SYSTEMIC OPHTHAL NOTES

                         

                                                 SYSTEMIC

             OCULAR  LESIONS IN AIDS [D-02,J-04,]

1. OCULAR ADNEXA-

1. HERPES ZOSTER OPHTHALMICUS-

• Varicella zoster
• UL
• Any age
• More sev in immunocompromised
• C/F- Pain –V N

          -Maculopapular rash on forehead→vesicles→pustules→crusting ulceration

          -Periorbital edema

*  T/T-

Systemic-

1.  T.Acyclovir 1gm TDS  Or

2.  T. Famciclovir 250mg TDS / 750 mg OD

Topical-

1. acyclovir cream 3% TDS

    2. Hydrocortisone 1 %  + Fusidic acid 2%  or   Oxytetracycline 3%  till crusts separate.

2. KAPOSI’S SARCOMA-

• Human herpes virus-8
• Affects lids & conj
• Vascular tum
• Pink, red-violet to brown small lesion or large , rapidly growing tum that may ulcerate & bleed
• T/T- RT or Excision

3. MOLLUSCUM CONTAGIOSUM-

• Pox virus
• Pale, waxy, umbilicated nodule.
• IL chr follicular c’vitis [ d/t viral shedding in fornix]
• T/T- 1. Shave excision
• 2. Cautery /cryo /laser

4. Conjunctival microvasculopathy

• Sludging, dilated, tortuous, comma shaped Vs

5. Allergic C’vitis

2. ANTERIOR SEGMENT LESION-

1. DRY EYE-

• D/T- systemic malabsorption of nutrients to maintain a healthy tear film / toxicity from sys drugs.
• Burning sensation & watering
• T/T- Tear subs & punctual occlusion

2. INFECTIVE KERATITIS-

HERPES ZOSTER OPHTHALMICUS-

1. Acute phase-

• Skin rash
• Pain
• Influenza like illness
• Keratitis-

1. Acute epithelial keratitis- Dendrite / stellate lesion
2. Nummular keratitis- Subepithelial deposits surrounded by halo of stromal haze
3. Disciform keratitis

2. Chronic phase-

• Keratitis-

1. Nummular keratitis- Peripheral lesions form facets which become vascularized & infiltrated with lipids
2. Disciform keratitis
3. Neurotrophic keratitis- Ulceration→sec bact inf→ perforation
4. Mucus plaque keratitis

3. Recurrent phase.

T/T-

1. T. Aciclovir 800mg 5 times / day for 10 days
2. Oral analgesics
3. Topical Hydrocortisone 1% + Fusidic acid 2 % [ or oxytetracycline3 %] till the crusts separate

HERPES SIMPLEX KERATITIS-

• Herpes virus DNA
• Primary ocular infection-
• Follicular C’vitis
• Blepharoconjunctivitis
• Sec canalicular obstrn

• Epithelial keratitis-
• Dendritic ulcer- Linear branching ulcer with terminal end-bulbs.

• Disciform keratitis-

-Central zone of epith edema overlying stromal thickening

- Surrounding Wessely’s ring of stromal ppts

• Stromal necrotic keratitis-

-Cheesy necrotic stroma

  -Ant uveitis with KPs underlying active stromal infiltration

T/T-

1. T Aciclovir 400mg 5 /day
2. Topical- Aciclovir e/o- 3 %- 5 / day for 14 days

       -   Ganciclovir 0.15 %   e/ gel   5/ day

       -  Trifluorothymidine 1 %   e/d   2 hrly

3. Debridement

MICROSPORIDIAL KERATITIS-

• Obligate intracellular protozoa
• Bil diffuse chr. Punctate epith keratoconjunctivitis
• T/t- Topical Fumagillin

             -Oral Albendazole

             - HAART

C] POSTERIOR SEGMENT LESIONS-

1. HIV RETINOPATHY-

• CW spots- MC- result from occlusion of precapillary  

                   arterioles

                  -No effect on V/A

                  - No T/T

• Intraretinal h’age
• Ret telangiectasia
• Vascular tortuosity
• Venous / arteriolar occlusion

