visual perceptual deficits Perception: Sensation:  Sensation of “falling”  Likely to be impaired to when shifted toward light touch and joint correct vertical alignment position sense (med/lat)  May have disregard or neglect of involved Behavior: extremities  Impulsive  Poor judgment  Fear avoidance behavior such as clutching or grabbing with UE and shifting base of support

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DIAGNOSIS: SENSORY SELECTION AND WEIGHTING DEFICIT The primary movement dysfunction is the inability to maintain postural orientation or motor performance as a result of decreased ability to screen for and attend to appropriate sensory inputs. Patients may demonstrate sensory seeking or sensory avoidance behaviors.

Differential Movement Subjective/Medical History Key Tests and Signs Associated Signs Expected Outcome System Dx Associated Conditions: Task Analysis: Movement:  Movement Pattern  Ambulation with straight  Stroke Gait:  Fractionated Coordination Deficit line of progression and no  Brain Injury  Deviation in line of  Postural Vertical Deficit loss of balance in all  Unilateral vestibular progression to one or both Non-equilibrium  Sensory Detection Deficit regular sensory hypofunction sides Coordination: environments  Bilateral vestibular  Instability with head  Intact  May have decreased hypofunction turning tolerance to prolonged  BPPV with postural Turning Around: Head Thrust Test: exposure to highly instability  Loss of balance or  May be positive visually stimulating Sensory Integration increased ankle or hip environments Autism Spectrum sway at termination Dynamic Visual Acuity:  May have symptoms with Disorder  Worse with faster  May be positive head/body turning tasks Pervasive Developmental movement with mild to no instability Disorder  Dizzy Sensation/Sensory Rhett’s Syndrome Behavior: Asperger’s Syndrome Postural Control: May show signs of gaze Sensory Processing  Able to stand unsupported aversion Disorder but may require practice May show signs of self  Increased sway or stimulation behaviors Pt / Caregiver May Report: instability with eyes such as rocking, spinning  Symptoms when riding in closed or other change in and banging a car, when walking sensory conditions along patterned  May demonstrate hip walkways, or in visually strategy during static stimulating environments standing tasks Repetitive non-purposeful  Postural responses may movements be delayed or Impaired social behaviors exaggerated; exaggerated Aversion to a variety of responses lead to sensory stimuli postural instability Delayed acquisition of  May improve with motor milestones modification of sensory appropriate for age needs and practice, instruction and

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encouragement

Dizziness:  Dizziness associated with head turning

Sensory Sensitivity:  Symptoms with smooth pursuit and/or saccadic eye movement or in situations with visual motion cues.  Symptoms with transitions from one sensory environment to another

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DIAGNOSIS: SENSORY DETECTION DEFICIT The primary movement dysfunction is the inability to execute intersegmental movement due to lack of joint position sense or multi-sensory failure affecting joint position sense, vision, and/or the vestibular system. May involve UE, LE, or both.

Differential Movement Subjective/Medical History Key Tests and Signs Associated Signs Expected Outcome System Dx Associated Conditions: Sensation: Movement:  Movement Pattern  Ambulation with assistive  Stroke  Moderate to severe  Poor timing and Coordination Deficit device  Brain Injury impairment of joint coordination of limb  Postural Vertical Deficit  Increased difficulty in  Incomplete spinal cord position sense or movement during tasks  Sensory Selection and conditions of poor lighting injury (ASIA C or D) protective sensation of Weighting Deficit and uneven surfaces  Peripheral one or both LEs Non-equilibrium  Limited standing stability polyneuropathy  Mild or greater loss of Coordination: for functional tasks  Multi-system failure joint position sense and  Slow and clumsy  Lack of hand function  Bilateral vestibular loss touch sensation of one or  Some improvement with without visual guidance both UEs visual guidance Pt / Caregiver May Report:  New visual field deficit  Unable to stand still greater than 50% Exposure to vincristine for childhood cancer Task Analysis: Child trips while walking Sit to Stand: or running  Failure during the Can hear child walking execution phase with due to foot slap hyperextension of the knee(s) before hip extension, instability of the ankle, and/or stepping to alter base of support Gait:  Variation in foot placement, hyperextension of the knee during stance, loss of eccentric ankle control (foot slap during gait)  Requires assistance  Some improvement with visual guidance if possible

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Reach and Grasp:  Slow and dyscoordinated  Improves with visual guidance

Postural Control:  Unable to stand unsupported or difficult  If able to stand unsupported, significant increase in sway or LOB with eyes closed  Limited improvement in performance with practice

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DIAGNOSIS: HYPOKINESIA The primary movement dysfunction is related to slowness in initiating and executing movement. May be associated with stopping of ongoing movement.

