Neuro neuromuscular Disease, Vestibular, everything else Flashcards
Duchenne muscular dystrophy is most appropriately classified as a/an:
autosomal dominant disorder
autosomal recessive disorder
X-linked recessive disorder
X-linked dominant disorder
X-linked recessive disorder
Duchenne muscular dystrophy is an X-linked recessive disorder caused by mutations in the dystrophin gene. Since it is X-linked recessive, males are affected clinically and females are usually carriers.
The progression of weakness related to Duchenne muscular dystrophy is most accurately described as:
proximal to distal
The typical progression of weakness in Duchenne muscular dystrophy is symmetrical and proximal to distal. Marked weakness of the pelvic and shoulder girdle musculature typically precedes marked weakness in the distal extremity muscles. Bladder and bowel function is typically spared.
Pathology involving otoconia that loosens and travels into the semicircular canal
Benign paroxysmal positional vertigo
Another name for ear posioning
ototoxicity
Name of inflammation of cranial nerve VIII
Labryrinthitis
Name of a benign tumor on cranial nerve VIII
Acoustic neuroma
Which canal is most commonly affected in benign paroxysmal positional vertigo?
Posterior semicircular canal
Benign paroxysmal positional vertigo occurs when otoconia are detached from the macula and float freely in a semicircular canal. The posterior semicircular canal is most commonly the location of the displaced otoconia due to its anatomical position.
What is spontaneous nystagmus
caused by imbalance of vestibular signals that causes a constant drift in 1 direction
What is peripheral nystagmus
occurs with a peripheral vestibular lesion and is inhibited when vision is fixed on an object
What is the central nystagmus
occurs with a central lesion of the brainstem/cerebellum and is not inhibited by fixation
What is positional nystagmus
occurs with changes in head position
What is gaze evoke nystagmus
occurs when eyes shift from a primary position to an alternative position, indicative of CNS pathology
What is the orientation of the canals?
Horizontal -
– tilt head 30 deg forward (gets both)
– detects rotation about vertical axis (spinning in chair)
Anterior
– rotate 45 deg to L and tip FWD (for L ant)
– detects rotation (bending fwd with rotation)
Posterior
– rotate 45 deg L and tip BKWD (for R post)
– detects rotation (bending backwards with rotation)
Vestibulo-ocular reflex
aka VOR Gain
Eye velocity = head velocity
however fast the head is moving the eyes should be moving in the opposite direction
– Reflex occurs when the head is moving greater than 60 deg per second (less than that gaze stability is maintained with smooth pursuits)
What is nystagmus and how is it named?
– names for the fast beated
– typically will beat to the more neurally active side (healthy side)
– brain perceives higher input from the normal ear and therefore perceives that the head is rotating to that side.
Vertigo definition
false sense of rotation
Causes:
- unilateral vestibular hypofunction
- BPPV
- acute unilateral brainstem lesion (possibly after a stroke)
What is Oscillopsia?
vestibular dysfunction with deficits in VOR
words or things are jumpy. The eyes are adjusting to maintain position.
What is disequilibrium?
- pt feels unsteady but its not visible to others
Causes:
- B vestibular hypofunction
- Chronic unilateral vestbiular hypofunction
- LE somatosensation loss
- upper brainstem/vestibular cortex lesion
- cerebellum and motor pathway
Causes of lightheadedness
- hypoglycemia
- hypotension
- anxiety
- panic disorder
Multiple sclerosis central vestibular issues
- Can affect CN VIII
- presents just like unilateral vestibular hypofunction
post CVA vestibular issues
Brainstem infarction:
- clumsiness, weakness, loss of vision, diplopia, drop attacks, dysarthria
Cerebellar infarction:
- AICA (more hearing loss), PICA, vertebral artery (most common for vestibular issues)
- Vertigo will have other cerebellar signs like dysdiodokinesia or dysmetria
post TBI and vestibular issues
Will often complain of vertigo
Can be from multiple different causes:
- abnormal central processing
- reduced input
- prolonged positioning (body acclimates to supine and so when sitting up they think they are falling)
- Could be BPPV or perilymphatic fistula (both peripheral issues that can be treated)
Arnold Chiari Malformation vestibular issues
- prolapse or displacement of the medulla and cerebellum into the foramen magnum
- will present with other CNS deficits
Cerebellar degeneration vestibular issues
Causes:
- Genetic
- Alcohol induced (can be reversed)
- anti-epileptic drugs
- tumor
Presentation:
- gazed evoked nystagmus
- trouble cancelling VOR
- resting nystagmus
- balance/ataxia
Do PTs treat central vestibular disorders?
