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