idiopathic intracranial hypertension

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An interesting case of An interesting case of headache headache G.Balaji med.pg G.Balaji med.pg Prof.Dr.G.sundharamurthy’ Prof.Dr.G.sundharamurthy’ s unit s unit

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Page 1: Idiopathic Intracranial Hypertension

An interesting case of An interesting case of headache headache

G.Balaji med.pgG.Balaji med.pg

Prof.Dr.G.sundharamurthy’s Prof.Dr.G.sundharamurthy’s unitunit

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historyhistory

• 35 year old female presented with c/o 35 year old female presented with c/o headache of 2 weeks duration.headache of 2 weeks duration.

• Headache -generalised throbbing pain Headache -generalised throbbing pain present through out the day. present through out the day.

• Aggravated by bending forward , coughing Aggravated by bending forward , coughing and sneezing. Pain reduced by NSAIDs but and sneezing. Pain reduced by NSAIDs but not completely absent.not completely absent.

• Headache associated with nausea and Headache associated with nausea and vomiting occasionally. vomiting occasionally.

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• No h/o loc, seizures, fever, altered No h/o loc, seizures, fever, altered sensorium, weakness of limbs, hard of sensorium, weakness of limbs, hard of hearing ,tinnitus.hearing ,tinnitus.

• H/o blurring of vision for both distant and H/o blurring of vision for both distant and near objects. No h/o double vision, near objects. No h/o double vision, deviation of eyes to one side.deviation of eyes to one side.

• No h/o trauma, head injury, falls.No h/o trauma, head injury, falls.

• No h/o any drug in take.No h/o any drug in take.

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• Past history: Past history:

not a known case of type 2 dm, sht, not a known case of type 2 dm, sht, ihd, pulmonary tb. ihd, pulmonary tb.

no chronic drug intake. no chronic drug intake.

No similar episodes in the past.No similar episodes in the past.

No h/o any hospitalisation.No h/o any hospitalisation.

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• Personal historyPersonal history::

Nil addictions.Nil addictions.

bowel and bladder habits normal.bowel and bladder habits normal.

• Menstrual& marital historyMenstrual& marital history::

Married with 2 children. Last child birth 7 Married with 2 children. Last child birth 7 years ago. Underwent sterilisation after years ago. Underwent sterilisation after birth of 2 childbirth of 2 child

Menstrual cycles regular.Menstrual cycles regular.

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On examinationOn examination

Patient conscious, oriented, afebrile.Patient conscious, oriented, afebrile.

pulse- 80/ minpulse- 80/ min

Bp- 110/70 mm hg.Bp- 110/70 mm hg.

No pallor, icterus, lymphadenopathy, cyanosis, No pallor, icterus, lymphadenopathy, cyanosis, clubbing.clubbing.

Cvs –S1,S2 heard, no murmurs.Cvs –S1,S2 heard, no murmurs.

Rs – nvbs heard, no added sounds.Rs – nvbs heard, no added sounds.

Per abdomen- soft, no organomegaly. No Per abdomen- soft, no organomegaly. No mass.mass.

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• Cns:Cns:

Pt is conscious, oriented , Pt is conscious, oriented ,

No cranial nerve palsiesNo cranial nerve palsies

Motor- no weakness of limbs. Reflexes normal.Motor- no weakness of limbs. Reflexes normal.

No sensory deficitNo sensory deficit

No cerebellar signs.No cerebellar signs.

No signs of meningeal irritation.No signs of meningeal irritation.

Fundus- bilateral papilledema.Fundus- bilateral papilledema.

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Provisional diagnosis- Provisional diagnosis-

intra cranial hypertension.intra cranial hypertension.

cause?cause?

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investigationsinvestigations

• Complete blood count- normalComplete blood count- normal

• RFT – normal.RFT – normal.

• Chest x ray- normalChest x ray- normal

• ECG– normal.ECG– normal.

• CT brain – no mass ,bleedingCT brain – no mass ,bleeding..

• MRI brain- MRI brain- normalnormal..