CMV RETINITIS- Folder- uvea-pg6

PORN [ uvea-pg7], ARN [uvea-pg8], Toxoplasma [uvea pg9]

FUNGAL ENDOPHTHALMITIS-

CANDIDA CHORIORETINITIS-

• Unilat
• Ocular pain
• DOV
• Floaters
• White, fluffy, chorioretinal lesions with overlying vitritis
• Inf can spread into vitreous-white snow-ball like or cotton ball opacities
• Satellite lesion adjacent to prim lesion
• T/T- IV Amphotericin B 1 gm for 4-6 wks

          -5-Fluorocytosine 150mg/kg daily

          -Ketoconazole 200-400mg/day

          -If no response→  PPV + Intravitreal Amphotericin-B 5μg/0.1ml

    PNEUMOCYSTITIS CARINII CHOROIDOPATHY-

• Extrapulmonary dissemination→ choroidal inv
• Seen in pts receiving inhaled aerosolized pentamidine
• BL
• Flat, yellow,round choroidal lesions
• Vitreous NOT inv
• V/A not impaired even in subfoveal inv
• T/T- IV cotrimoxazole [ Trimethoprim + sulphamethoxazole ] or pentamidine.

CRYPTOCOCCUS CHOROIDITIS-

• Multifocal , yellow-white, choroidal lesions
• Small glistening spheres at vitreoret interface
• ON inv→ Rapid visual loss
• T/T- IV Amphotericin-B 1gm

         -Endoph- IV Amphotericin B + PPV

D] ORBIT-

- burkitt’s lymphoma

- orbital cellulites

E] NEUROPHTHALMOLOGY-

- Cranial N palsy

-Papilloedema

-OA

-Lagophthalmos

             MYAESTHENIA GRAVIS [J-05,D-03]

DEF-

An autoimmune dis in which antibodies mediate damage & destruction of acetylcholine receptors at the postsynaptic juncn in the striated ms

EPID-

• 2F : M
• 3rd decade

PATHOGENESIS-

Antibodies → damage Ach receptors→ impaired neuromuscular contraction→ weakness & fatiguability of skeletal ms [not cardiac / invol ms]

TYPES-

1. Ocular
2. Bulbar
3. Generalised

SYMPTOMS-

• Ptosis
• Diplopia
• Painless fatigue on exercise which worsens towards the end of the day & with inf / stress.

SIGNS-

1. Peripheral ms weakness-arms & legs
2. Facial-lack of expression- Mask face
3. Bulbar- Dysphagia

              -Dysarthria

              -Diff in chewing

4.Resp- Diff breathing

5. Ocular- 90% inv

a)  Ptosis-

*  Insidious

*  Bil & asymmetric

*  Worse at the end of the day & least on awakening

*  Worse on prolonged upgaze

*  If one lid is elevated manually while the pt looks up, the fellow lid shows fine oscillatory movts

* COGAN TWITCH SIGN-

-Brief upshoot of the lid as the eyes saccade from depression to prim pos.

• Positive ice test-

Ptosis improves after ice is kept on the lid for 2 min.

2. Diplopia- vertical
3. Nystagmus
4. Pseudointernuclear o’plegia

INV-

1. EDROPHONIUM TEST-

Short acting anticholinesterase → increases the amt of Ach at the NM juncn→ Transient improvement of ptosis & diplopia

Performed as foll-

• Objective baseline measurement of ptosis & diplopia [hess chart]
• IV Atropine 0.3mg [to counteract muscarinic S/E]
• IV 0.2ml [ 2mg ] Edrophonium HCl
• Wait for 60 sec
• Inject 0.8ml [8mg ]
• Final measurements within 5min & results compared

