ramsay hunt syndrome (rhs)

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  • CLINICAL PRESENTATION OF

    PATIENT DX WITH RAMSAY HUNT

    SYNDROME

    By 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 pts 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 mms of the face and scalp

    Functions include taste and the innervated mms proprioception of touch,

    pain, and temperature

    Motor portion

    Axons of somatic motor neurons that innervate facial, scalp, and neck mms,

    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 ADLs goals were set at subsequent reevaluations

  • Reevaluation

    February 24, 2015 (47 days after initial assessment)

    All LTGs 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 LTGs 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

    Chal

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