Neuro Pathology Flashcards
Meningitis
Bacterial: sicker, more rapid course; treat infective organism
OR
Viral: treat with antibacterial therapy (antibiotic, antipyretic) and maintain fluid/electrolyte balance
Provide supportive symptomatic therapy
Bed positioning, PROM, skin care, safety measures
Encephalitis
Severe infection and inflammation of the brain
Caused by arboviruses or a sequela of influenza, chronic and recurrent sinusitis, otitis, or other infections; bacterial encephalitis, prioncaused disease (“mad cow”)
Treat infective organism (bacterial encephalitis)
Provide supportive symptomatic therapy
Brain abscess
Infectious process in which there is a collection of pyogenic material in the brain parenchyma
S/S: H/A, fever, brainstem compression, focal signs CN II and VI
Can be an extension of an infection, typically frontal or temporal lobes or cerebellum
Treat infective organism, surgical intervention
Provide supportive symptomatic therapy
Acquired immunodeficiency syndrome (AIDS)
Viral syndrome characterized by acquired and severe depression of cell-mediated immunity
S/S: wide ranging, 1/3 exhibit CNS/PNS deficits
- AIDS dementia complex (ADC): range from confusion to memory loss to disorientation
- Motor deficits: ataxia, weakness, tremor, loss of fine motor coordination
- Peripheral neuropathy: hypersensitivity, pain,s sensory loss
Treat with anti-HIV drugs
Provide palliative and supportive therapy
Cerebrovascular Accident (CVA) (PT GOALS only)
ROM and prevent deformity Skin integrity Sensory compensation strategies Strengthen all available muscles Normalize of tone Selective movement control (out of synergy) Postural control, balance and symmetry Task-specific training Respiratory and oromotor control Isokinetic training Locomotor training with body weight support Biofeedback training Constraint-Induced movement therapy (CIMT)
DO NOT:
Use overhead pulleys on hemiplegic UE (traction injury risk)
Prescribe exercise/progressive physical activity without monitoring HR/BP
Use CIMT if pt doesn’t meet minimal movement criteria at wrist and fingers
Traumatic Brain Injury (TBI)
MOI
Concussion
MOI is contact forces to skull and rotational acceleration forces, causing varying degrees of injury to brain
Concussion: LOC either temporary or permanent resulting from injury or blow to head with impaired functioning of RAS
TBI: PRIMARY BRAIN DAMAGE
Primary brain damage (types of injuries):
(1) Diffuse Axonal: disruption/tearing of axons and small blood vessels from shear-strain of angular acceleration leading to neuronal death and petechial hemorrhages
(2) Focal: contusions, lacerations, mass effect from hemorrhage and edema
(3) Coup-Contracoup: injury at point of impact and opposite point of impact
(4) Closed or Open: fracture of skull
TBI: SECONDARY BRAIN DAMAGE
Secondary brain damage:
(1) Hypoxic-ischemic: systemic problems (respiratory or cardiovascular) that compromise cerebral circulation
(2) Swelling/edema: mass effect with increased ICP, brain herniation and death
(3) Electrolyte imbalance: mass release of damaging neurotransmitters
TBI DEFINITIONS
COMA
State of unconsciousness in which there is neither arousal nor awareness
Eyes remain closed, No sleep/wake cycles
TBI DEFINITIONS
UNRESPONSIVE VIGILIANCE/VEGETATIVE STATE
Marked by the return of sleep/wake cycles and normalization of vegetative functions (respiration, digestion, BP control)
Persistent vegetative state: vegetative state >1 year post TBI
TBI DEFINITIONS
MUTE RESPONSIVENESS/MINIMALLY RESPONSIVE
State in which patient is not vegetative and does show signs, even if intermittent, of fluctuating awareness
TBI DEFINITIONS
CONFUSIONAL STATE
Mainly a disturbance of attention mechanisms
All cognitive operations are affected, patient is unable to form new memories
May demonstrate either hypoarousal or hyperarousal
TBI DEFINITIONS
EMERGING INDEPENDENCE
Confusion is clearing and some memory is possible
Significant cognitive problems and limited insight remain
Frequently uninhibited social behaviors
TBI DEFINITIONS
INTELLECTUAL/SOCIAL COMPETENCE
Increasing independence, although cognitive difficulties (problem solving, reasoning) persist along with behavioral and social problems (enhancement of premorbid traits, mood swings)
TBI
PT GOALS FOR RANCHOS LOS AMIGOS LEVELS OF COGNITIVE FUNCTIONING (LOCF) I-III
(5)
(1) Maintain ROM, prevent contracture development: PROM, positioning, splinting and serial casting
(2) Maintain skin integrity, prevent decubitus ulcers through frequent position changes
(3) Maintain respiratory status, prevent complications: PD, percussion, vibration, suctioning to keep airway clear
(4) Sensory stimulation for arousal and to elicit movement: environmental/direct stimulation
(5) Promote early return of FMS: upright positioning and proper body alignment
TBI
PT GOALS FOR RANCHOS LOS AMIGOS LEVELS OF COGNITIVE FUNCTIONING (LOCF) IV-VI
(7)
(1) Structure and prevent overstimulation for confused, agitated patient: closed, reduced stimulus environments, daily logs, relaxation
(2) Provide consistency: team-determined behavioral modification techniques, clear feedback, written contacts
(3) Task-specific training: familiar and well-liked activities, off options, break down complex task
(4) Verbal or physical assistance
(5) Control rate of instruction, frequent orientation to time, place, etc.