2. Electromyography
3. Increased ser Ach receptor antibodies level
4. Thoracic CT scan & MRI- for thymoma

 T/T-

1. Anticholinesterase drugs-Pyridostigmine 60mg qid

                                           -Neostigmine

2.  Steroids.[ may precipitate resp crisis],so close monitoring is a must

3. Immunosuppressive – Azathioprine [1-3mg/kg/day]-OD /BD

                                    _  Cyclosporin [2-5mg/kg/day]

4. Plasma exchange

5. Intravenous immunoglobulins

6. Thymectomy

7. For ptosis- BIL  Frontalis sling

Diff from Eaton Lambert syn-

• Eaton Lambert syn-
• Affects pre-synaptic receptor [ MG- postsynaptic]
• Power is increased foll exercise
• Reduced tendon reflex [ nml in MG]
• Seen in oat cell carcinoma

             SARCOIDOSIS

DEF-

Non-caseating , granulomatous disease.

SYSTEMIC FEATURES-

LOFGREN SYN-

• Fever
• Erhythema nodosum
• BL Hilar LNpathy
• Arthralgia
• Acute iridocyclitis

    HEERFORDT SYN-

• Fever
• Parotid gld enlargement            [PUF]
• Uveitis

• VII N Palsy
• LUNGS- Pulmonary fibrosis

                 -Bronchiectasis

                 -BL Hilar LNpathy

-SKIN- EN

         -Lupus pernio-indurated blue plaques

OCULAR FEATURES-

ANTERIOR SEGMENT-

• KCS
• Band KP
• Conj granuloma
• Lacrimal gld enlargement
• Uveitis- granulom / non-granulom

                -Mutton fat KPs

                -Choroidal granulomas

               - Chorioretinitis

               -Parsplanitis

*  Cataract

*  Glaucoma

POSTERIOR SEGMENT-

* Vitritis & snowballs

*  Papilloedema

*  Optic neuritis

*  Neuroretinitis

*  Retinal vasculitis

*  perivascular granulomata & ON granuloma

*  Perivenous exud with CANDLE WAX DRIPPING

*  Ret NV

*  VH

*  BRVO & CRVO

INV-

1. Skin test – Kweim test

                 _  Mantoux

2. Ser. Lysozyme

Ser. Globulin

Ser. ACE

Ser Ca -raised

3. X-ray –chest & phalanges
4. Gallium scan
5. Biopsy –Lacrimal gld & conj nodules
6. BAL-broncho alveolar lavage
7. Bronchoscopy with transbronchial biopsy

T/T- 1)  Steroids- periocular- Triamcinolone acetonide 40mg or

                                            Methyl prednisone 40 mg

                                             ( ant / post subtenon)

                      -Systemic – oral prednisone 1 mg/kg/ day

2) Low dose Methotrexate

            

             NEUROFIBROMATOSIS

                 { PHAKOMATOSIS }

  VON RECKLINGHAUSEN’S DISEASE

• NF-1
• Gene locus- 17q11
• AD

• SIGNS-

NEURAL  TUMORS-

• CNS
• Cranial Ns
• Peripheral Ns
• Sympathetic Ns

SKELETAL-

• Short stature
• Macrocephaly
• Facial hemiatrophy
• Absence of greater wing of sphenoid
• Scoliosis
• Thinning of cortex of long bone

    SKIN-

• Café-au-lait spots- flat , light brown patches
• Axillary freckles
• Fibroma molluscum-pedunculated flabby nodules
• Plexiform  neurofibroma

   OCULAR-

• 1.Orbit- Optic N glioma
•         -Spheno-orbital encephalocele→ Pulsating        

                                                   Exophthalmos

            -Neurilemmoma

            -Plexiform neurofibroma

            - Meningioma

2.Lid-

Neurofibroma – Plexiform

                      _  nodular

3.Iris-

• Lisch nodules
• Cong ectropion uveae
• Mamillations

4.Prominent corneal Ns

5.Glaucoma

Pathogenesis-

• Obstrn of aq outflow by NF tiss
• Dev angle anomaly
• Forward displacement of peripheral iris asstd with NF thickening of CB→ sec angle closure
• Contraction of fibrovascular memb→ Synechial angle closure

