Other Neurological Disorders

What causes Multiple Sclerosis?

Degeneration & loss of myelin throughout the CNS, primarily in white matter → causes plaques

Destruction & inflammmation of oligodendrocytes

What are oligodendrocytes responsible for?

Myelination of the CNS, conduction of pathway

What type of motor & nervous system symptoms does MS display?

upper motor neuron & central nervous system

What are the usual UMN symptoms?

Hypertonia

Spasticity/rigidity

Hyperflexia

Muscle spasms

Synergistic movement Patterns

Weakness/paralysis not segmental or focal

What are the usual LMN symptoms?

Hypotonia

Flaccidity

Hyporeflexia

Fasciculations (muscle twitching)

No synergistic movement patterns

Weakness/paralysis segmental or focal (dermatomal)

MS Environmental Etiology

Autoimmune induced by viral or infectious agent such as herpes, chlamydia, Epstein Barr, measles

No proven direct trigger

MS Genetic susceptibility Etiology

Gen pop → 0.1% risk, 1st degree relative w/ 2.5-5% risk, identical twin rises to 25%

MS Geographical Etiology

More common in areas further from equator, areas of greater temp variations

MS Pathophysiology

Inflammatory lesions result in demyelination of white matter & axonal damage causing plaques in brain & spinal cord

Causes slow neural transmission, rapid neural fatigue, severe; conduction block & loss of function

Permanent disability

What are the 6 types of MS?

Relapsing Remitting

Primary Progressive

Secondary Progressive

Progressive Relapsing

Benign MS

Malignant MS(Marburg’s Variant)

Differences between Relaping Remitting & Primary/Secondary Progressive

Relapsing: relapses w/ periods of remission; no disease progression between & most common

Primary: progressive functional decline from onset; no distinct relapse

Secondary: initial relapsing remitting; progresses to steady decline; occasional acute attacks

Difference between Progressive Relapsing & Benign & Malignant

Progressive: progression from onset w occasional attacks

Benign: mild disease; remain fully functional in all neurological symptoms 15 yrs after onset

Malignant: rapid progression w severe disability or death; rare

What are relapses in regards to MS?

New or recurring symptoms lasting >24 hrs unrelated to other etiology

What is remission in regards to MS?

Partial or total disappearance of symptoms; as disease progresses periods of exacerbation become more frequent & remission more limited

What is Pseudoexacerbation in regards to MS?

Temporary worsening of symptoms <24hrs

What is Uthoff’s Phenomenon?

Worsening symptoms when body gets overheated from hot weather, exercise, fever or saunas & hot tubs; causes slow or block conduction

How can you treat Utoff’s Phenomenon?

Ice vest, bring core body temp down

What is Lhermitte’s sign?

Flexion of neck = electrical pain/shock down spine into LE’s

Caused by posterior column damage in SC

What are usual sensory symptoms than can occur in MS?

Paresthesia: abnormal sensation tingling (often 1st sign)

Decreased proprioception & vibration

What are common visual changes in MS?

Optic neuritis: inflammation of optic nerve → ice prick pain & blurring

Scotoma: blind spot

Eye mov’t dysfunction: nystagmus & diplopia; lesion CN 3,4,6

What are pain symptoms in MS?

Trigeminal neuralgia

Paroxysmal limb pain; brief spasms/shooting pains

Neuropathic pain

Headache

What are the two types of weakness that can occur in MS?

Corticospinal tract: UMN signs

Cerebellar: generalized weakness & ataxia

What is the most common symptom in MS?

Fatigue; sudden onset & not related to disease severity

Exacerbated by exertion, heat, humidity, depression, sleep & mood disorders

How does balance & coordination display in a MS patient?

Cerebellum & cerebellar tract lesions

Postural & intention tremors

Progressive ataxia & dizziness

How does gait display in a MS patient?

Related to lesion

Discoordination of alternating mov’ts, drunken gain, increased tone or weakness

What types of speech & swallowing issues can a MS patient have?

Dysarthia → motor speech

Dysphonia → vocalization

Dysphagia → swallowing

How can cognition deficits look in a MS patient?