(6) Safety, behavioral management
(7) Model calm, focused behavior
TBI
PT GOALS FOR RANCHOS LOS AMIGOS LEVELS OF COGNITIVE FUNCTIONING (LOCF) VII-VIII
(5)
(1) Increasing independence
(2) Assist in behavioral, cognitive, emotional reintegration: honest feedback, prepare for community re-entry
(3) Independence in functional tasks: FMS, ADLs, real-life environments
(4) Improve postural control, symmetry and balance
(5) Active lifestyle, improve endurance
SPINAL CORD INJURY
CENTRAL CORD SYNDROME
Loss of more centrally located cervical tracts/arm function with preservation of more peripherally located lumbar and sacral tracts/leg function
Typically caused by hyperextension of cervical spine
Loss of spinothalamaic tracts: B pain/temp
Loss of ventral horn: B motor function (UE)
Preservation of proprioception and discriminatory sensation
SPINAL CORD INJURY
BROWN-SEQUARD SYNDROME
Hemisection of spinal cord typically caused by penetration wounds (gunshot or knife) with asymmetrical symptoms
IPSI loss of dorsal col: tactile discrim, pressure, vibration and proprioception
IPSI loss of corticospinal: motor function and spastic paralysis below level of lesion
CONTRA loss of spinothalamic: pain/temp below level and B pain/temp loss at level
SPINAL CORD INJURY
ANTERIOR CORD SYNDROME
Damage mainly in anterior cord leading to loss of motor function, pain and temperature with preservation of light touch, proprioception and position sense
Typically caused by flexion injuries of cervical spine
Loss of lateral corticospinal: motor function and spastic paralysis below level
Loss of spinothalamic: B loss pain/temp
Preservation of dorsal col: proprio, kinesthesia and vibratory sense
SPINAL CORD INJURY
POSTERIOR CORD SYNDROME
Loss of posterior columns with preservation of motor function, sense of pain and light touch; extremely rare
Loss of dorsal col B
B loss proprioception, vibration, pressure, and epicritic sensations (stereognosis, 2pt discrim)
Preservation of motor function, pain and light touch
SPINAL CORD INJURY
CAUDA EQUINA
Injury below L1 results in injury to lumbar and sacral roots of peripheral nerves (LMN) with sensory loss and paralysis and some capacity for regeneration
A LMN lesion with autonomous or nonreflex bladder
Flaccid paralysis with no spinal reflex activity
Flaccid paralysis of bowel and bladder
Potential for nerve regeneration
SPINAL CORD INJURY
SACRAL SPARING
Sparing of tracts to sacral segments with preservation of perianal sensation, rectal sphincter tone or active toe flexion
SPINAL CORD INJURY
CHANGES TO MONITOR FOR (5)
(1) Spinal Shock: transient period of reflex depression/spasticity, up to 24 weeks
(2) Spasticity/Spasms: location of tone, examine nociceptive stimuli that trigger incr tone
(3) **Autonomic Dysreflexia: emergency situation in which noxious stimulus precipitates pathological autonomic reflex
- Leads to bradycardia, H/A, diaphoresis, flushing, diplopia, or convulsions
- Examine for irritating stimuli
- ELEVATE head, check/empty catheter FIRST
(4) Heterotopic bone formation: abnormal bone growth in soft tissues; soft tissue swelling, pain, erythema–generally near large joints
(5) Deep venous thrombosis: edema and tenderness in LE
SPINAL CORD INJURY
PT GOALS
Respiratory capacity: deep breathing, strengthening, assisted coughing, respiratory hygiene, abdominal support Maintain ROM, prevent contracture Maintain skin integrity, free of decubitus ulcers and injuries Improve strength Reorient patient to vertical position Promote early return of FMS and ADLs Improve sitting tolerance Cardiovascular endurance
SPINAL CORD INJURY WHEELCHAIR PRESCRIPTION: C1-4
Electric wheelchair with tilt-in-space seating or reclining seat back
Microswitch or puff-and-sip controls
Portable respirator may be attached
SPINAL CORD INJURY WHEELCHAIR PRESCRIPTION: C5
Manual wheelchair with propulsion aids
Independent for short distances on smooth, flat surfaces
May choose electric wheelchair for distance and energy conservation