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• Ophthal examination-Ophthal examination- Bilateral papilledema.Bilateral papilledema. Enlarged blind spot, peripheral Enlarged blind spot, peripheral

constriction of visual field.constriction of visual field.• Lumbar puncture-Lumbar puncture- opening pressure was high.(more than opening pressure was high.(more than

250 mm water).250 mm water). Cell count, cytology, gram stain -normalCell count, cytology, gram stain -normal

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Final diagnosisFinal diagnosis

IdiopathicIdiopathic intracranial intracranial hypertensionhypertension

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Idiopathic intracranial Idiopathic intracranial HypertensionHypertension

- Pseudotumor CerebriPseudotumor Cerebri

- Benign Intracranial Benign Intracranial HypertensionHypertension

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DefinitionDefinition

1.1. Clinical features of raised Clinical features of raised intracranial pressure (ICP)intracranial pressure (ICP)

2.2. Absence of space-occupying lesion Absence of space-occupying lesion (SOL) on brain imaging(SOL) on brain imaging

3.3. Exclusion of other causesExclusion of other causes

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Physiology of raised ICPPhysiology of raised ICP

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EpidemiologyEpidemiology

• General population = 1 / 100,000 / yrGeneral population = 1 / 100,000 / yr• Women aged 15 – 44 = 3.5 / 100,000 Women aged 15 – 44 = 3.5 / 100,000

/ yr/ yr• Women BMI >29 = 20 / 100,000 / yrWomen BMI >29 = 20 / 100,000 / yr

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Clinical features of Clinical features of Idiopathic Intracranial Idiopathic Intracranial

HypertensionHypertension• HeadacheHeadache• VomitingVomiting• Visual symptoms / signsVisual symptoms / signs

– Transient visual obscurationsTransient visual obscurations– Diplopia (VIth Nerve palsy)Diplopia (VIth Nerve palsy)

• “ “false localising sign”false localising sign”

– Enlarged blind-spotEnlarged blind-spot

• Papilledema on fundus examinationPapilledema on fundus examination• Rest of neurological examination should Rest of neurological examination should

be normalbe normal

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LUMBAR PUNCTURELUMBAR PUNCTURE

• Measure CSF opening pressure with Measure CSF opening pressure with pt lyig in left lateral position.pt lyig in left lateral position.

• If opening pressure elevated, remove If opening pressure elevated, remove enough CSF to decrease closing enough CSF to decrease closing pressure to about 150 mm H2O.pressure to about 150 mm H2O.

• Send for cell count, protein, glucose, Send for cell count, protein, glucose, cultures (bacterial, viral, and fungal), cultures (bacterial, viral, and fungal), and cytology.and cytology.

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NEUROIMAGINGNEUROIMAGING

• MRI preferred over CTMRI preferred over CT• Possible MRI FindingsPossible MRI Findings

– Empty sella – 70%Empty sella – 70%– Flattening of posterior sclera – 80%Flattening of posterior sclera – 80%– Enhancement of prelaminar optic nerve – 50%Enhancement of prelaminar optic nerve – 50%– Distention of perioptic subarachnoid space – 45%Distention of perioptic subarachnoid space – 45%– Vertical tortuosity of orbital optic nerve – 40%Vertical tortuosity of orbital optic nerve – 40%– Intraocular protrusion of prelaminar optic nerve – Intraocular protrusion of prelaminar optic nerve –

30%30%

• Consider Magnetic Resonance Venography to Consider Magnetic Resonance Venography to r/o cerebral venous thrombosisr/o cerebral venous thrombosis

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Identifying papilledemaIdentifying papilledema

Normal Papilledema

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Conditions to ExcludeConditions to Exclude

• SOLSOL• HydrocephalusHydrocephalus• Venous Sinus ThrombosisVenous Sinus Thrombosis• Chronic MeningitisChronic Meningitis

•InfectiveInfective•Inflammatory / granulomatousInflammatory / granulomatous•Neoplastic (Carcinomatous / lymphomatous)Neoplastic (Carcinomatous / lymphomatous)

• ““Medical causes” Medical causes” – COCO2 2 retentionretention– Malignant hypertensionMalignant hypertension