Due to specific lesion location or meds

Depression, emotional lability, euphoria, pseudobulbar affect

MS Diagnostics

MRI: shows lesions/plaques

CSF: elevated immunoglobulin → elevated immune response

EMG: evoked potentials abnormal → slowed or abnormal conduction

MS Medical Management

Corticosteroids: used for acute relapses; gets rid of inflammation → reduce episode duration

Plasmapheresis: plasma exchange; increases recovery with no response to steroids

Interferons: modify immune system response by decreasing inflammation, swelling & proliferation

Immunosuppressants: temporarily shut down immune system; @ risk for other things

MS & Exercise

Significantly reduces fatigue & manages symptoms of weakness, decreased ROM, & decreased CV endurance

Assist with neuroplasticity

Exercise parameters for MS

2-3x/week, 20-30 min

50-75% of max HR, 1-3 sets of 15-18 reps for strength training → goal: increase to 3-4 sets

Important considerations for MS Therapy

Lukewarm aquatic therapy for pain

Early intervention; schedule alternate days

HR & BP may be blunted

Energy conservation

Weighted vest for ataxia

Outcome Measures for MS

6 min walk test, TUG, MS quality of life scale, 12 item MS walking scale, Nine Hole Peg Test, BERG, DGI/FGA

What is Guillain Barre Syndrome?

Acute, inflammatory, demyelinateing condition that results in motor and sensory deficits

Attacks Schwann cells; demyelination of PNS

What type of progression does GBS have?

Ascending symmetrical muscle weakness, areflexia & sensory/motor weakness

Lower motor neuron condition, flaccid paralysis(LMN)

What is the pathology of GBS?

Bacteria or viruses initiate inflammatory process

Demyelination due to autoimmune reaction 2-4 wks after viral infections, mild flu like respiratory infection or flu

What do Schwann cells do ?

Myelination of PNS → decrease nerve conduction

After 3 wks, cells may regenerate but take longer is axonal degeneration

How does GBS present?

Progressive sensory & motor loss; occurs distal to proximal & ascending symmetrical

Areflexia, pain is an early symptom

What are the 4 types of GBS?

1. Acute Inflammatory Demyelintating Polyradiculopathy

2. Acute Motor Axonal Neuropathy

3. Acute Motor & Sensory Axonal Neuropathy

4. Miller Fisher Syndrome

What is the difference & similarities between Acute Motor & Acute Motor/Sensory?

Acute Motor: only motor nerves affected

Acute Motor & Sensory: both nerves are affected; 70% w/ CN involvement & facial palsy

Both not demyelinating but nodes of Ranvier are attacked

What is Acute Inflammatory Demyelinating Polyradiculopathy?

Most common; attacks myelin sheath

Schwann cells destroyed but axons are intact

What are the 3 phases of GBS?

Progression(1-4wks): lose sensory & motor function; paresthesia, areflexia, weakness in all limbs

Plateau: symptoms stabilize, no declining or improved; 4 weeks

Recovery: any improvement of motor or sensory impairments; may have residual deficits

What poorer outcomes associated with?

Patients required ventilator support

Rapid progression

Low distal motor amplitudes (>axonal damage)

GBS Strengthening Intervention

Short bouts of non fatiguing exercise; low resistance, low reps

Over exerting partially denervated muscle affect force capability → <3/5 gravity minimized until full ROM w/ weight of limb only

What types of medical interventions are used for GBS?

Plasmapheresis & IVIG

Must be initiated in week 1-2 of symptom onset to decrease course

PT Intervention for Acute Phase of GBS

Support pulmonary function, prevent skin breakdown & contractures

ROM w/in pain or fatigue tolerances *use PROM

*extreme care with support & mov’t of denervated muscles

PT Intervention for Plateau Phase of GBS

Acclimate & increase tolerance to upright positions

Improve pulmonary function

Maintenance of ROM

PT Intervention of Recovery Phase of GBS

Strength begins to recover 2-4wks after plateau

Returns in reverse order of loss; proximal → distal

Respiratory weakness can limit upright tolerance

What is ALS?

Progressive degeneration of motor neurons in the brain & spinal cord (UMN) & anterior horn cells of the spinal cord (LMN)

Rapid disease progression; onset death 3-5 yrs after diagnosis due to compromised respiration

Etiology of ALS

Unknown but could be genetics & environmental factors → excessive exposure to glutamate may cause motor neurons to die

Pathophysiology of ALS

Degeneration of: UMN in cortex, lateral corticospinal tract in SC, anterior horn cells in SC(LMN), brainstem & CN V, VII, IX, X, XII

Spared: CN III, IV, VI (ocular muscles), bowel & bladder, sensory system

What are the usual ALS symptoms?