SPINAL CORD INJURY WHEELCHAIR PRESCRIPTION: C6
Manual wheelchair with friction surface hand rims
Independent
SPINAL CORD INJURY WHEELCHAIR PRESCRIPTION: C7
Manual wheelchair with friction surface hand rims with increased propulsion ability
Independent
SPINAL CORD INJURY WHEELCHAIR PRESCRIPTION: C8-T1 AND BELOW
Manual wheelchair, standard hand rims
LOCOMOTOR TRAINING FOR INDIVIDUALS WITH COMPLETE INJURIES: T6-9
Supervised ambulation for short distances requires bilateral KAFO’s and crutches
Swing-to gait pattern
Requires assistance
May prefer standing devices/wheelchairs for physiological standing
LOCOMOTOR TRAINING FOR INDIVIDUALS WITH COMPLETE INJURIES: T12-L3
Independent in ambulation on all surfaces and stairs using a swing-through or four point gait pattern and bilateral KAFO’s and crutches
May use RGOs with walker with or without FES system
Independent household ambulators, wheelchair use for community ambulation
LOCOMOTOR TRAINING FOR INDIVIDUALS WITH COMPLETE INJURIES: L4-5
Independent with bilateral AFO’s and crutches or canes
Typically independent community ambulatory
May use wheelchair for activities with high-endurance requirements
SPINAL CORD INJURY
ABSOLUTE CONTRAINDICATIONS TO EXERCISE TESTING
Autonomic dysreflexia Severe/infected skin on WB surfaces Symptomatic hypotension UTI Unstable fracture Uncontrolled hot/humid environment Insufficient ROM to complete exercise
LOCOMOTOR TRAINING FOR INDIVIDUALS WITH INCOMPLETE INJURY
Treadmill Training (TT) with body weight support (BWS)
Incomplete injuries (ASIA B, C and D)
Variable levels of loading: 35% to 10% to full
High frequency (4 days/wk)
Moderate duration (20-30 min)
8-12 weeks
Multiple Sclerosis
Characteristics
Chronic, progressive, demyelinating disease of the CNS affecting mostly young adults
Demyelinating lesions (plaques) impair neural transmission causing nerves to fatigue rapidly
Variable symptoms: lesions scattered in time and place, lesions common in pyramidal tract, dorsal columns and periventricular areas of cerebrum, cerebellar peduncles
Variable course with fluctuating periods
Precipitating or exacerbating factors: infections, trauma, pregnancy, stress
Transient worsening of symptoms: adverse reactions to heat, hyperventilation, dehydration and fatigue
Relapsing-remitting MS (RRMS)
Relapses with either full recovery or some remaining neurological S/S and residual deficit on recovery; periods between relapses characterize by lack of disease progression
Primary-progressive MS (PPMS)
Disease progression from onset without plateaus or remissions or with occasional plateaus and temporary minor improvemnts
Secondary-progressive MS (SPMS)
Initial relapsing-remitting course followed by progression at a variable rate that may also include occasional relapses and minor remissions
Progressive-relapsing MS (PRMS)
Progressive disease from onset but without clear, acute relapses that may or may not have some recovery or remission
Commonly seen in individuals who develop disease after 40 years old
Multiple Sclerosis
Medical Management
PT GOALS
Med Management:
(1) Immunosuppressant drugs (ACTH, steroids)
(2) Interferon drugs (Avonex, Betaseron, Copaxone)
(3) Symptomatic management of spasticity (drugs, baclofen pump, phenol block surgery)
(4) Symptomatic management of urniary problems (anticholinergic drugs)
PT GOALS: Monitor changes: UTI, respiratory infection Restorative: intensive, time-limited Functional maintenance ROM, prevent contractors Skin integrity Sensory awareness Strength Motor control coordination Postural control, symmetry, balance Independence with mobility and ADLs Energy conservation techniques
AVOID
Precipitating exacerbations: schedule PT during optimal times for function
Overheating
Stressors
Parkinson’s Disease
Characteristics
Stages
Chronic, progressive disease of CNS with degeneration of dopaminergic substantia nigra neurons and nigrostriatal pathways
