WELCOME

A Middle Aged Woman

with
Chronic Headache
& Vision loss.

Presented by:
Dr. Tanjimul Islam (Ovi)
MD (Thesis) Neuromedicine, RMCH
 Particulars of the Patient:
• Name: Rehnuma Bilkis
• Age: 38 yrs
• Occupation: Housewife
• Marital Status: Married
• Address: Putia, Rajshahi.
• Date of Admission: 18/07/19
 Chief Complaints:

Chief Complaints

1. Headache for 6 months

 Chief Complaints:

Chief Complaints

1. Headache for 6 months

2. Vision loss for 3 months
 H/O Presenting Illness
• According to the statement of the patient, she
was relatively alright 6 months back. Then she
developed Headache which was diffuse,
persistent, daily, mild to moderate in intensity
& associated with nausea. It did not caused
significant impairment in daily activities.
• The Headache was aggravated by light &
partially relieved by taking analgesic. The pain
was radiated towards neck.
 H/O Presenting Illness
• There was also decreased Vision for 3 months.
Patient complaints of blurring of vision &
decreased vision for near objects. There were
also few episodes of black outs in last 3 months.
• She also had history of pain & redness of both
eyes. Pain was dull, intermittent, localized to
eyes with no radiation, aggravating & relieving
factors. Redness was acute, non progressive in
both eyes, associated with photophobia.
 H/O Presenting Illness
• There was also history of whitish discoloration of
skin which was started in eyelids & eyelashes, then
spread to the upper limb & trunk. The lesions are
progressive & aggravated by trauma. There was
also history of Hair loss which progressive & focal.

• The patient also gave history of reduced hearing

which is progressive & associated with tinnitus.
 H/O Presenting Illness
• There was history of 3 times of Hospital admission
& 6 times of Outdoor Visits within last 6 months.

• Her 1st admission was in ‘Medicine’ department 6

months ago, diagnosed as a case of ‘Migraine’ &
discharged with treatment.

• Her 2nd admission was in ‘Neurology’ department

after 1 month, was diagnosed as a case of ‘Acute
Meningitis’ & discharged with treatment.
 H/O Presenting Illness
• The 3rd admission was in ‘Ophthalmology’ department
after 4 months, was diagnosed as a case of ‘Acute
Anterior Uveitis’ & discharged with treatment.

• In all the hospital admissions she was cured completely

with no squeal.

• She was also diagnosed as a case of ‘Segmental

Vitiligo’ from ‘Dermatology’ outdoor & treated with
topical potent steroid.
 H/O Presenting Illness
• There was NO complaints of thunderclap
headache, aura, rhinorrhea & lacrimation.

• NO aggravating factors like loud noise. Rest,

sleep had given no relieve from Headache. NO
history of Painful Eye movement. NO history of
Cough, vesicles, localized headache, trigger
points, or psychiatric problem. NO history of
OCP & other drugs. NO complains of ear pain,
fullness or Ear discharge.
 H/O Presenting Illness
• She had NO history of fever, evening rise of temperature, night
sweats, nausea, vomiting & weight loss.

• There was also NO history of vertigo, facial numbness, itching &

hair loss in other parts of body.

• NO history of Joint pain, Rash, oral ulcer or Morning Stiffness.

• With the above complaints, the patient was admitted to again in

Neurology department of RMCH for better management.
 History of Past Illness
• Hypertensive for 2 years.
• Diabetic for 1 year.

• No history of :
• Asthma, COPD, Jaundice.
• Tuberculosis
• Allergy
• Trauma
 `
Family History:
• All his family members are well.