Fasciculations in tongue to muscles

Muscle cramps, fatigue, atrophy, weakness → progresses to complete paralysis

What are the 2 types of onset symptoms?

Limb: LE first then UE, distal before proximal

Bulbar: speech, chewing, swallowing impairments

What is Phase 1 (Independent) of ALS?

Stage 1: minimal weakness/clumsiness

Stage 2: increased extremity weakness, changes in ADL function

Stage 3: severe weakness in distal, respiration starts to be affected

What is Phase 2 (partially independent) of ALS?

Stage 4: performs most ADL’s, fatigues easily, w/c for mobility

Stage 5: severe weakness, risk of skin breakdown

What is Phase 3 (dependent) of ALS?

Stage 6: bed bound, respiration severely compromised

ALS Clinical Presentation

Combo of LMN & UMN loss; weakness is cardinal sign & is asymmetrical & focal

LMN: isolated muscles, distal → proximal, cervical extensor weakness

UMN: decrease with disease progression

What is sialorrhea?

Excessive saliva

What type of Respiratory & Cognitive Presentations can occur in ALS?

Decreased vital capacity, daytime sleepiness, morning headache due to hypoxia

Frontotemporal dementia: decreased function, difficulty planning, personality, behavior changes

ALS Medical Management

Riluzole: anti-glutaminergic glutamate blocker

Primarily palliative & directed at specific signs & symptoms

Exercise for ALS patient

Mod resistance for muscles >=3/5, avoid heavy eccentric exercise

NO resistive exercise with muscles <3/5

ALS Scales & Outcomes

ALS Functional Rating Scale; lower scales → more involved

Tinetti, BERG, TUG

What is Huntington’s Disease?

Autosomal dominant neurodegenerative disease; only need one copy of the gene to develop

What does Huntington’s Disease affect?

Neuronal cell loss due to excess glutamate → caudate & pudamen in BG & cortex & thalamus

What is the triad of HD?

Motor, cognitive & psychiatric signs

Pre-Manifest of HD

Motor: mild gait changes

Cognitive: difficulty with complex thinking task

Psychiatric: depression, aggression, irritability

Early stage of HD

Motor: mild chorea, decreased rapid alternating mov’t, increased muscle stretch reflexes, abnormal extraocular mov’t

Cognitive: mild problems with planning, sequencing, organizing, prioritizing tasks

Psychiatric: sadness, depression, irritability

Middle stage of HD

Motor: chorea, dsytonia, rigidity/spasticty, voluntary mov’t abnormalities, decreased coordination, difficulty holding things, balance deficits/fall, dysphagia/dysarthia

Cognitive: intellectual decline, memory loss, perceptual problems, lack of insight or self-awareness, difficulty with dual tasking

Psychiatric: apathy, preservation, impulsivity, antisocial/suicidal behavior, paranoia, delusions/hallucinations

Late stage of HD

Motor: bradykinesia, rigidity/spasticity, severe voluntary mov’t abnormalities, dysarthia/dysphagia, incontinence

Cognitive: global dementia

Psychiatric: deliriu

What are examples of dystonic postures?

Torticollis, opisthotonus(head, neck, trunk extension), arching of feet

HD Medical Management

Symptom management: anti- choreic, psychotic, depressant, epileptic

What is Post Polio Syndrome?

Progressive or new muscle weakness & lack of endurance, cold intolerance, profound fatigue & pain

What is the criteria for PPS?

Previous diagnosis of polio, neurological & functional recovery >15 yrs, slow onset of new or amplified muscle weakness, fatigue & muscle atrophy

Pathology of PPS

Polio damages motor neurons in anterior horn of SC → new fibers sprout & promotes recovery of use of muscle but stress causes gradual breakdown of fibers

PPS Clinical Presentation

Fatigue (most common), triad w pain & strength loss

Cold intolerance

New weakness; asymmetrical, proximal & slowly progressive

Myasthenia Gravis Treatment

Meds: anticholinesterase → stop breakdown of ACh, plasmapheresis, immunosuppressants

PT: energy conservation, strengthening w isos