Deficiency of dopamine within basal ganglia corpus striatum with degeneration of substantial nigra
Loss of inhibitory dopamine results in excessive excitatory output from cholinergic system (acetylcholine) of basal ganglia
Rigidity (leadpipe or cogwheel)
Bradyinesia
Resting tremor
Impaired postural reflexes
STAGES
I: minimal or absent disability, unilateral Sx
II: minimal B or midline involvement, NO balance involvement
III: all sx present and severe; stands and walks only with assistance
IV: confinement to bed/wheelchair
Parkinson’s Disease
Medical Management
PT GOALS
Med Management:
(1) Sinemet (levdopa/carbidopa): provides dopamine and decreases effects of disease; effect is prolonged with low-protein diet; in on-off phenomenon: sudden changes from normal function to immobility to severe dyskinetic movement
(2) Dopamine agonist drugs: enhance effects of Sinemet therapy
(3) Anticholinergic drugs: control tremors
(4) Amantadine: enhances dopamine release
(5) Selegiline (deprenyl): monoamine oxidase inhibitor increases dopamine; used early in disease to slow progression
(6) Deep brain stimulation in thalamus or sub thalamic nucleus
PT GOALS: Monitor changes Prevent/minimize secondary impairments Compensatory strategies: initiate movement, repetitive auditory stimulation Improve strength Relaxation skills Postural control, symmetry, balance Improve gait, locomotor training Independence in FMS and ADLs Maximum mobility and safety
Myasthenia gravis
Neuromuscular junction disorder characterized by progressive muscular weakness and fatiguability on exertion
Muscular strength worse with continuing contraction, improved with rest
4 types:
ocular myasthenia (confined to extraocular mm)
mild generalized myasthenia
severe generalized myasthenia
crisis
-Generalized: bulbar (extraocular, facial and muscles of mastication) and proximal limb-girdle mm
May progress form mild to severe within 18 months
EXAMINE
cranial nerves
respiratory function
mm strength: proximal more involved, fatigue
functional mobility: climb stairs, sit to stand, lifting
EMG and repetitive nerve stimulation: slow abnormal responses
MED MANAGEMENT: acetylcholinesterase inhibitors corticosteroids immunosuppressants alternative: plasmapheresis, thymectomy
PT GOALS:
changes in condition: vitals, respiration, swallowing
energy conservation
Epilepsy
Symptoms
Causes
Disorder characterized by recurrent seizures (repetitive abnormal electrical discharges within the brain)
Symptoms: altered consciousness altered motor activity (convulsion): involuntary mm contraction, tonic (stiff/rigid) and clonic (rhythmic jerking) sensory phenomena autonomic phenomena cognitive phenomena
Causes:
acquired brain disease or trauma: tumor, stroke
degenerative brain disease: alzheimers, amyloidosis
developmental brain defects
drug overdose/withdrawal
electrolyte disorders
hyperthermia
infections, brain abscess, meningitis, neurocysticerocosis
pregnancy complications
Epilepsy
Classification of Generalized Seizures
Tonic-Clonic (Grand Mal): dramatic loss of consciousness, with a cry, fall and tonic-clonic convulsions of all extremities
- tongue biting and arrested breathing, incontinence
- contractions subside after 2-5 min and consciousness is gradually regained
- pt is confused, drowsy and amnesiac after event
- full recovery may take several hours
Absence Seizures (Petit-Mal): brief, almost imperceptible lapse of consciousness followed by immediate and full return to consciousness
- posture is maintained with no convulsive muscle contractions
- may occur 100x/day
Epilepsy
Classification of Partial Seizures
Simple Partial Seizures: focal, begin locally limited to a portion of the body, consciousness preserved; usually has an identified structured cause
Focal Motor: clonic activity involving a specific area of the body
Focal Motor with March (Jacksonian): orderly spread or march of clonic movements from initial muscles to involve adjacent muscles, with spread to entire side
