presentation diag dilemma

Clinical ChallengeClinical Challenge

Episodes of Episodes of headache in pastheadache in past

BUTBUT

Nature of current Nature of current headache was headache was entirely different from entirely different from previous episodesprevious episodes

Weapons of mass Destruction were

Not found

Hurricane KATERINA Created havoc

Due to mismanagement

OPEC denies to raiseOil production

RECESSION IS RUINING US ECONOMY

Case presentationCase presentation

Dr. Sunil KumarDr. Sunil Kumar FRCS MSFRCS MS

Classical Differential DiagnosisClassical Differential Diagnosis

Painful red eyePainful red eye– Microbial keratitisMicrobial keratitis– Acute iridocyclitisAcute iridocyclitis– Acute congestive Acute congestive

glaucomaglaucoma

Painful red eye with Painful red eye with headacheheadache– MigraineMigraine– SinusitisSinusitis– Orbital inflammatory Orbital inflammatory

diseasesdiseases

Clinical ChallengeClinical Challenge Painful red eyePainful red eye

withwith

Severe headacheSevere headache


At 2 AM.At 2 AM.

35 year-old-male35 year-old-male

Unbearable pain in right eyeUnbearable pain in right eye


vomitingvomiting

Apparently the patient was restless and Apparently the patient was restless and suffering.suffering.


Presumed diagnosis- MIGRAINE ATTACKPresumed diagnosis- MIGRAINE ATTACK

Inj. Diclofenac was givenInj. Diclofenac was given

Advised to consult internist in morningAdvised to consult internist in morning

Patient came back in morningPatient came back in morning

With agonizing pain in Rt. eye, With agonizing pain in Rt. eye, severe headache, vomiting severe headache, vomiting

and watery rhinorrheaand watery rhinorrhea

Patient was admittedPatient was admitted

For further evaluationFor further evaluation

Detailed systemic Detailed systemic examinationexamination

Not rewardingNot rewarding


CBCCBC

ESRESR

Renal function testRenal function test

Liver function test Liver function test

Serum electrolyte were within normal limit.Serum electrolyte were within normal limit.

ENT examination was ENT examination was

unremarkableunremarkable

Detailed ocular examinationDetailed ocular examinationduring pain-free period during pain-free period

was normal except for the was normal except for the conjunctival congestionconjunctival congestion

Normal CT Scan of brainNormal CT Scan of brain

EXCLUDEDEXCLUDED

Intracranial space occupying Intracranial space occupying lesion presenting as headachelesion presenting as headache

Meanwhile patient was treatedMeanwhile patient was treated

Parenteral antibiotics, metroclopramide Parenteral antibiotics, metroclopramide and diclofenac for presumed diagnosis of and diclofenac for presumed diagnosis of meningitis for three daysmeningitis for three days

Symptom complex resolved &Symptom complex resolved &

Patient was discharged in morningPatient was discharged in morning

Patient came back again Patient came back again

Patient returned again same day with similar Patient returned again same day with similar complaints and he was re-admittedcomplaints and he was re-admitted

Patient was re-evaluatedPatient was re-evaluated

History was revised again & patient revealed History was revised again & patient revealed


The pain started suddenly along superior The pain started suddenly along superior orbital marginorbital margin

In no time spreaded to whole head but In no time spreaded to whole head but more intense in right halfmore intense in right half

Within minutes pain was excruciating, eye Within minutes pain was excruciating, eye become red and he started vomitingbecome red and he started vomiting

During episode of painDuring episode of pain

1mm drooping of right upper lid (ptosis)1mm drooping of right upper lid (ptosis)

Extra-ocular movements were fullExtra-ocular movements were full

Conjunctiva was congested & chemosed Conjunctiva was congested & chemosed

Mild anisocoria with right pupil was smallerMild anisocoria with right pupil was smaller

Bilateral disc were pink and well definedBilateral disc were pink and well defined

