Idiopathic Intracranial Hypertension w ith Papilla Edem a & - PowerPoint PPT Presentation

Is there any correlation betw een Idiopathic Intracranial Hypertension w ith Papilla Edem a & Intracranial Venous Disorders? Judit Som lai* , Bernadett Salom váry ** * Unit of Neuro-Ophthalm ology , Department of Neurology & Stroke dr@Som laiJudit.hu Military Hospital, Budapest, Hungary www.Som laiJudit.hu/ en * ** Unit of Neuro-Ophthalm ology National Institute of Clinical Neuroscience , Budapest, Hungary

OCULAR SYMPTOMS – as a precursor of CSF +/ - VENOUS circulatory disease 1. Reversed way of thinking is needed in Neuro- Ophthalm ology because Eye sym ptom s precede and pre-indicate the disorders of Central Nervous System 2. The neuro - ophthalm ological syndrom es help us to find the exact etiology of the IIH syndrom e w ith „ unknow origin” In case of vision loss caused by IIH There is no m orphological sign of disorders on MRI * 3. In case of a negative MRI: Neuro-Opthalm ological exam inations show the Topographic Localisation of the optic nerve’s disorders * 4. The earlier w e diagnose the disease, the better chance to prevent an irreversible visual loss

How rapidly will the optic nerve diseases – with different pathogenesis - cause significant visual loss ?

The long and com plex history of diagnostic criteria of IIH classification Friedm an DI, Jacobson DM (2002). "Dia gnostic criteria for id iop a thic intra cra nia l hy p ertension". Neurology. 59 (10): 1492–1495.( 20 0 2 ) A CHRONOLOGY Modified version of the Dandy PTC criteria of the im portant landm arks (the latest classification in defining the CRITERIA system of the IIH of IIH) Quincke ( 18 90 ) LP: liquor pressure > 25 water cm s • „m eningitis serosa ” - headache, visual loss, papilledema & liquor without biochemical and cytological disorders • etiology: hypersecretion of CSF Nonne (190 4) Clinical sym ptom s of HIP : • Headeache First called as „ pseudotum or cerebri” (PTC) Vom iting Transient visual obscurations Dandy (1937) Papilledem a • Dandy’s criteria of PTC • Modification of the Dandy criteria (1982) No focal neurological signs (exception: paresis of the abducens nerve) Dandy WE (October 1937 ). Annals of Surgery. 106 (4): 492–513. Foley (1955) • No typical signs of the Higher Intracranial „Benign Intracranial Hypertension - BIH „ Pressure (HIP) on CT/ MRI Corbett et al. (198 2) * • „Idiopathic Intracranial Hypertension” (IIH) Norm al CT/ MRI findings without evidence of IC throm bosis Sm ith JL (198 5). • " Whence pseudotum or cerebri?". Unknown etiology of HIP J of Clinical Neuroophthalm ology. 5 (1): 55–6. 198 5.

Com parison of the diagnostic criteria of HIP & IIH in the background of Papilla Edem a The latest classification of Higher Intracranial Pressure (HIP) Intracranial Idiopathic Hypertension in the b a ckground of p a p illa ed em a (IIH) (A m odified version of the Dandy PTC criteria) Significant elevation of LP: liquor pressure > 25 water cm s Intracranial Pressure >>> 25-30 m m Hg Monro-Kellie principles: No typical signs of the HIP 1. Incom pressible: on CT/ MRI Brain - CSF - Blood Norm al CT/ MRI findings (Vbrain+V CSF+V blood=constant) without evidence of throm bosis 2. Disproportion inside the rigid cranium ; Clinical sym ptom s of HIP : CAUSES of the HIP: Headeache Intracranial tum our o CSF secretion-, CSF circulatory-, Transient Visual Obscurations o &/ CSF flow disorder Papilledem a Bra in ed em a o IC v enous circula tory d isord er o Unknown etiology of HIP ! CSF reabsorption problem – sinus thrombosis

of HIP? What is & the actual cause of IIH? Higher Intracranial Relationship betw een : CSF circulation & IC venous circulation Pressure (HIP) Matthew J. Thurtell et coll. Causes An Update on Idiopathic Intracranial Hypertension Rev Neurol Dis . 20 10 Spring-Summer; 7(0): e56–e68. 1. Cerebrospinal Fluid (CSF) secretion CSF : secretion disorder circulation hydrocephalus hy p ersecretiona l resorption hydrocephalus (m eningitis) 2. Disproportion between the volum e + content of cranium intracranial tum our Higher CSF : flow- circulation developm ental disease IC Pressure diseases obstructiona l hydrocephalus (HIP) (IC tum our) 3. IC venous circulatory disorders CSF reabsorption problem : NPH CSF : reabsorption Sa gitta l Sup erior sinus(SSS) disorder s stenosis/ throm bosis Norm al Pressure Hydrocephalus (NPH) SSS throm bosis 4. Primary Intracerebral stenosis of SSS Venous Circulatory Disorders