Drug/ Treatement History:

• Losartan 50 mg daily for HTN.
• Metformin 500 BD for DM
• Topical Steroid (moderate potent) for Vitiligo.
• Amitriptyline 25 mg for Headache.
Personal History:
• Bettel nuts, leaves: 10/day

Socio economic History:

• Lives in Semi pakka House.
• Housewife

Allergy History:
• No history of Allergy

Immunization History:
• No history.
 General
Examination:
General Examination
Appearance Anxious/ Ill looking
Body Built Lean Thin
Co operation Co operative
Dicubitus On choice
Anaemia Mild
Jaundice Absent
Cyanosis Absent
Oedema Absent
Dehydration Absent
Koilonychia Absent
Leuconychia Absent
Lymph Nodes (Cervical, Non Palpable
Axillary, Inguinal)
Bony Tenderness Absent
Respiratory rate 18 / min
Pulse 100 / min
Blood pressure 150/90 mm Hg
Postural drop Absent
Temperature 98.6 F
Weight 58 Kg
 SYSTEMIC
EXAMINATION
 Higher Cerebral Function

Consciousness Level of Consciousness Normal

Attention
Concentration
Orientation

Affect Mood, Behavior Normal

Cognition Language Normal
Memory
Reasoning
Judgment
Abstract Thinking,
Insight
MMSE 28/30 Normal
Emotional Lability Absent
Upper & Lower Limb Examination:
INSPECTION OF UPPER & Rt & Left
• LOWER LIMB
Inspection
Muscle Atrophy Absent
Thigh, forearm
Fasciculation Absent
Dorsal guttering Absent
Skin Changes Absent
Hair Changes Absent
Scar Mark Absent
Pigmentation Absent
Joint Deformity Absent
 Motor System Examination:
• Bulk of the muscle:
Upper Limb Lower Limb
Right Left Right Left
12cm 12cm 13 cm 12 cm

• Tone: Normal
• Power: MRC Grading
LIMBS Right Left
UPPER LIMB 5 5
LOWER LIMB 5 5
 Reflexes :
Jerks Findings
Deep Reflexes Rt Lf
Planter Flexor Flexor
Ankle ++ ++
Knee ++ ++
Supinator ++ ++
Biceps ++ ++
Triceps ++ ++
Superficial Reflexes
Abdominal, Cremesteric ++ ++
Cornael, conjuctival Reflex ++ ++
 Sensory System Examination
Superficial Sensations:
Touch, Pain, Temperature Intact (Normal)
Deep Sensations:
Proprioception, Vibration Intact (Normal)

Discriminative sensory Intact (Normal)

function
Steriognosis, Intact (Normal)
Two point discrimination
Co ordination:
• Finger nose test: Normal
• Heel knee test: Normal
• Involuntary movements: Absent

Gower’s Sign: Absent

Gait: Normal
 Nerve Impingement test:
Medial Nerve
Phalans test Negative
Tinel’s sign Negative
Ulner Nerve Test
Fromet’s Sign Negative
Ulnar Stretch Test Negative
Radial Nerve Test
Nerve Thickening Absent
Addson’s Menuaver Negative
Spine Examination
Tenderness Absent

Deformities Absent

Range of motion Absent

Meningeal Signs
Neck rigidity Absent
Kernig’s Sign
Brudzinski’s Sign:
 Cranial Nerve Examination

Optic (II) Visual Acuity: 6/18

Fundoscopy:
Retinal detachment with
sunset glow appearance
(bilateral)