Temporal Lobe Seizure: episodic changes in behavior with complex hallucinations, automatisms (lip smacking, chewing, etc.), altered cognitive and emotional function, preceded by an aura
Epilepsy
Secondarily generalized seizures
Status epilepticus
Secondary generalized seizures: simple or complex partial seizures evolving to a generalized seizure
Status epilepticus: prolonged seizure or a series of seizures (>30 min) with very little recovery between attacks; may be life threatening medical emergency
Epilepsy
Medical Management
PT GOALS
Med Management:
Antiepileptic meds: phenytoin (Dilantin), carbamazepine (Tegretol), phenobarbital
Surgical: lobe resection, hemispherectomy
PT GOALS:
Protect pt from injury
Establish airway, prevent aspiration
Lesions of archicerebellum, paleocerebellum and neocerebellum
LESIONS OF CEREBELLUM = IPSI S/S
Archicerebellum:
- central vestibular sx: ocular dysmetria, poor eye pursuit, dysfunctional VOR, impaired eye-hand coordination
- gait and trunk ataxia
- little change in tone or dyssynergia of extremity movements
Paleocerebellum:
- hypotonia
- truncal ataxia
- ataxic gait
Neocerebellum:
- intention tremor
- dysdiadochokinesia
- dysmetria
- dyssynergia
Additional impairments: asthenia, hypotonia, motor learning impairments, cognition, emotional dysregulation
PT GOALS:
improve accuracy of limb movement, postural stability, functional mobility, VOR
Unilateral vestibular disorders (UVD)
Trauma: vestibular symptoms in 30-65% of patients with TBI
Vestibular neuronitis, labyrinthitis: acute infection with prolonged attack of sx, persisting for several days/weeks caused by viral/bacterial infection
Menieres disease: recurrent, usually progressive; episodic attacks may last from minutes to several hours with severe sx; assoc with tinnitus, deafness, sensation of pressure/fullness in ear; edema of membranous labyrinth
Benign paroxysmal positional vertigo (BPPV): brief attacks of vertigo/nystagmus that occur with certain head positions; may be related to degenerative processes, mechanical impairment of peripheral vestibular system
Tumor: acoustic neuroma, gliomas/brainstem or cerebellar medulloblastoma
Medical management: vestibular suppressant medications
PT GOALS:
safety measures
habituation training: repetition of mvoements/postures that provoke dizziness and verge
gaze stability exercises: eye movements (X1/X2), head movements
postural stability: sitting, standing, dynamic balance
relaxation training
BPPV:
canalith repositioning treatment (CRT)–horizontal and posterior SCC BPPV
liberatory maneuver–posterior SCC BPPV
brandt-daroff exercises–residual/mild vertigo
Bilateral vestibular disorders (BVD)
Toxicity: ototoxic drugs
Bilateral infection: neuritis, meningitis
Vestibular neuropathy, otosclerosis (Paget’s disease)
Medical management: vestibular suppressant medications
PT GOALS:
safety measures
habituation training: repetition of mvoements/postures that provoke dizziness and verge
gaze stability exercises: eye movements (X1/X2), head movements
postural stability: sitting, standing, dynamic balance
relaxation training
Peripheral nerve disease/injury
Wallerian degeneration
Transection (neurotmesis) results in degeneration of axon and myelin sheath distal to site
- chromatolysis and repair process in nerve cell body
- endoneurium (sheath) does NOT degenerate but forms a tube directing regeneration
Peripheral nerve disease/injury
Segmental demyelination
axons are preserved; demyelination restores function
Peripheral nerve disease/injury
Axonal degeneration
degeneration of axon cylinder and myelin, distal to proximal
Peripheral nerve disease/injury
Neuropathy
Radiculopathy
Neuropathy: disease of nerves characterize by deteriorating neural function
Polyneuropathy: B symmetrical involvement of peripheral nerves, LE>UE, distal earlier and more involved proximal
Mononeuropathy: involvement of a single nerve
Radiculopathy: involvement of nerve roots
Peripheral nerve diease/injury