   6.  Fundus- Choroidal naevi-watch for melanomatous changes

              -  Ret astrocytoma-no t/t

NEUROFIBROMATOSIS 2

• Gene- 22q12
• BL acoustic tum
• Post subcapsular cataract
• Fundus- Hamartoma RPE & ret

               -Perifoveal epiret membrane

    TUBEROUS SCLEROSIS

• Also k/as- Bournville’s dis
• AD
• Triad -  Epilepsy

             - MR

             -Skin lesions- Adenoma sebaceum

                                 - Ash-leaf spots

                                 - Shagreen patch

                                 - Depigmented naevi

                                 - Fibroma molluscum

                                 -Fibrous plaques on forehead

                                 -Subungal hamartoma

- CNS- Ependymomas

        -Astrocytomas

-OCULAR-

*  Retinal astrocytomas-50%

# white semitransparent or mulberry appearing tum in the superficial retina.

*  Hypopigmented patches on iris & ret

*  Papilloedema & VI N palsy

INV-

1. CBC
2. .CT and MRI-BRAIN
3. EEG
4. Echocardiography
5. X ray chest
6. CT scan –abdomen.

Rx-

1. Genetic councelling
2. Retinal astrocytomas require no t/t

VON HIPPEL LINDAU SYN

[J-07, J-05]

• Gene- Chromosome 3
• Tumors-

-Haemangioblastoma- cerebellum, spinal cord, medulla ,pons

- Renal cell ca

-Phaeochromocytoma

• Cysts- Renal , pancreatic. Hepatic, epididymis, ovary, pulmonary
• Polycythaemia
• Ocular- Capillary haemangioma of retina / ONH →

 --Earliest lesion

--small ,red,orange –red tum with a prominent dilated feeding A & draining V

--Subretinal exud

--SRF

--Total RD

--Macular traction & ERM

INV-

1. CBC
2. Ser electrolytes
3. Urine levels of epinephrine & Norepinephrine
4. MRI brain
5. CT scan –abdo
6. IV fluorescein angio

    T/T-

1. Retinal capillary haemangioblastoma-

• Photocoagn
• Cryotherapy
• Diathermy
• Radiation therapy-plaque RT

                               - Proton beam irradiation

-  Microsurgical resection

-Enucleation ( blind painful eye)

            STURGE WEBER SYNDROME-

• Encephalotrigeminal angiomatosis[ETA]
• Sporadic
• Trisystem- Face

                    - Leptomeninges

                    - Eyes

*  Bisystem- Face& Eyes

                           Or         

                 - Face & Leptomeninges

• Presents at birth.
• SIGNS-

A ) FACE-  Port wine stain-over the V N distribution

B) LEPTOMENINGES-

• Ipsilateral parietal / occipital leptomeningeal haemangioma→

                                                                 - Seizures

                                                                 - Hemiparesis

                                                                 -Hemianopia

- MR

C) EYES-

• Glaucoma [IL]
• Diffuse choroidal haemangioma
• IL Episcleral haemangioma
• Heterochromia iridis

   INV-

1. X-ray – “Tramline” cerebral calcification
2. CT scan & MRI- for haemangioma

   T/T-

1. Intractable seizures→ Subtotal Hemispherectomy
2. Portwine stain→ dermatological laser
3. Choroidal haemangioma-

• Photo- argon, xenon, krypton or dye laser
• TTT
• PDT
• Radiation therapy
• Enucleation

4. Glaucoma- Latanoprost 0.005% HS

                    - Goniotomy- For angle anomalies

                    - Trabeculotomy + Trabeculectomy→

                      Removes barrier to aq outflow

             OCULAR  TUBERCULOSIS

DEF-

Chronic granulomatous infection caused by tubercle bacilli

• Mycobacterium bovis [ bovine]- by milk
• Mycobacterium tuberculosis [human]- droplet inf