Differential Movement Subjective/Medical History Key Tests and Signs Associated Signs Expected Outcome System Dx Associated Conditions: Movement: Muscle Tone:  Force Production Deficit  In milder forms may see  Stroke  Able to move against  Rigid with passive  Cognitive Deficit improvement in step  Seizure Disorder gravity movement of U/LE and/or length and consistency of  Parkinson’s Disease  Arrests in ongoing trunk foot placement movement during functional  Improvement in use of  Extra-pyramidal tasks Non-equilibrium adaptive strategies in syndromes Coordination: more severe forms  Parkinsonism or Postural Control:  Undershoots movement Parkinson’s Plus  Delayed timing of postural  Likely to fall  Psychomotor adjustments or absent when aimed toward a Disadaptation Syndrome postural adjustments in target  Dementia response to or in  Slowness or arrests in IVH preparation of a movement reciprocal movement Seizure Disorder  Loss of balance posteriorly  Inability to use appropriate Reflexes: postural control strategy in Delayed integration of context early/primitive reflexes

Task Analysis: Sit to Stand or Floor to Stand:  Slow or lack of preparatory movement  Assistance with initiation  Loss of balance on termination  Unable to shift center of mass forward Gait:  Difficulty initiating ambulation  Often requires assistance due to arrests in ongoing movement  Unable to regulate step length

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DIAGNOSIS: DYSMETRIA

The primary movement dysfunction is related to the inability to grade forces appropriately for the distance and speed aspects of a task. Rapid movements are generally too large, and slow movements are generally too small for their intended purpose. Performance deteriorates with faster speeds. May involve UE, LE, or both. Generally associated with cerebellar dysfunction.

Differential Movement Subjective/Medical History Key Tests and Signs Associated Signs Expected Outcome System Dx Associated Conditions: Non-Equilibrium Postural Control:  Movement Pattern  Ambulation in home with  Stroke Coordination:  Generally able to sit with Coordination Deficit device and perhaps  Brain Injury  Difficulty directing UE support; may be able  Sensory Detection Deficit bracing  Cerebellar degeneration movement toward a target to sit unsupported  May require wheelchair  Multiple Sclerosis resulting in undershooting  Unable to stand in community or overshooting unsupported or stands  Limited independence Cerebral Palsy  Abnormal rhythm and with wide base of support Agenesis of the Corpus incoordination during with UE ADL tasks with rapidly alternating and high guard adaptive equipment Callosum movements Fragile X  No change with practice Task Analysis: Ataxia Gait: Movement:  Variable foot placement Pt / Caregiver May Report:  Able to move against in step length and step  Falls gravity width  Messy when eating  Lack of fluidity  Generally requires Is clumsy assistance Task Analysis: Has frequent injuries  Overshooting or

undershooting of targets; repeated stepping and wide base of support in standing tasks; excessive sway at trunk Sit to Stand:  Wide base of support; may see excessive sway at trunk; uses UE to stabilize Reach and Grasp:  Unable to reach to targets  Difficulty grasping small or light objects

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CLASSIFICATION: COGNITIVE DEFICIT The primary deficit in movement is impaired motor control related to lack of arousal, attention, or ability to apply meaning to situation that is appropriate for age.

Differential Movement Subjective/Medical History Key Tests and Signs Associated Signs Expected Outcome System Dx Associated Conditions: Cognition: Movement:  Fractionated Movement  May be dependent in all  Anoxia  Lack of arousal  May demonstrate loss of Deficit mobility or  Brain Injury  Lack of response to spontaneous or voluntary  Movement Pattern  May be mobile only  Neoplasm stimuli movement Coordination Deficit within a structured  Dementia  Absent attention to  May be able to move  Force Production Deficit environment  Severe mental examiner and situation against gravity but not in  24 hour retardation  Absent ability to apply relationship to situational assistance/supervision demands  Persistent vegetative meaning to situation

state

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