Normal MRI of brain excludedNormal MRI of brain excluded

Pseudo-tumor of orbitPseudo-tumor of orbit

Tolosa Hunt syndromeTolosa Hunt syndrome

In the clinical settings of In the clinical settings of

Agonizing pain in and Agonizing pain in and around congested around congested eyeeye

Mild ptosisMild ptosis

AnisocoriaAnisocoria

RhinorrheaRhinorrhea


Normal systemic Normal systemic examinationexamination

Normal biochemical Normal biochemical profileprofile

Normal CT brainNormal CT brain

Normal MRI brain and Normal MRI brain and orbitorbit

Diagnosis was revisedDiagnosis was revised

Patient was thought of Patient was thought of suffering fromsuffering from

Rectuls headacheRectuls headache

Treatment was commenced and Treatment was commenced and episode of pain didn’t recur episode of pain didn’t recur

Patient came back again 9 Patient came back again 9 months later with similar months later with similar

complaintscomplaints

Symptom-complex resolved Symptom-complex resolved dramatically on same dramatically on same

treatmenttreatment


Case number 2Case number 2


52 year-old-female52 year-old-female


Episode of excruciating pain around left Episode of excruciating pain around left eye with severe headache for last three eye with severe headache for last three daysdays


Pain started suddenly along left supra-Pain started suddenly along left supra-orbital marginorbital margin

Within minutes pain spreaded to scalp, Within minutes pain spreaded to scalp, around the eye and became unbearablearound the eye and became unbearable

Redness of eye closely followed onset of Redness of eye closely followed onset of pain and lid became ptoticpain and lid became ptotic

But vision remain unaffectedBut vision remain unaffected


Inj. Diclofenac sodium was given to treat Inj. Diclofenac sodium was given to treat the symptom-complex at Talimi.the symptom-complex at Talimi.


Past medical and ophthalmic history was Past medical and ophthalmic history was not remarkablenot remarkable

She didn’t suffer episode of similar nature She didn’t suffer episode of similar nature in the pastin the past

She was not on any chronic medicationShe was not on any chronic medication

Detailed systemic Detailed systemic examinationexamination

Was within normal limitWas within normal limit

Work upWork up

Overt diabetic Overt diabetic

Fasting blood sugar 286 mg%Fasting blood sugar 286 mg%

Work upWork up

CBCCBC

ESRESR

RFT, LFT RFT, LFT

VDRL non reactiveVDRL non reactive

Antinuclear antibodies were not detectedAntinuclear antibodies were not detected

ENT examination and x-ray ENT examination and x-ray para-nasal sinus viewpara-nasal sinus view

excluded sinusitisexcluded sinusitis

She was diagnosed suffering She was diagnosed suffering fromfrom

Painful partial third nerve Painful partial third nerve paresis with diabetesparesis with diabetes

Ophthalmic consultation Ophthalmic consultation

was advisedwas advised

Attack of cluster headacheAttack of cluster headache

Ophthalmic examinationOphthalmic examination


Conjunctival congestionConjunctival congestion

Intra ocular pressure Intra ocular pressure – 32 mm Hg in right eye32 mm Hg in right eye– 30 mm Hg in left eye30 mm Hg in left eye

Well defined optic disc with CD ration of Well defined optic disc with CD ration of

0.7 & 0.6 in right and left eye respectively 0.7 & 0.6 in right and left eye respectively

Diagnosis was revised to Diagnosis was revised to

Painful third nerve paresis Painful third nerve paresis with diabetes with glaucomawith diabetes with glaucoma

Automated perimetry was doneAutomated perimetry was done

Field changes were Field changes were consistent with the consistent with the diagnosis of diagnosis of glaucomaglaucoma

Treatment was commenced Treatment was commenced withwith

Timolol meleate 0.5% eye Timolol meleate 0.5% eye drops twice per daydrops twice per day