MORPHOLOGY-

• Slender rods with branching filamentous forms
• Resemble fungal mycelium ,so k/as mycobact i.e fungus like bacteria
• Do not stain readily , but once stained resist decolourisation with dilute mineral acid – k/as Acid fast
• Aerobic
• Non-motile
• N0n-capsulated
• Non-sporing
• Slow growth

    PATHOGENESIS-

       Inhalation of aerosolized droplets→ Asymptomatic , self-limited pulmonary granuloma→ resolves & becomes dormant→ reactivates later→ disseminates to other organs.

OCULAR TB-

CONJ-

• Phlyctenular c’vitis- small, grey –yellow nodules on bulbar conj near limbus
• Prod refex lacrimation

CORNEA-

• Phlyctenular keratitis
• Fascicular ulcer-
• Phlyctenular ulcer migrates slowly from the limbus to the centre of the cor in a serpiginious way.
• It carries a leash of BVs  which lie in a shallow gutter formed by the ulcer.
• Superficial allergic type of ulcer
• Never perforates
• When the ulcer heals, the BVs attenuate with a dense cor opacity near the apex of the ulcer

UVEA-

• Tuberculous iritis

1. Metastatic [granulomatous]-

• Miliary-small ,yellowish white nodule surrounded by small

               Satellites near the pupillary or ciliary margin

• Conglomerate- Larger, yellowish white tumor

                          - smaller satellites may be present

                          - Nodules contain giant cells

2. Exudative [non-granulomatous]

• Tuberculous choroiditis-Tubercles

                                          -Tuberculomas

Tubercles-Post pole

              - Solitary/multiple

              -Multifocal

             -0.3-3mm dia

             -  Yellowish, grayish or white

             -overlying serous RD

Tuberculomas- Solitary

                      -Grayish white

                      -Raised

                      -2-3 DD

                      -Overlying exud RD

RETINA-

Eale’s disease [retinal periphlebitis]-

• recurrent VH
• young healthy adult males
• Periphlebitis→ intraretinal h’age→vascular tortuousity & collaterals→ NV

INV-

1. Sputum exam for AFB
2. Chest X-ray
3. Montoux test
4. Anticord factor antibody

T/T-

5 anti-TB drugs

1) ISONIAZID [H]- 5mg/kg

s/e – optic neuritis

• hepatitis

2) RIFAMPICIN [R]- 10mg/kg

S/E –Hepatitis

     -  Red –orange urine

3) STREPTOMYCIN [S]-15mg/kg

S/E- Ototoxic

• Nephrotoxic

  4) ETHAMBUTOL [E]- 20mg/kg

  S/E- ON

       -Red-green colour blindness

5. PYRAZINAMIDE [Z]-25mg/kg

    S/E –Hepatitis

          -Hyperuricemia

                                    LEPROSY [J-03, J-02]

DEF-

Chronic granulomatous infn caused by an acid fast bacilli-myco leprae.

MORPHOLOGY-

• Straight / slightly curved rods
• Acid fast
• Gm +ve
• Appears as agglomerates,being bound by a lipid subst- ‘glia’.These masses are k/as- “GLOBI”
• Parallel rows of bacilli in globi gives ‘ cigar bundle’ appear.