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IMPLICATED ETIOLOGIC IMPLICATED ETIOLOGIC MEDSMEDS• NSAIDSNSAIDS• TetracyclineTetracycline• OCPsOCPs• NitrofurantoinNitrofurantoin• IsotretinoinIsotretinoin• MinocyclineMinocycline• TamoxifenTamoxifen• Nalidixic AcidNalidixic Acid• LithiumLithium• Steroids (stopping or starting them)Steroids (stopping or starting them)

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DIAGNOSIS OF DIAGNOSIS OF PSEUDOTUMOR CEREBRIPSEUDOTUMOR CEREBRI

•Based on Based on modified Dandy criteriamodified Dandy criteria– Awake, alert pt.Awake, alert pt.– Signs and symptoms of increased ICPSigns and symptoms of increased ICP– Absence of localized neurological findings, Absence of localized neurological findings,

except for CN VI paresisexcept for CN VI paresis– Normal CSF fluid findings except for Normal CSF fluid findings except for

increased pressureincreased pressure– Absence of deformity, displacement, and Absence of deformity, displacement, and

obstruction of ventricular systemobstruction of ventricular system– No other identifiable cause of ICP,SOLNo other identifiable cause of ICP,SOL

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How do we make the How do we make the diagnosis?diagnosis?

• Clinical features of raised ICP without Clinical features of raised ICP without apparent causeapparent cause

• Normal brain imagingNormal brain imaging

• Normal imaging of venous systemNormal imaging of venous system

• LP (serves 3 purposes):LP (serves 3 purposes):1.1. Checks pressure – establishes diagnosisChecks pressure – establishes diagnosis

2.2. CSF analysis – excludes infectious, CSF analysis – excludes infectious, inflammatory and neoplastic etiologiesinflammatory and neoplastic etiologies

3.3. Symptomatic improvementSymptomatic improvement

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Associated FactorsAssociated Factors

• Female > MaleFemale > Male• ObesityObesity• DrugsDrugs

– TetracyclinesTetracyclines– Vitamin AVitamin A

• Iron Deficiency AnemiaIron Deficiency Anemia• Endocrine abnormalitiesEndocrine abnormalities

– HypothyroidismHypothyroidism– HypoparathyroidismHypoparathyroidism– PCOS (probably independent of obesity, acne PCOS (probably independent of obesity, acne

treatment)treatment)

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TreatmentTreatment

• Treat risk factorsTreat risk factors– Weight lossWeight loss– Correct endocrine abnormalitiesCorrect endocrine abnormalities– Stop offending medicationStop offending medication

• Medical ( decrease CSF production)Medical ( decrease CSF production)– Carbonic anhydrase inhibitorsCarbonic anhydrase inhibitors– FurosemideFurosemide

• SurgicalSurgical– CSF diversion proceduresCSF diversion procedures– Optic nerve sheath fenestrationOptic nerve sheath fenestration

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““Benign Intracranial Benign Intracranial Hypertension?”Hypertension?” - No longer!- No longer!May lead to irreversible visual lossMay lead to irreversible visual loss

Normal Optic atrophy

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Follow upFollow up

• Symptoms of raised ICPSymptoms of raised ICP

• Neuro-opthalmological assessmentNeuro-opthalmological assessment

- Visual Field TestingVisual Field Testing

- Fundus ExaminationFundus Examination

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treatmenttreatment

• T. acetaolamide 250 mg tdsT. acetaolamide 250 mg tds• T.furesamide- 40 mg od.T.furesamide- 40 mg od.• Lumbar puncture- about 30 ml of csf Lumbar puncture- about 30 ml of csf

drained till 200 mm water pressure drained till 200 mm water pressure reached.reached.

• Patietnt improved with above therapy Patietnt improved with above therapy and was discharged with and was discharged with t.acetazolamide 250 mg tds. To t.acetazolamide 250 mg tds. To review after 15 days.review after 15 days.

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British medical bulletin-june British medical bulletin-june 2006. pg 233-2442006. pg 233-244

• Idiopathic intracranial hypertension and visual function

• James F. Acheson*

• Department of Neuro-Ophthalmology, National Hospital for Neurology and Neurosurgery, London,

and Neuro-Ophthalmology and Strabismus Service, Moorfields Eye Hospital, London, UK

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THANK YOUTHANK YOU