If symptoms are stable but uncompensated (MS, CVA) rehab is primary treatment
If symptoms are unstable rehab is adjunctive (Chiari malformation , MS, cerebellar degeneration)
This includes:
- Adaptation exercises
- Fall prevention strategies
- Postural and balance training
If a patient has spontaneous nystagmus but it goes away with fixation it is likely (peripheral or central) vestibular disorder?
Peripheral.
List of peripheral vestibular disorders
vestibular neuritis
labyrinthitis
Meniere’s disease
Acoustic Neuroma
Perilymphatic fistula
Unilateral or Bilateral vestibular disorder
BPPV
Vestibular Neuritis
- Viral insult or ischemia that affects CNVIII
- Presentation: Spontaneous onset of vertigo (hours-days) with nausea, vomiting, and imbalance
- as they get better get oscillopsia
Management: vestibular suppressants and adaptation exercises
good prognosis
Labryinthitis
- Viral or bacterial infection to the labyrinth
- Presentation: Spontaneous onset of vertigo (hours-days) with nausea, vomiting, and imbalance AND auditory symptoms
Management: antibiotics/steroids, vestibular suppressants, adaptation exercises
Good prognosis (variable for hearing)
Meniere’s Disease
- Unknown etiology. Irritation and swelling of the membranous labyrinth
- Presentation: recurrent spontaneous attacks of tinnitus, vertigo, nausea, and imbalance (last 20 min but <24 hrs; avg ~4 hours)
- To dx has to have 2 spontaneous episodes of vertigo (20 min or longer), hearing loss verified by hearing test (on 1 occasion), tinnitus or aural fullness and exclusion of other sensory problems.
Medical management:
- vestibular suppressants (acutely)
- limit sodium
- labyrinthectomy
- gentamicin injections (ototoxin)
- only management no cure
PT is typically not good if Meniere’s is active. Only good for stable patients.
Acoustic Neuroma
- Tumor on CN VIII
- Presentation: hearing loss, tinnitus, imbalance (rarely veritgo)
- Medical management: surgical removal, radisurgery, therapy after to speed up recover
Perilymphatic fistula
- Rupture of the oval or round window that causes leakage of the perilymph into the middle ear
- Causes: TBI, pressure changes, or really loud noises
- Management: rest and limited activity (PT is contraindicated)
Unilateral Vestibular Disorders
- Initial vertigo and nystagmus impairments last 3-7 days (as long as the pt is exposed to light they acclimate to some extent)
*** if there is no recovery after this points assume unstable lesion or central lesion. - Some individuals will have longstanding issues with motion sensitivity, gaze stability, balance, and function (PT)
Bilateral Vestibular Disorders
- unsteadiness, difficulty walking (rarely dizzy or vertigo unless ears or unequally affected),
- oscillopsia, hearing loss, imbalance
- problems reading
- walking in the dark
- turning in small crowded spaces
- showering and washing hair (EC)
- reaching or bending
- keeping balance
- stairs
- driving
Management: PT for postural control and oscillopsia (meds dont help)
Usually caused by ototoxic medications
What is the most common cause of vertigo?
BPPV
When will BPPV feel the symptoms? And what symptoms?
When:
- bed mobility
- washing hair
- working under car
- changing light bulb
- dental chair
- reaching for objects on the floor.
What symptoms:
- Poor balance (positional imbalance)
- Vertigo
Do vestibular suppressants work for BPPV?