IOP dropped down to IOP dropped down to 20 mm Hg20 mm Hg

But episode of But episode of

symptom-complex recurredsymptom-complex recurred

Patient was revaluated againPatient was revaluated again

BCVA 6/6 in both eyesBCVA 6/6 in both eyes

No proptosisNo proptosis

Extra-ocular movements fullExtra-ocular movements full

No clinical evidence of iritis or scleritisNo clinical evidence of iritis or scleritis

Pupil were reactive without any afferent Pupil were reactive without any afferent pupillary defect pupillary defect

GonioscopyGonioscopy

Grade 3 open angle in all quadrantsGrade 3 open angle in all quadrants

Absence of blood in schlemm’s canal Absence of blood in schlemm’s canal excluded the possibility of excluded the possibility of

Low flow carotid-cavernous fistulaLow flow carotid-cavernous fistula

Normal contrast enhanced CT brain Normal contrast enhanced CT brain & orbit excluded& orbit excluded

Intracranial space occupying lesionIntracranial space occupying lesion

Orbital inflammatory diseasesOrbital inflammatory diseases

In the clinical setting ofIn the clinical setting of

Unbearable peri-ocular painUnbearable peri-ocular pain

Conjunctiva congestionConjunctiva congestion


Normal contrast enhanced CT orbit & Normal contrast enhanced CT orbit & brainbrain

Diagnosis was revised to Diagnosis was revised to

Rectules headacheRectules headache

Treatment was commenced Treatment was commenced &&

Episode of pain didn’t recurEpisode of pain didn’t recur

Final diagnosisFinal diagnosis

Both patient were suffering Both patient were suffering from from

CLUSTER HEADACHECLUSTER HEADACHE

Both patient were suffering Both patient were suffering from from


Both patient were treated as per Both patient were treated as per Campbell protocol (Mayo clinic)Campbell protocol (Mayo clinic)

Tablet prednisolone 60 mg/day for three Tablet prednisolone 60 mg/day for three days and tapered by 10 mg every third daydays and tapered by 10 mg every third day

Cluster headache as a cause of painful red eye with headache may be missed both by internist as well as ophthalmologist.

Non clinic, Population based Non clinic, Population based internet survey in USinternet survey in US

Average delay of 6.6 yearAverage delay of 6.6 year

Average number of incorrect diagnosis 3.9Average number of incorrect diagnosis 3.9

Average 4.3 physician were consulted Average 4.3 physician were consulted before diagnosis was madebefore diagnosis was made

Non clinic, Population based Non clinic, Population based internet survey in USinternet survey in US

Average 4% of patients undergo sinus orAverage 4% of patients undergo sinus ordeviated septum surgerydeviated septum surgeryAntibiotics, Propanolol and Amitryptiline Antibiotics, Propanolol and Amitryptiline were commonly prescribedwere commonly prescribed

Klapper JA, Klapper A, Voss T. The misdiagnosis of cluster headache: a nonclinic, population-based, internet survey. Headache

2000;40:730-5.


Most painful primary headacheMost painful primary headache

Episodic patternEpisodic pattern

Attacks occur in series lasting for few daysAttacks occur in series lasting for few days

Remission period lasting for months to Remission period lasting for months to yearsyears

At least five attacks fulfilling criteria A-C:

A. Severe or very severe unilateral orbital or supra-orbital and or temporal pain lasting 15-180 minutes if untreated.

B. Headache accompanied by at least one of the following:

1. Ipsilateral conjunctival injection and/or lacrimation

2. Ipsilateral nasal congestion and/ or rhinorrhea

3. Ipsilateral eyelid edema

4. Ipsilateral forehead and facial sweating

5. Ipsilateral ptosis and miosis

6. A sense of restlessness and agitation

C. Attacks occur in a frequency of one every other day to eight per day

D. Pain not attributable to any other disorder.

International Headache Society criteria for diagnosis of cluster headache

Cluster headache episodeCluster headache episode

Severe unilateral supra-orbital, orbital or Severe unilateral supra-orbital, orbital or temporal pain which may extend to scalp temporal pain which may extend to scalp or neckor neck


Pain start without any warning, Pain start without any warning,

intensify rapidly and intensify rapidly and

becomes agonizing in short timebecomes agonizing in short time


Frequency of attack may vary from six per Frequency of attack may vary from six per day to once in a weekday to once in a week


Attack of pain tend to occur at the same Attack of pain tend to occur at the same hour every day during a bout although hour every day during a bout although additional attack may occur randomly additional attack may occur randomly throughout the day in some patients.throughout the day in some patients.