Traumatic nerve injury classes
Neurapraxia (Class 1): injury to nerve that causes a transient loss of function (conduction block ischemia); nerve dysfunction may be rapidly reversed or persist a few weeks (compression)
Axonotmesis (Class 2): injury to nerve interrupting the axon and causing loss of function and wallerian degeneration distal to the lesion; with no disruption of the endoneurium, regeneration possible (crush injury)
Neurotmesis (Class 3): cutting of the nerve with severance of all structures and complete loss of function; rein nervation typically fails without surgical intervention because of aberrant regeneration (failure of regenerating axon to find its terminal end
Clinical symptoms of LMN syndrome and Diagnostic tests
Weakness/paresis of denervated muscle, hyporeflexia and hypotonia, (rapid) atrophy, fatigue
Sensory loss: corresponds to motor weakness; proprioceptive losses may yield sensory ataxia; insensitivity may yield limb trauma
Autonomic dysfunction: vasodilation and loss of vasomotor tone (dryness, warm skin, edema, orthostatic hypotension)
Hyper excitability of remaining nerve fibers
- Sensory dyesthesias: hyperalgesia, pins and needles, N/T, burning
- Motor: fasciculations, spasms
Muscle pain (myalgia) with inflammatory myopathies
Diagnostic tests:
NCV=slowed conduction times or complete conduction block may be evident
EMG=signs of denervation atrophy (spontaneous fibrillation potentials); evidence of reinnervation (low-amplitude, short duration, polyphasic motor unit potentials)
Trigeminal neuralgia
Neuralgia of trigeminal nerve (CN V) results from degeneration or compression (tortuous basilar artery or cerebellopontine tumor) occurs in older pop. (mean age 50 y/o), abrupt onset
Brief paroxysms of neurogenic pain (stabbing and/or shooting) reoccurring frequently
- along distribution of trigeminal nerve, mandibular and maxillary divisions, restricted to ONE SIDE of the face
- autonomic instability: exacerbated by stress, cold; relieved by relaxation
Medical management: medications (anti-convulsants, vitamin B12), alcohol injections, surgery
TENS may be effective for pain relief
Bell’s palsy
Facial paralysis
LMN lesion involving CN VII (facial nerve) resulting in UNILATERAL facial paralysis
Acute inflammatory process of unknown etiology (immune or viral) resulting in compression of the nerve within the temporal bone
Characteristics:
(1) mm of facial expression on one side are weakened or paralyzed
(2) loss of control of salivation or lacrimination
(3) onset actue, with max severity in a few hours or days, commonly preceded by a day or two of pain behind ear; most recover in several weeks or months
(4) sensation is normal
Meds: corticosteroids, analgesics
PT GOALS:
protect cornea (artificial tears or patching)
e-stim to maintain tone
active facial exercises
sling to prevent overstitching of facial mm
functional retraining
Bulbar palsy
bulbar paralysis
Weakness or paralysis of muscles innervated by motor nuclei of the lower brainstem, affecting mm of face, tongue, larynx and pharynx
Result of tumors, vascular or degenerative diseases of lower cranial nerve motor nuclei
EXAMINE:
Glossopharyngeal and vagal paralysis: phonation, articulation, palatal action, gag reflex, swallowing
Voice quality
B involvement: airway restriction
Psuedobulbar palsy: B dysfunction of corticobulbar innervation of brainstem uncle; central or UMN lesion analogous to corticospinal lesions disrupting function of anterior horn cells
-Examine for hyperactive reflexes: increased jaw jerk and snout reflex
PT GOALS:
suctioning, oral care
maintain respiratory function
elevate head of bed
Guillain-Barre syndrome
Polyneuritis with progressive muscular weakness with an autoimmune attack, usually occurs after recovery from an infections disease (respiratory or GI)
Characteristics:
(1) Acute demyelination of both cranial and peripheral nerves (LMN disease)
(2) Sensory loss, paresthesisas (tingling, burning) pain; less than motor loss