CULTURE-

• Specimen collected from –skin, ear lobule, nasal mucosa
• Smear stained with Zeihl-Nelson stain using 5% H2SO4 acid for decolourization
• Grading of smear-
  1-10 bacilli / 100 fields – 1+

1-10 bacilli / 10 fields- 2+

1-10 bacilli /field       - 3+

10-100 bacilli / field -  4+

100-1000 bacilli / field – 5+

> 1000 bacilli & globi / field -6+

Bacteriological index- Total no of + /  no of smear

Morphological index- %  uniformly stained bacilli / total no of bacilli counted

Leprosy – skin

           - nose

           -eyes

           - peripheral Ns

           -bones

           - testes

TYPES-

  1 . LEPROMATOUS-

*  cutaneous

*   decreased CMI

*   Direct ocular inv

2. TUBERCULOID-

• Neural
• Good CMI
• Indirect ocular inv-Neurotrophic & neuroparalytic KP

    PATHOLOGY-

   Tuberculoid form-

   Granuloma + Lack of large no of bacilli [ b/o good CMI]

   Lepromatous form-

   Macrophages with numerous AFB [ b/o decreased CMI]

   Iris pearls- Macrophages filled with bacilli

SYSTEMIC –

SKIN-

• Hypopigmentation
• Erhythema nodosum
• Plaques & nodules

NERVES-

• Skin anaesthesia
• Thickened peripheral Ns
• Facial palsy

DEFORMITIES-

• Saddle-shaped nose
• Leonine facies
• Claw hand [ulnar N palsy]

OCULAR-

LIDS-

• Madarosis
• Trichiasis
• Lagophthalmos

CONJ & SCLERA-

• Conjunctivitis
• Episcleritis
• Scleritis

CORNEA-

• Neurotrophic KP
• Cor anaesthesia
• Keratitis

UVEA-

• Acute iritis –d/t immune complex deposit
• Chronic iritis- d/t direct invasion

• Aq flare & cells
• Fine dust –like KPs
• Collection of lepra bacilli k/as- “ IRIS PEARLS “- adhere to papillary margin & iris surf like a necklace → Pearls slowly enlarge→ Coalesce → Pedunculated → Drop into AC→ disappear → iris atrophy → miosis

INV-

1. Skin biopsy
2. Skin test- Mitsuda reac

  T/T-

1. Trichiasis-

• Epilation
• Electrolysis-A fine electrocautery needle is passed down into the hair follicle & a current of 2 mAmp is passed
• Cryotherapy [ double freeze –thaw at -20 deg C]
• Argon laser ablation [ 50 µm. 0.2 sec, 1000mW]
• Surgery-Full-thickness wedge resection or Anterior lamellar excision

2. Lagophthalmos- Tarsorrhaphy
3. Episcleritis- Fluorometholone e/d qid

                     -oral NSAIDs

4. Scleritis- oral NSAIDS

                - oral steroids

                - Immunosuppressants- cyclophosphomide, azathioprine,cyclosporine

5. Conjunctivitis- antibiotic e/d or e/o

6.Keratitis- Cycloplegic-mydriatic

               -Analgesic

              -Antibiotic e/d

               -BSCL- for perforation → Tiss adhesive→       Therapeautic KP

7. Iritis- Topical mydriatic & steroid

8. Systemic-

T. Dapsone 100mg /day

Or T. Rifampicin [ 600mg]/ Clofazimin [100mg]

        VOGT KOYANAGI HARADA SYN

[J-05, D-01]

Also k/as Uveomeningeal syn

DEF-

Idiopathic multisystem disorder with granulomatous uveitis.

EPIDEMIOLOGY-

• BL
• 4-5th decade
• Females
• HLA-DR-4 & HLA-DW-15
• Pigmented race
• Asians [Japanese]

    VKH-

     VOGT –KOYANAGI- Skin + Ant uveitis

      HARADA – CNS + Exud RD

     C/F- 4 phases

1. Prodromal
2. Acute uveitic
3. Convalescent
4. Chronic recurrent

1. PRODROMAL PHASE-

CNS- Meningitis- Headache & neck stiffness

      -Encephalopathy-Convulsions, paresis & CN palsies

AUDITORY- Vertigo, deafness, tinnitus

2. ACUTE UVEITIC-

Anterior uveitis-

• AC cells
• KPs- small & large

           -Granulomatous

*  Iris nodules

*  Synechiae

Posterior uveitis-

*Exud RD

*  Dalen Fuch’s nodules

*  Sunset glow fundus (depigmented fundus)

*  NV

*  `Vitritis

*  H’age

* Edema- optic disc & macula

3. CONVALESECENT PHASE-

• Localised alopecia
• Poliosis
• Vitiligo
• Sunset glow fundus
• Depigmented limbal lesion- Sugiura’s sign

4. CHRONIC RECURRENT PHASE-

    Ant uveitis is more prominent.

