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CLINICAL PRESENTATION OF PATIENT DX WITH RAMSAY HUNT SYNDROMEBy Jere Hess
What is Ramsay Hunt Syndrome (RHS)
a.k.a Herpes Zoster Otticus Shingles attack C.N VII near one of the ears Caused by same virus as chickenpox (Variclela-
Zoster Virus) Lies dormant in nerves for years and if the virus
reactivates and affects C.N VII the result is RHS Classified as a rare disease by the Office of Rare
Diseases of the National Institutes of Health Fewer than 200,000 affected out of est. 300 million
people
Signs of RHS 2 primary signs
Painful red colored rash containing fluid-filled blisters on, in, or around ear
Facial weakness or paralysis Occurs Ipsilateral to side of affected ear
Other S/S Hearing loss Tinnitus Vertigo Changes in perception of taste Difficulty closing one eye Ear pain
Who is Affected and Risk Factors by RHS
Anyone who has had chickenpox can develop RHS
More commonly Post menopausal women over 60 Anyone with a weakened immune system Head traumas
Complications Hearing loss and facial weakness
Can be permanent but more often temporary Damage to eye (eye pain and blurred vision)
Occurs secondary to facial weakness Incomplete eye closure causes damage to cornea
Postherpetic neuralgia Occurs when shingles virus damages nerve fibers
causing pain Can endure after all other S/S of RHS have been
eliminated
RHS Treatment Initially
Anti-viral drugs Corticosteroids Anti-anxiety meds (vertigo symptoms only) Pain relievers
Long-term PT may be prescribed to restore functional capacity
Dx RHS First
Medical History and Physical Exam to identify unique s/s Next
PCR (Polymerase Chain Reaction) test Take sample of fluid from blisters on the ear Can also use blood or tear sample (fluid from blister is more
accurate) Misdiagnosis
Why? B/c of rarity of RHS What? Most commonly misdiagnosed as Bells Palsy
B/c of sudden onset (less than 48 hours) of facial paralysis Vertigo and otalgia are often disregarded as severe BP or
med side effects Also misdiagnosed as bacterial ear infection, flu, or
inflammation of sinus
Patient History Onset began May 1, 2014
Pt. spontaneously experienced nausea vomiting, dizziness, and left facial paralysis
Hospitalized for 9 days Upon discharge from hospital, pt required a walker for
ambulation Secondary to vestibular deficiencies
Pt. stated when symptoms were most severe, unable to move head in any direction without vomiting
Pt. underwent a variety of Tx before reporting to outpatient PT
Initial Evaluation Pt. reported to outpatient PT January 8, 2015 Diagnosis:
Left Ramsay Hunt Syndrome Impaired VOR (Vestibular-occular-reflex) Impaired balance and gait
Clinical Assessment read… RHS affected pt’s Left Cranial nerve VII and VIII causing
complete left lateral facial paralysis accompanied by nausea vomiting, dizziness, and slight left hearing loss. Pt. showed no s/s of acute BPPV (Benign Paroxismal Positional Vertigo). The patient did present with stable left unilateral peripheral loss with impaired static and dynamic balance, impaired VOR, and slightly decreased oculomotor control
Pt. reported Condition was improving but dizziness still occurred daily
during movement
Cranial Nerve VII a.k.a Facial Nerve Mixed Nerve
(both sensory and motor components) Sensory portion
Axons in taste buds of anterior tongue Axons from proprioceptors in mm’s of the face and scalp Functions include taste and the innervated mm’s proprioception of
touch, pain, and temperature Motor portion
Axons of somatic motor neurons that innervate facial, scalp, and neck mm’s, plus parasympathetic axons that stimulate lacrimal and salivary glands
Functions include facial expressions and secretion of tears and saliva
Cranial Nerve VIII a.k.a Vestibularcochlear Nerve Sensory Nerve 2 branches
Vestibular and Cochlear Cochlear Branch
Axons from the organ of corti Function is hearing
Vestibular Branch Axons from the semicircular canals, saccule, and utricle Function is equilibrium
Clinical Assessment and C.N VII and VIII
Left Lateral Facial Paralysis Due to infection of C.N VIII
Slight hearing loss Due to infection of cochlear branch of C.N VIII
Impaired VOR, static, and dynamic balance Due to infection of vestibular branch of C.N VIII
Vestibular System Equilibrium
Balance Spacial orientation
Innervated by Cranial Nerve VIII Primary organs
Semicircular canals, saccule, and utricle
Vestibular System (Semicircular Canals)
3 semicircular canals Anterior, posterior, and horizontal Should only contain endolymphatic fluid Detect angular rotation of the head
Collectively the anterior and posterior canals are called the verticle semicircular canals Detect flexion and extension of head in saggital plane Nodding head to say “yes”
Horizontal Canal Detects rotation of head in transverse plane Rotating head to say “no”
Vestibular System (Saccule and Utricle)
Referred to as Otolithic organs Detect Horizontal and Verticle displacement Saccule
Responds to verticle displacement as in jumping rope Utricle
Responds to horizontal displacement Contains crystals
Another important note Left unilateral peripheral loss was stable Vestibular system has ability to utilize the unaffected side to “make
up” for deficiencies of the affected side In this case pt. contralateral side of infection (Right) could