(3) Motor paresis/paralysis: symmetrical distribution of weakness; LE to UE and distal to proximal
(4) Dysarthria, dysphagia, diplopia and facial weakness
(5) Progression evolves over a few wks/days recovery usually slow (6 mo to 2 yrs) and usually complete
COMPLICATIONS: Respiratory impairment/failure Autonomic instability Pain Risk of pneumonia Prolonged hospitalizations/immoblity Relapse
Medical: good nursing care, plasmapheresis, IVIG, analgesics
PT GOALS: maintain respiratory function prevent indirect impairments: PROM, positioning, skin care prevent injury to denervated muscles muscle re-educatoin energy conservation
Amyotrophic lateral sclerosis
(ALS, Lou Gehrig’s Disease)
Description
Characteristics
Degenerative disease affecting UMNs and LMNs (degeneration of anterior horn cells and descending corticobulbar and corticospinal tracts)
Characteristics:
(1) Progressive, often leading to death in 2-5 years
- Bulbar onset: progressive bulbar palsy
- Spinal cord onset: progressive mm atrophy
(2) Muscular weakness that spreads over time limbs to body; mm fasciculations or twitching (LMN signs)
(3) Spasticity, hyperreflexia (UMN signs)
(4) Dysarthria, dysphagia, dysphonia
(5) Absence of sensory changes
(6) Autonomic dysfunction (1/3 pts)
(7) Pain due to spasticity, cramping, postural stress syndrome, hypo mobility or instability
(8) Respiratory impairments: weakness
(9) Sparing of bowel/bladder function
(10) Depression common
Amyotrophic lateral sclerosis
(ALS, Lou Gehrig’s Disease)
Stages (6)
Stage I: Early disease, mild focal weakness, asymmetrical distribution, symptoms of hand cramping and fasciculations
Stage II: moderate weakness in groups of mm, some wasting (atrophy) of mm; modified independence with assistive devices
Stage III: severe weakness of specific mm, increasing fatigue; mild to moderate functional limitations, ambulatory
Stage IV: severe weakness and wasting of Les, mild weakness of UEs, moderate assistance and assistive devices required; wheelchair user
Stage V: progressive weakness and deterioration of mobility and endurance, increased fatigue, moderate to severe weakness of whole limbs and trunk; spasticity, hyperreflexia; loss of head control; maximal assistance
Stage VI: bedridden, dependent ADLs, FMS; progressive respiratory distress
Amyotrophic lateral sclerosis
(ALS, Lou Gehrig’s Disease)
Examination
Medical management
PT GOALS
Examine: Cranial Nerves VII, IX, X, XI, XII Motor function: symmetrical distribution Spasticity, hyperreflexia ALS Functional Rating Scale (ALSFRS)
Medical management: no effective tx; Riluzole (glutamate antagonist) slows progression, symptomatic relief
PT GOALS:
respiratory function
nutritional needs
indirect impairments: PRM, positioning, skin care
moderate exercise program (monitor fatigue)
energy conservation
family/caregiver education
Postpolio syndrome (PPS)
New slowly progressive muscle weakness occurring in individuals with confirmed history of acute poliomyelitis due to possible hyper functioning of motor neurons, one term overuse at high levels resulting in new denervation
Characteristics:
(1) new weakness/atrophy; ASYMMETRICAL
(2) abnormal fatigue
(3) pain
(4) decreased function with reduced endurance
(5) slow progression: steady or stepwise
(6) environmental cold intolerance
(7) difficulty concentrating, memory, attention: damage to reticular formation, hypothalamus, dopaminergic neurons
(8) sleep disturbances
(9) decreased functional mobility
Examine:
motor function: spotty involvement, asymmetrical
EMG to identify prior anterior horn cell disease and new motor unit pathology
Medical management: antidepressants, neurotransmitter inhibitors
PT GOALS:
maintain respiratory function
energy conservation (avoid unnecessary activities to maximize important work)
preserve/increase muscle strength (mod exercise program)
aerobic conditioning (mod to low level)
orthoses, mobility aids, assistive devices