Trigeminal (V) Normal

3rd, 4th, 6th Normal

8th, 9th, 10th, 11th, 12th Normal

 Ophthalmoscopic Examination
Right Left
Visual Acquity 6/18 6/12
With pinhole 6/18 6/12
Color vision Normal Normal
Extra ocular movements Normal Normal
Lids & Adenexa Normal Normal
Conjunctiva & Sclera Circumcorneal congestion Circumcorneal congestion
Cornea Clear Clear
Ant Chamber Cells grade 3+, Cells grade 2+,
Flare grade 4+ Flare grade 2+
Iris & Lens Posterior Synechie Posterior Synechie
Pupils Round, Miotic Round, Miotic
Fundoscopy Retinal detouchment Sunset glow appearance
Sunset glow appearance
 Ophthalmoscopic Examination
Right Left
Visual Acquity 6/18 6/12
With pinhole 6/18 6/12
Color vision Normal Normal
Extra ocular movements Normal Normal
Lids & Adenexa Normal Normal
Conjunctiva & Sclera Circumcorneal congestion Circumcorneal congestion
Cornea Clear Clear
Ant Chamber Cells grade 3+, Cells grade 2+,
Flare grade 4+ Flare grade 2+
Iris & Lens Posterior Synechie Posterior Synechie
Pupils Round, Miotic Round, Miotic
Fundoscopy Retinal detouchment Sunset glow appearance
Sunset glow appearance
 Ophthalmoscopic Examination
Right Left
Visual Acquity 6/18 6/12
With pinhole 6/18 6/12
Color vision Normal Normal
Extra ocular movements Normal Normal
Lids & Adenexa Normal Normal
Conjunctiva & Sclera Circumcorneal congestion Circumcorneal congestion
Cornea Clear Clear
Ant Chamber Cells grade 3+, Cells grade 2+,
Flare grade 4+ Flare grade 2+
Iris & Lens Posterior Synechie Posterior Synechie
Pupils Round, Miotic Round, Miotic
Fundoscopy Retinal detouchment Sunset glow appearance
Sunset glow appearance
 Ophthalmoscopic Examination
Right Left
Visual Acquity 6/18 6/12
With pinhole 6/18 6/12
Color vision Normal Normal
Extra ocular movements Normal Normal
Lids & Adenexa Normal Normal
Conjunctiva & Sclera Circumcorneal congestion Circumcorneal congestion
Cornea Clear Clear
Ant Chamber Cells grade 3+, Cells grade 2+,
Flare grade 4+ Flare grade 2+
Iris & Lens Posterior Synechie Posterior Synechie
Pupils Round, Miotic Round, Miotic
Fundoscopy Retinal detouchment Sunset glow appearance
Sunset glow appearance
 Ophthalmoscopic Examination
Right Left
Visual Acquity 6/18 6/12
With pinhole 6/18 6/12
Color vision Normal Normal
Extra ocular movements Normal Normal
Lids & Adenexa Normal Normal
Conjunctiva & Sclera Circumcorneal congestion Circumcorneal congestion
Cornea Clear Clear
Ant Chamber Cells grade 3+, Cells grade 2+,
Flare grade 4+ Flare grade 2+
Iris & Lens Posterior Synechie Posterior Synechie
Pupils Round, Miotic Round, Miotic
Fundoscopy Retinal detouchment Sunset glow appearance
Sunset glow appearance
 Dermatological Examination
• Dermatological
Hypo Exam:
• Whitish macules:
pigmented Upper Eyelids, Back.
lesions: • Shape: Round, irregular
Vitiligo • Size: Mixed
• Depigmented hair (Poliosis/ Leukotrichia)
Hair: •Scalp: Alopecia
•Non Inflammatory, Non scarring
•Patchy
•Poliosis: eyebrows
•Other body area: Normal
• Cardiovascular system exam:
• Apex Beat: 5th ICS, MCL
• Heart sounds: Normal.
• Murmur, Thrill: Absent

• Respiratory system exam:

• Trachea: Central
• Breath sound: Vesicular
• Added sound: No
• Abdominal Exam:
Liver, spleen, kidney : Not palpable
Ascites: Absent

• Muskuloskeletal System Exam: NAD

Joint Tenderness: Absent
Bony deformity: Absent

Ear Exam:
External Ear: Normal
Discharge: Absent
Tympanic Membrane: Normal
 Positive Findings
YES
•Neurological Findings:

• Ophthalmologic Findings

• Dermatological Findings:
 Positive Findings
YES
•Neurological Findings:
• Headache
• SN Hearing loss, Tinnitus
• Ophthalmologic Findings

• Dermatological Findings:
 Positive Findings
YES
•Neurological Findings:
• Headache
• SN Hearing loss, Tinnitus
• Ophthalmologic Findings
• Visual acuity: Decreased
• Pain & Redness
• Fundoscopy: Retinal Detachment, Sunset Glow fundus
• Dermatological Findings:
 Positive Findings
YES
•Neurological Findings:
• Headache
• SN Hearing loss, Tinnitus
• Ophthalmologic Findings
• Visual acuity: Decreased
• Pain & Redness
• Fundoscopy: Retinal Detachment, Sunset Glow fundus
• Dermatological Findings:
• Vitiligo
• Poliosis
• Hair loss (Non scarring, Non inflammatory)
Provisional
Diagnosis