NO
What is the Dix Hallpike?
A test for the posterior semicircular canal (and indirectly anterior)
To test the R posterior – long sitting then lay down with head rotated 45 deg to the R and 10-20 deg of extension.
If positive will continue with the Epley maneuver
What direction for nystagmus of the eyes for posterior or anterior canal?
Posterior: UPBEAT and TOWARD affected side.
For R post canal = upbeat and torsion to the R
Anterior: DOWNBEAT and TOWARD affected side.
For L ant. canal = downbeat and torsion to the L.
These will show as opposite
Treatment for the listed examples would the same
Difference between canalithiasis and cupulothiasis
Canalithiasis: otoconia broke away from utricle and are free floating
- onset of nystagmus with slight latency (1-40 secs)
- MOST COMMON
Cupulothiasis: otoconia broke away and are adhered to cupula.
- onset of nystagmus is immediate
- nystagmus will last >30 sec
- Have to dislodge the otoconia before replacing in vestibule.
Epley manuever for the R posterior canal
- long sitting
- head rotated to the right at 45 deg
- lay down with head rotated and extended 10-20 deg
- roll head to 45 deg on L side (keep extension)
- roll on L side with looking down
- sit up to short sitting keep rotation
- then look straight
hold each position for ~1 min. If nystagmus stops early you can move on.
** This is same treatment for L anterior SCC**
Brandt-Daroff Exercises
- NOT as effective as Epley manuever
- can help move debris out of canal and can be used as HEP. (5-10 days, 3x/day for 2 weeks or until no symptoms for 2 consecutive days)
- short sitting and rotate head one direction and lay down the other direction. Maintain until dizziness subsides or ~1 min then switch side and rotate head other way.
Treatment for Cupulothiasis for the Posterior canal (or anterior)
Called Liberatory Semont Manuever
Then follow up with the Dix-Hallpike and Epley
- rotate head away from involved – go to affected S/L (hold 3 mins) – quickly move through seated and onto other side (don’t switch rotation of head…so will be looking at ground here)– hold 3 min – return slowly to seated position
Testing for horizontal canal?
Roll Test (*don’t do if get positive Dix Hallpike):
- pt supine with head on pillow (elevated to ~30 deg)
- supine, eyes open
- quickly rotate to 1 side (look for nystagmus)
- repeated on other side
- if it shows up it is will typically be after short delay
- the (+) ear is down
- Geotrophic or Apogeotrophic
Geotrophic or Apogeotrophic
For horizontal canal testing
- Geotrophic: eyes are beating towards the ground (canalithiasis) - (canals are in ground)
- Apogeotrophic: eyes are beating towards the ceiling (cupulothiasis) - (cupids fly)
2 main treatments for horizontal canal BPPV
Sidelying Maneuver - aka Gufoni Maneuver (horizontal canal BPPV):
* Patient sits on the side of the mat/bed with head straight forward.
* Tip head back 60 seconds, then return to upright head position. (to get otoconia in a good place)
* Very quickly lie patient down on UNAFFECTED ear in a sidelying position
* Hold for 1 minute or until symptoms gone.
* Very quickly turn head 45° downwards.
* Hold 2 minutes.
* Slowly return to sitting position keeping head in rotated position until upright.
* Repeat (except for tipping head back, DO NOT repeat that) and see if symptoms gone. If so, you’re done. If symptoms still present, then do until symptoms stop.
Lempert 360° Roll: (alternative treatment for horizontal canal BPPV): BBQ Roll
* Head on 20° incline, head rotated 45 deg towards AFFECTED side.
* Hold at least 15 seconds or until symptoms subside.
* Patient’s head is then rotated to the other side at 45 degrees.
* Hold at least 15 seconds or until symptoms subside.
or tucked
* Move patient into prone on elbow position with nose down (head in neutral, NO extension)
* Roll the patient back towards the affected side, into sidelying and help sit the patient back up (head is supported throughout the maneuver)
Prolonged positioning for Horizontal canal?
