Guillain Barre Syndrome
most common cause of rapidly evolving motor paresis/paralysis and sensory deficits, syndrome of flaccid paralysis and areflexia, some subtypes - most common: Acute inflammatory demyelinating polyneuropathy
immune mediate disease, 2/3 cases have previous acute infection such as campylobacter, influenza, Epstein-Barr virus, CMV, surgery
post polio syndrome: new neuromuscular sxs occur decades after recovery from acute polio
surviving axons innervate many more muscles than originally; weaker system, overuse of muscles with , 5/5 strength
most common disorder of neuromuscular transmission, affects all age groups, autoimmune disorder affecting the neuromuscular junction and motor end plate
dizziness happens with age due to hair cell loss in inner ear, problems can occur in inner ear, vestibular nerve, CNS - brainstem, perceptual problems
causes nystagmus, involuntary, repetitive eye movement
BPPV: episodic intense vertigo related to head position - imbalance and disorientation. Mechanical dysfunction of labyrinths, most common cause of vertigo
50% of cases are idiopathic, Canalithiasis is most common: otoconia "crystals" float in endolymph of Semicircular canals, movement of fluid causes sensation of head rotation causing nystagmus. Lasts 20 seconds to 1 min, sense of spinning.
pathogenesis
lesions appear throughout PNS, from spinal nerve roots to distal motor and sensory fibers. Antibody-mediated demyelination - autoimmune (demyelination at nodes of Ranvier, inflammatory process, Schwann cells divide and remyelinate nerves)
symptoms
declining muscle strength, in previously affected muscles, joint pain, muscle atrophy excessive fatigue
risk factors and pathogenesis
thymic disorders, DM, RA, SLE (lupus), pregnancy, chronic infections, familial disposition
pathogenesis: defect at the neuromuscular junction, number of acetylcholine receptors is decreased, decreased efficiency of neuromuscular transmission
clinical manifestations
vertigo/dizziness/nystagmus, head tilt, disturbance to vision/balance, ataxia and gait disturbance, spatial perception impaired, fear of falling, anxiety, nausea
BPPV: diagnosis, treatment, prognosis
dx: by hx, sxs, Dix-Hallpike Maneuver
treatment: canolith repositioning - using passive movements to move canaliths through semicircular canals (Epley manuver), teach patients home program
prognosis: can cause significant disability even though benign, symptoms remit in one treatment in 85% of cases, resolves spontaneously in 50% by 3 months, but can recur. can have prolonged imbalance after the vertigo stops
clinical manifestations: course of disease
Ascending symmetric distal motor weakness and sensory impairments
parethesia in toes, distal weakness in LEs, weakness progresses to trunk, UEs, facial muscles, breathing, flaccid paralysis with absence of DTRs, "classic form": < 4wks from onset to peak impairment, 90% of cases: sxs stop progressing in 4 weeks, static phase 2-4 wks before recovery, recovery occurs from proximal to distal, recovery takes months to years
diagnosis and treatment
exclude other disorders, EMG.
treat symptoms, modify lifestyle, surgery to correct deformities
clinical manifestations
weakness and fatigue of skeletal muscles, 85% of cases have generalized, proximal weakness (shoulders/hips), spectrum of mild to sever disease (4 major categories: ocular, mild generalized, acute fulminating, late severe), cranial musces especially eyes show weakness first (diplopia, ptosis), difficulty chewing, swallowing, aspiration, head bobbing
diagnosis
dx: presyncope or lightheadedness: orthostatic hypotension or lack of integration with somatosensory system, imaging of brain, visual-ocular system testing
Meniere's Disease; overaccumulation of endolymph causeing volume and pressure changes, pathogenesis
swelling of the endolymphatic compartment of the inner ear, rupture of membranous labyrinth, episodic lasting hrs to days (vertigo, tinnitus and hearing loss, nausea, aural fullness)
diagnosis
symptoms required for dx: progressive weakness in more than 1 extremity, loss of DTRs, CSF features: protein levels increased after 1 wk, contains , 10 mononuclear leukocytes/mm3
symptoms supportive of dx: weakness develops rapidly and ceases to progress by 4 wks, symmetric weakness, mild sensory s/s, facial weakness, recovery begins in 2-4 wks, absence of fever
prognosis
prognosis: slowly progressive disorder, stable periods of 3-10, decreased function = decreased QOL.
diagnosis
R/O hyperthyroidism, intracranial mass, or eye disease. Immunologic dx - anti acetylcholinesterase receptor antibodies, Pharmacologic dx - Tensilon test (inhibits acetylcholinesterase), Electrophysiologic dx - normal EMG at rest, repeated stimuli causes decreased action potential
intervention
treatment depends on etiology, goal is to decrease abnormal sensation of motion and related sxs (n/v, anxiety), sedative in first 24 hrs only, PT vestibular rehab
Meniere;s Disease treatment and prognosis
treatment: management of symptoms, salt restriction, diuretics, corticosteroids
prognosis: clusters of attacks followed by remission, symptoms are reversible early in disease, gradually progress to permanent vestibular and hearing loss
treatment and prognosis
Rx to control the autoimmune response - Plasmapheresis/plasma exchange (plex) - filters antibodies. IV immunoglobulin, corticosteroids have NO beneficial effect, mechanical ventilation, rehabilitation is essential.
PT implications early stages: ROM, monitor muscle strength, east fatigability, avoid complications of immobility, respiratory support, skin care
Recovery stages: active assistive exs; progress to active; then resistive. Aquatic therapy, neuromuscular facilitation, assistive devices, orthotics
PT implications
Never exercise to point of fatigue, monitor vitals for response, orthoses for stability
treatment and PT implication
treatment: improvement with rest, acetylcholinesterase inhibitor, immunosuppressives, thymectomy (85% success, 35% remission), Plasmapheresis for short term.
PT implications: monitor respiratory function, treat side effects of corticosteroids, energy conservation to avoid fatigue, strength training with close fatigue monitoring, family/patient education
prognosis, peripheral or central?
prognosis: unilateral peripheral vestibular problem with CNS intact - function can be restored. Brain begins to adapt in 2 days to 4 weeks to almost normal in 6 wks.
Complete bilateral dysfunction - compensation must occur in other systems (visual, somatosensory)
Central dysfunction recovery depends on lesion and neuro status - MS has progressive dysfunction
Peripheral: unilateral vestibular loss, bilateral vestibular loss, BPPV (usually unilateral but can be bilateral), Meniere's disease, Cervicogenic dizziness (both in peripheral and central)
Central: Migraine, MS, CVA, TBI/Concussion, Cerebellar Degeneration, Orthostatic hypotension
Vestibular rehabilitation
good evidence for effectiveness, PT should understand complexity of vestibular system, critical to identify which aspect of vestibular system for best outcome, particularly canal, vision, inner ear. Interventions: gaze stabilization, balance exercises, visual exercises, functional exercises. Pt education stigma/anxiety of insecurity support