• Mutton Fat KPs
• Koeppe nodules
• Post synechiae
• Gonioscopy-Depigmented CB

                       -NV in ac angle

Posterior uveitis-

• Retinal vasculitis
• Subretinal NV- H’age

   INV-

1. FFA- Dye leakage from RPE & accumulation in subret space- Hyperfluorescent dots
2. ICG- Choroidal NV
3. Ultrasound biomicroscopy-

• Narrow AC angle
• Thick choroid
• Thick sclera

4. CSF analysis-

• CSF Pleocytosis
• Increase CSF protein

    T/T-

1. Steroids-

• IV prednisolone 100-200mg/day for 1 week
• Oral steroids 40-80mg/day for 3 mo ( till FA shows absent leakage)
• Topical 0.1 % Dexamethasone 4-6 times/ day

2. Cycloplegic 2-4 times/ day for 3mo till AC cells clear

If  no response-

3. Immmunosuppressants

• Azathioprine 1-3 mg/kg/day
• Cyclophosphamide 1-3 mg/kg/day
• Cyclosporine 2.5 mg/kg/day

COMPLICATIONS-

1. Sec angle closure glauc
2. Subret NV
3. Exud RD

                 BEHCET’S DISEASE [J-02]

• Idiopathic
• Recurrent
• Multisystem dis
• Young men
• HLA-B-5
• 3rd-4th decade

Presents with aphthous ulcer.

MAJOR diagnostic criteria-

1. RECURRENT APHTHOUS ULCER-

• Painful
• Shallow
• Yellowish necrotic base

2. SKIN LESIONS-

• Erythema nodosum
• Subcutaneous thrombophlebitis
• Papulo-vesicular-pustular rash
• PATHERGY TEST- on puncturing the skin with a needle, a pustule appears
• DERMATOGRAPHISM-on stroking the skin corresponding lines appear.

3. RECURRENT GENITAL ULCERATION-

Penis & scrotum- males

Labia & vagina- females

4. UVEITIS-Both ant & post

MINOR Diagnostic criteria-

1. ARTHRITIS- Knees, ankles, sacroiliac jts
2. EPIDIDYMITIS
3. INTESTINAL ULCERATION
4. VASCULAR- obliterative thrombophlebitis & aneurysm
5. CNS- Brainstem syn

           -Meningoencephalitis

Complete Behcet;s dis- All 4 MAJOR criteria

Incomplete Behcet’s dis-

• 3 MAJOR
• 2 MAJOR + 2minor
• Uveitis + 1 MAJOR
• Uveitis + 2 minor

OCULAR-

• Episcleritis
• Keratoconjunctivitis
• Recurrent acute iritis with hypopyon
• Chr iridocyclitis
• Edema- ret, disc, macula
• Retinitis
• Periphlebitis
• Vitritis
• OA

T/T – Steroids

• Immunosuppressants

             DEMYELINATING DISEASES    [j-04]

MULTIPLE SCLEROSIS-

• Autoimmune dis
• Relapsing-remitting
• Adolescence-mid age
• 2F=M

PATHOLOGY-

• Focal inflamn
• Demyelination & gliosis or scarring
• Lesions are disseminated thru out the brain,so also k/as Disseminated sclerosis
• Selective demyelination with relative sparing of axons-hallmark
• PLAQUES- multiple grayish,sclerotic lesions scattered in the white matter-periventricular
• Medullary sheaths of the nerve fibres are specially attacked,axons being spared