?
Provisional Diagnosis
DIFFENTIAL
DIAGNOSIS

?
 Differential Diagnosis

1.
2.
3.
4.
 Differential Diagnosis

1. Cerebello-pontine angle tumor

2.
3.
4.
 Differential Diagnosis

1. Cerebello-pontine angle tumor

2. Chronic Meningitis
3.
4.
 Differential Diagnosis

1. Cerebello-pontine angle tumor

2. Chronic Meningitis
3. Vogt Koyanagi-Harada Syndrome
4.
 Differential Diagnosis

1. Cerebello-pontine angle tumor

2. Chronic Meningitis
3. Vogt Koyanagi-Harada Syndrome
4. ?
INVESTIGATIONS:
 1st Line Investigation
• CBC:
• Hb: 10.1 gm/dl
• WBC: 9000/ cumm
• Platelet: 2.6 lac/ cumm
• ESR: 20 mm 1st hour
• CRP: 2.6
• RBS: 6.8 mmo/l
• S. Creatinine: 0.9 mmol/l
• Urine R/E : Normal Study
Chest X ray ECG
 2 Line Investigations
nd
• :

• TSH: 4.8; T4: 5.6

• S. Electrolytes: Normal

• Imaging Study:
MRI of Brain: Normal Study
MRI of Brain: Normal Study
 2nd Line Investigations
RA test Negative

Anti CCP Ab Negative

ANA Negative

VDRL Negative

Slit Skin Smear Negative

Audiometry Sensory Neural loss

Woods lamp Exam Positive for Vitiligo

Skin Biopsy Absence of Melanocytes

 2nd Line Investigations
RA test Negative

Anti CCP Ab Negative

ANA Negative

VDRL Negative

Slit Skin Smear Negative

Audiometry Sensory Neural loss

Woods lamp Exam Positive for Vitiligo

Skin Biopsy Absence of Melanocytes

Tuberculin Test: Negative
 CSF Examination
Result
Appearance Cristal clear
Pressure 1 drop/ second
Cob web Absent
Cells 20
ADA 2
Protein 35
Glucose 45
Gram Stain & AFB Negative
Malignant Cells Absent
USG of Eye: Focal Retinal Detachment
Optical Coherence Tomography (OCT)
Optical Coherence Tomography (OCT)

Result:
• Exudative Retinal Detachment (Both Eye)
• Retinal Oedema (Both Eye)
 Confirmed Diagnosis:

Vogt Koyanagi
Harada Syndrome
(VKH)
 Management :

General Specific Management

Management
• Analgesics
•Control DM, HTN
 Management :

General Specific Management

Management
• Analgesics •Inj. Methyprednisolone 1 gm daily 5 days
•Control DM, HTN •Tab. Prednisolone 60 mg
•Sub tenon’s injection of Triamcinolone
•Tab. Azathioprine 50 mg
 ‘VKH’
(Vogt Koyanagi Harada Syndrome)
 VKH

‘VKH’ is a Multi-systemic disorder

characterized by Granulomatous
Panuveitis with exudative Retinal
Detachment that is often associated with
Neurological & Skin manifestations.
 Pathogenesis of VKH

• Autoimmune process driven by T Lymphocytes

against melanocytes.

• HLA DR-1 & HLA DR-4 have associations (84%)

• Antigenic peptides are involved

(tyrosinase or tyrosinase related proteins)
 VKH History

• Vogt (1906) Swiss •Poliosis + Ocular inflammation

 VKH History

• Vogt (1906) Swiss •Poliosis + Ocular inflammation

• Koyanagi (1930) Japan •Iridocyclitis + Vitiligo

 VKH History

• Vogt (1906) Swiss •Poliosis + Ocular inflammation

• Koyanagi (1930) Japan •Vitiligo + Iridocyclitis

• Harada (1940) Japan •CSF pleocytosis + Occular

 VKH History

• Vogt (1906) Swiss •Poliosis + Ocular inflammation

• Koyanagi (1930) Japan •Iridocyclitis + Vitiligo

• Harada (1940) Japan •CSF pleocytosis + Occular

• Babel (1945) UK •Vogt- Koyanagi-Harada

 Diagnostic Criteria of VKH (1978)
1. Absence of history of Occular trauma/ Surgery
2. Presence of any 3 of the following
a. Bilateral Iridocyclitis
b. Uveitis with retinal detachment, Sunset glow fundus
c. Neurological signs:
* Neck Stiffness
* Tinnitus
* CSF fluid pleocytosis
d. Skin involvement:
* Poliosis, Vitiligo
* Alopecia
Revised Diagnostic Criteria (2010)
VKH
Revised Diagnostic Criteria (2010)
VKH