GEOTROPHIC:
resting in bed for at least 12 hrs with head turned toward the UNAFFECTED ear
APOGEOTROPHIC:
resting in bed for at least 12 hrs with head towards AFFECTED ear (affected = apogeotrophic)
Vertebrobasilar Insufficiency (VBI) S&S, causes, and test
S&S: 5 D’s and 3 N’s
- diplopia
- dysphagia
- drop attacks
- dizziness
- dysarthria
- nystagmus
- numbness
- nausea
Causes: MVA, cervical spondylosis
Test:
- 1 way: supine – neck extension with max rotation – test is positive if symptoms are reproduced with this (not sensitive)
- 2nd way: sitting – thinkers position – go into rotation, lateral flexion, and extension
What you hear in the hx of someone with vestibular hypofunction?
Dizziness &/or vertigo
OR
visual blurring with head movement
OR
Imbalance
(or all 3)
or suspicious if recent infection or ototoxic antibiotic use
What tests will be (+) with central vestibular disorder?
- smooth pursuits
- saccades
- spontaneous nystagmus
- VOR cancellation
What tests will be (+) with unilateral peripheral dysfunction?
Acute:
- spontaneous nystagmus
- active and passive VOR
- head thrust (in direction of lesion)
- VOR cancellation
- Dynamic visual acuity (DVA) - 3 or more lines
Chronic:
- Dynamic visual acuity (DVA)
What tests will be (+) with B vestibular dysfunction?
- active and passive VOR
- head thrust (usually bilaterally)
- Dynamic Visual Acuity (DVA) - will go up more lines than unilateral hypofunction.
Gaze evoked nystagmus
may be typical or CNS pathology
- have them look at target at 3 different position
Head thrust test
- tilt head fwd 30 deg and rotate small movement to get them to relax and then move quickly in one direction and then the other
- (+) if eyes fall off target
- vestibular hypofunction
- Affected ear is on the side that the head was quickly rotated towards (so if you rotate to the R and eyes fall off and then come back then the R ear is hypofunctional)
- this is testing the VOR
Gaze stability exercises
- VOR x1 (head moving; target still)
- VOR x2 (head moving; target moving)
- Gaze shift with active head movement (look at object then turn head)
- Remember the target (look at target, close eyes, move head, keep eyes where target is and open to see how you did)
What aggrevates the symptoms of pt with BPPV?
- bed mobility (rolling)
- washing hair
- working under car
- changing light bulb
- dental chair
- reaching for object on the floor
What would the eyes do if it is R posterior canal?
When rotated to the R UPBEAT and torsion to the R (to affected side).
What would the eyes do if it is L anterior canal?
When rotated to the R DOWNBEAT and torsion to the L (to unaffected side)
What to be cautious of with anterior canal?
- could be a brainstem or cerebellar lesion
- check CN (facial, trigeminal, hypoglossal)
- cerebellum (non-equillibrium test)
Bells Palsy
- Full hemi facial paralysis due to Facial nerve irritation (CNVII)
- Limited or no sensation changes
- Eye stuck open (because CNVII) closes the eye
- Resolves in several months usually
- Inflammation of the facial n. stress, autoimmune, or unknown
Trigeminal Neuralgia
- CN V
- decreased sensation to forehead, cheek and jaw
- decreased and corneal reflex (absent)
- weakness of temporalis and masseter m.
- facial pain or numbness
- Unilateral or B
- Exacerbated with stress or cold
Corneal Reflex test
- touch cornea with cotton to determine sensory vs motor
- Sensory – CN V Trigeminal
- Motor – CNVII Facial
Provide stimulus to R eye and it doesn’t close but L does = R VII Bells Palsy
Provide stimulus with no response B. R sided CN V problem.
Rinne test
put tuning fork at mastoid process. When sound i s no longer hear move the tuning fork 1-2 cm away from auditory canal until no longer heard.
Normal ratio – air conduction 2x longer than bone conduction
Waldron test
place on forehead and see if the hear equally in B