strengthen to make up for deficiency of Left side B/c it was stable the affected side (Left) any strengthening on the
unaffected side (Right) would improve the overall functioning of the vestibular system
Functional Measurements of Initial Assessment Romberg firm and foam surface Eyes Close
moderate sway Tandem firm surface eyes closed
Maintained for 5 seconds before losing balance Single leg balance Eyes closed
Unable to perform VOR Test (static and dynamic) using eye chart
Static results 20/20 Verticle results 20/30 Horizontal results 20/50
Other important notes of initial assessment Pt unable to drive When ambulating eyes closed pt. would walk in circles. Loss of Independence (driving, grocery shopping, etc…)
Long Term Goals Romberg firm and foam surface eyes closed with
minimum sway Tandem firm eyes closed for 15 seconds
minimum before losing balance Single leg balance eyes closed 7-10 seconds Horizontal VOR 20/30 Driving and ADL’s goals were set at subsequent
reevaluations
Reevaluation February 24, 2015 (47 days after initial assessment) All LTG’s from Initial Assessment were met except
single leg balance eyes closed and horizontal VOR Single leg balance eyes closed had improved from unable
to perform to 3-5 seconds. Did not reach goal of 7-10 seconds
Horizontal VOR improved from 20/50 to 20/40. Did not reach goal of 20/30
All LTG’s that were met were advanced with each subsequent assessment as they were met and goals involving other functional assessment tools were added
Current Pt. Progress Romberg firm eyes closed
Initial: Moderate sway. Current: No sway Romberg foam eyes closed
Initial: Moderate sway. Current: Minimum sway Tandem firm eyes closed
Initial: 5 seconds. Current: 30 seconds Single leg balance eyes closed
Initial: Unable to perform. Current: 25 seconds Can perform single leg balance eyes closed with head turns
Driving Initial: Unable. Current: 15 mins. of driving (including highway)
Pt. has been able to resume other activities Grocery shopping, hiking, walking stroller
How the Improvements Were Made. Treatment Plan.
Vestibular strengthening and Neuromuscular Re-ed.
When looking at functional measurements of initial assessment it is important to note what is being measured
Maintaining balance involves 3 systems Proprioception, Visual, Vestibular
Visual Visual is dominant system used for
balance Eyes open allows for use of Visual in
balance Eyes closed eliminates use of Visual in
balance Results in vestibular and proprioception
increasing their function to maintain balance
Proprioception Firm surface and “normal” stance is least
challenging Treatment challenged proprioception by
tandem stance, single limb support, foam surface When challenged it places the
proprioceptors at a deficit which makes available systems (vestibular and visual) increase function to maintain balance
Vestibular Rotation of Head in Transverse Plane
Challenges the horizontal semicircular canal Flexion Extension of Head in Saggital Plane
Challenges the anterior and posterior semicircular canals
Verticle displacement Challenges the saccule
Horizontal displacement Challenges the utricle
Challenging any of these movements places the associated organ at a deficit which requires the other organs of vestibular system, visual, or proprioceptors to increase function
Examples
Tandem on Foam Eyes Open
Tandem on foam. Challenges the proprioceptors putting them at a deficit. Eyes open allows full visual use. And no head movement or displacement allows full use of vestibular system.
Examples
Single limb support
Eyes closed Horizontal head turns
Single limb support challenges the proprioceptors thus placing them at a deficit. Eyes closed eliminates use of visual function. Horizontal head turns challenge the horizontal semicircular canal thus placing it at a deficit. So the primary function for balance of this movement would come from the remaining organs of vestibular system (Ant. and post. canals, utricle, and saccule.
Other Treatment Strategies Jumping on trampoline and jumping rope
Causes verticle displacement Challenges saccule
Single leg ball pick-ups Single leg balance ball toss
Ambulation Training Walking forward and backward Tandem (toe to heel) Braided walking Walking w/ 180 degree turns Ladder work multiple patterns Jogging
VOR Treatment Patient performed VORx1 and VORx2
Eventually progressed to both in combination with forward walking ambulation
Pt. position is holding object with a single letter with shoulder flexed to 90 degrees and elbow extended Object was standard post-it note
VORx1 Pt. keeps gaze fixed on an object while rotating head
approximately 45 degrees in transverse plane VORx2
Pt. keeps gaze fixed on an object while rotating their head in one direction, as the object is moving simultaneously in the opposite direction of the head
VORx1
Head rotated 45 degrees to the
right.Gaze fixed on
object
Starting position. Gaze fixed on
object. Head in neutral position
Head rotated 45 degrees to the
left. Gaze fixed on object
VORx2
Starting position. Gaze
fixed on object. Head in neutral
position
Head rotating to right while object moves to left. Gaze stays fixed on
object
Head rotating to left while
object moves to right. Gaze
stays fixed on object