Complete Incomplete Probable

VKH VKH VKH
 Revised
Revised Diagnostic
Diagnostic Criteria
Criteria (2010)

VKH

Complete Incomplete Probable

VKH VKH VKH

Bilateral Ocular

Neurological

Skin
 Revised
Revised Diagnostic
Diagnostic Criteria
Criteria (2010)

VKH

Complete Incomplete Probable

VKH VKH VKH

Bilateral Ocular Bilateral Ocular

Neurological Neurological

Skin
Revised Diagnostic
Revised Criteria
Diagnostic Criteria
(2010)

VKH

Complete Incomplete Probable

VKH VKH VKH

Bilateral Ocular Bilateral Ocular Bilateral Ocular

Neurological Neurological

Skin
 Stages of VKH
Prodromal Stage

Uveitic Stage

Chronic Stage

Recurrent Stage
 Stages of VKH
• Headache, Meningismus
Prodromal Stage
• Tinnitus, Hearing Loss
• Nausea, Photophobia

Uveitic Stage

Chronic Stage

Recurrent Stage
 Stages of VKH
• Headache, Meningismus
Prodromal Stage
• Tinnitus, Hearing Loss
• Nausea, Photophobia

• Vision Loss, Blurring

Uveitic Stage
• Retinal detachment

Chronic Stage

Recurrent Stage
 Stages of VKH
• Headache, Meningismus
Prodromal Stage
• Tinnitus, Hearing Loss
• Nausea, Photophobia

• Vision Loss, Blurring

Uveitic Stage
• Retinal detachment

Chronic Stage • Skin Manifestaions

• Sugiura’s Sign

Recurrent Stage
 Stages of VKH
• Headache, Meningismus
Prodromal Stage
• Tinnitus, Hearing Loss
• Nausea, Photophobia

• Vision Loss, Blurring

Uveitic Stage
• Retinal detachment

Chronic Stage • Skin Manifestaions

• Sugiura’s Sign

Recurrent Stage
Sunset Glow Fundus
Sugiura’s Sign
Sugiura’s Sign
Poliosis & Vitiligo
Poliosis & Vitiligo
Poliosis & Vitiligo
Poliosis in VKH
Hair Loss in VKH (typical)
Hair Loss in VKH (typical)
Hair Loss in VKH (typical)
 Treatment of VKH
Acute & Recurrent
Chronic VKH VKH
 Treatment of VKH
Acute & Recurrent
Chronic VKH VKH

Inj. Methyl Prednisolone

Tab. Prednisolone 3-6 months

Topical Steroid Drop

 Treatment of VKH
Acute & Recurrent
Chronic VKH VKH

Inj. Methyl Prednisolone Sub Tenon’s

Immunosupressive
Tab. Prednisolone 3-6 moths injection

Topical Steroid Drop

 Treatment of VKH
Acute & Recurrent
Chronic VKH VKH

Inj. Methyl Prednisolone Sub Tenon’s

Immunosupressive
Tab. Prednisolone 3-6 months injection

Topical Steroid Drop

Triamcinolone
 Treatment of VKH
Acute & Recurrent
Chronic VKH VKH

Inj. Methyl Prednisolone Sub Tenon’s

Immunosupressive
Tab. Prednisolone 3-6 months injection

Topical Steroid Drop

Triamcinolone Azathioprine
Cyclophosphamide
Cyclosporine
Mycophenolate
Sub tenon’s Injection of Triamcinolone
Sub tenon’s Injection of Triamcinolone
 Prognosis of VKH

Good Visual Outcome •After Long term Steroid

Bad prognosis •Long term untreated
•Recurrence
 Prognosis of VKH

Good Visual Outcome •After Long term Steroid

Bad prognosis •Long term untreated
•Recurrence

Complications •Cataract
•Glaucoma
•Optic Atrophy
•Chronic Headache
•Permanent Vision loss
Thank You
VKH