More than three attacks per day may mask More than three attacks per day may mask the episodic nature of pain. the episodic nature of pain.

It seems that headache recur when the It seems that headache recur when the effects of analgesics wears off.effects of analgesics wears off.

Exact etio-pathology of Exact etio-pathology of cluster headachecluster headache

Still unknownStill unknown

Ipsilateral ventral Ipsilateral ventral hypothalamushypothalamus

Seems to play important role.Seems to play important role.

Cluster headache is a clinical Cluster headache is a clinical diagnosis diagnosis

History of recurrent episodeHistory of recurrent episode

Description of painDescription of pain

Temporal profile of eventTemporal profile of event

Accompanying autonomic menifestationsAccompanying autonomic menifestations

Prednisolone & cluster headachePrednisolone & cluster headache

Corticosteroids are the most rapidly acting Corticosteroids are the most rapidly acting agent to break the cycle of cluster agent to break the cycle of cluster headacheheadache

1. Silberstein SD. 1. Silberstein SD. Pharmacologic management of cluster headache.Pharmacologic management of cluster headache. CNS CNS Drugs 1994; 2: 199-207Drugs 1994; 2: 199-2072. Jammes JL. 2. Jammes JL. The treatment of cluster headaches with prednisone.The treatment of cluster headaches with prednisone. Dis Nerv Dis Nerv Syst.1975;36:375-376Syst.1975;36:375-3763. Couch JR, Ziegler DK. 3. Couch JR, Ziegler DK. Prednisone therapy for cluster headache.Prednisone therapy for cluster headache. Headache 1978;18:219-221 Headache 1978;18:219-221

Sumatriptan & cluster headache Sumatriptan & cluster headache

6 mg. Sumatriptan has been shown to 6 mg. Sumatriptan has been shown to abort the attack of cluster headache abort the attack of cluster headache

But it is contraindicated in patients with But it is contraindicated in patients with hypertension and ischemic heart diseaseshypertension and ischemic heart diseases

If a patient suffer more than two attack per If a patient suffer more than two attack per day the dosage exceed the recommended day the dosage exceed the recommended limitlimit

Take home recipeTake home recipe

High index of suspicionHigh index of suspicion

Take detailed information about sequence Take detailed information about sequence of eventsof events– Severe pain starting around the eye followed Severe pain starting around the eye followed

by redness of eye and headache consider by redness of eye and headache consider cluster headache in d.d.cluster headache in d.d.

Try prednisolone or sumatriptan as Try prednisolone or sumatriptan as therapeutic test if not contraindicated.therapeutic test if not contraindicated.

Thank youThank you

Fodder for the thoughtFodder for the thought

Courtesy: Sunil Kumar Courtesy: Sunil Kumar FRCS,MSFRCS,MS

Traditional D.D. of painful red eyeTraditional D.D. of painful red eye

Microbial keratitisMicrobial keratitis

Acute iridocyclitisAcute iridocyclitis

Angle closure glaucomaAngle closure glaucoma

Painful red eye with headachePainful red eye with headache

MigraineMigraine

Acute sinusitisAcute sinusitis

Orbital infection and inflammatory diseaseOrbital infection and inflammatory disease– Orbital cellulitisOrbital cellulitis– Orbital pseudotumorOrbital pseudotumor

presentation diag dilemma

Health & Medicine

right eye severe headache

left eye

minutes pain

clinical challenge case

onset of pain

episode of pain didnt

normal limit

painfree periodwas normal