C/F- OCULAR

• Retrobulbar neuritis-Hemianopic & quadrantic field changes
• OA
• Retinal venous sheathing
• Nystagmus [very imp sign]-horizontal,ataxic &increases on extreme lat gaze
• RAPD
• EO ms paralysis
• INO
• VI n Palsy

SYSTEMIC-

• Limb weakness
• Sensory loss
• Paraesthesia
• Vertigo
• Ataxia
• Lhermitte sign-A transient electric shock like sensation shooting down the spine into the legs induced by neck flexion
• CHARCOT’S TRIAD-SIN
• slurred speech
• intention tremors
• nystagmus

INV-M 3V 4AE

1. MRI- head & spine-periventricular plaques
2. CT-scan-perivascular & deep white matter lesions
3. Serum vit B12 levels
4. VDRL
5. ESR
6. Antinuclear antibody
7. Anti-DNA antibodies
8. Angiotensin converting enzyme
9. VEP
10. Automated perimetry

T/T-PIIP

1. Pulse IV methylprednisolone
2. Interferon –B1a or IFN-B1b
3. IV immunoglobins
4. Physiotherapy

NEUROMYELITIS OPTICA [DEVIC’S DISEASE ] [J-03]

• variant of MS-acute & violent form
• BL optic neuritis + Transverse myelitis
• Lumbar /dorsal myelitis

ACUTE DISSEMINATED ENCEPHALOMYELITIS [ADEM]

Young adults

• Fever,headache,drowsiness & convulsions
• EYE- Retrobulbar neuritis

           -Papillitis

           -OA

DIFFUSE PERIAXIAL ENCEPHALITIS [SCHILDER’S DIS]

• infancy & childhood
• Massive demyelination in the cerebral & cerebellar hemisphere f/b gliosis
• Systemic –deafness,psychosis,aphasia,UMN palsy,ataxia
• OCULAR-OccIpital lobe lesion-LOV

                    -Brainstem lesion-nystagmus,ocular palsies & papillary def

GIANT  CELL ARTERITIS

-Diag & T/T-[J-07]

DEF- Granulomatous necrotizing arteritis

-Predilection for- Superficial temporal A

                         -Ophthalmic A

                         -Posterior ciliary

                         -Proximal vertebral

-Severity is astd with the quantity of elastic tiss in the media & adventitia.

-Intracranial As [ little elastic tiss] are spared.

-7-8th decade

C/F-

• Scalp tenderness
• Headache-frontal ,occipital, temporal areas or generalized
• Jaw claudication-ischaemia of masseter
• Polymyalgia rheumatica-pain & stiffness in shoulders,worse in the morn & after exertion
• Occult arteritis-Sudden onset blindness with min systemic upset
• Superficial temporal arteritis-thick,tender,inflamed,nodular As.Pulsation is initially present,but later ceases [strongly suggestive of GCA].best location to palpate-in front of the pinna
• AION
• Rare- amaurosis fugax,CW spots,CRAO,cilioretinal A occlusion,pupil-sparing III n palsy,ocular ischaemic sy

INV-

1. ESR- > 60 mm/hr [not diagnostic]
2. C- reactive protein-raised [   “    ]-2.45mg/dl
3. Temporal A biopsy-

• Steroids shud never be withheld pending biopsy,which shud be ideally performed within 3 days of commencing steroids.Steroids after 7days impairs the nml histology response
• Ideal site- ipsilateral temple as it avoids damage to auriculotemporal N
• Atleast 2.5cm of the A shud be taken & serial sections examined b/o the phenomenon of ‘skip lesions’: segments of histologically normal arterial wall alternate with seg of granulomatous inflamn

Rx-

• IV Methyl prednisolone 1g/day  for 3 days + oral prednisone 80mg/day
• After 3 days oral dose is reduced to 60mg & then 50mg for 1 week each
• Daily dose is reduced by 5mg  weekly until 10mg is reached
• Maintenance daily dose-10mg
• T/t is reqd for 1-2 years
• Azathioprine