Where to find the reabsorption disorders within the SSS? IIH : etiopathom echanism – theories in the background of Papilla edem a CSF production CSF - liquor absorption Cerebral Venous & Circulatory CSF pressure= circulatory system Rout CSFform ation+Psss+Vascular com ponent? https:/ / courses.lumenlearning.com/ boundless- ap/ chapter/ protection-of-the-brain/ Diseases: Diseases: liquor absorption disorders HYDROCEPHALUS Non-resorptive HYDROCEPHALUS Diseases : • hypersecretional • obstructional • IC VENOUS CSF ABSORPTION =(P CSF – P SSS ) MALFORMATION R OUT • CSF pressure - manometer • SINUS THROMBOSIS LP - shunt : LP, VP Conrad E Johansson et al. Multiplicity of cerebrospinal fluid functions: King, J.et al.. Cerebral venography New challenges in health and disease and m anom etry in IIH. Cerebrospinal Fluid Res. 20 0 8 ; 5: 10. Neurology, Vol. 45, No. 12, ( 1995 ),

What leads to Throm bosis in the Cerebral Venous System ? IIH:etiopathom echanism – theories : in the background of papilledem a Syndrom e Groups Stenosis and throm bosis of venous sinuses Slowing and stasis of the IC Venous Network IC venous flow Circulatory Disorders The increase of the IC venous blood pressure Throm bosis of Cerebral Sinuses • Cavernosus Sinus (SV) CSF – reabsorption • Venous and Sagittal Superior Sinus (SSS) decrease • arterio-capillar Transversal Sinus (ST) intravascular pressure Norm al Pressure Hydrocephalus HIP with increases. brain swelling CSF- reabsorption disorders – caused by SSS stenosis/ stasis, throm bosis Com pression of Brain infarct venous sinuses Throm bosis in Multiple Sinus

Where to look for the diseases within the Intracerebral Venous Circulatory System w hen the patient has uni-, or bilateral papilledem a ? OCULAR CEREBRAL venous blood supply system venous circulatory system INTRACEREBRAL ORBITAL VENOUS SYSTEM network v. ophthalmica superior (VOS) to cavernous v. ophthalmica inferior (VOI) superficial veins-cortex sinus deep veins of substance DURAL SINUS system CENTRAL RETINAL VEIN Posterior - Superior connection with cavernosus sinus SSS ; SSI, s. rectus , s. transversus, Physiological relationship s. sigm oideus,s. tentorialis, s. occipitalis between CSF circulatory & IC venous Anterior - Superior sinus cavernous, s.intracavernous circulatory s. parietalis, s. basilar, s. sphenoparietal, s. petrosal:sup, inf. Farb RI et al. IIH: The p rev a lence a nd m orp hology of sinov enous stenosis. Neurology V. 60, No. 9, ( 20 0 3 ), p.1418-24 .

The Results of an International FERRO Study On Cerebral Vein and Dural Sinus Throm bosis (ISCVT) Prognosis of Cerebral Vein and Dural sinus throm bosis (624 pts.) José M. Ferro , Patrícia Canhão, Jan Stam, Marie-Germaine BousserB and Fernando Barinagarrementeria; Stroke. 20 0 4 ;35:664-70 Occluded Pts. sinuses % No. / veins (of 624 ) SAGGITAL 313 62,0 SUPERIOR sinus 279 Lateral Sinus 44,7 left headache: 553 (8 8 %) Lateral Sinus 257 4 1,2 * right papillaedem a: 174 Straight Sinus 112 18 ,0 (28 ,3%) * Deep Venous 6 8 10 ,9 visual loss: 8 2 (13,2%) System double vision 8 4 (13,5%) Cortical veins 10 7 17,1 Jugular veins 74 11,9 Cerebellar veins 3 0 ,3 CAVERNOUS 8 1,3 10 Sinus

Clinical Ocular Signs draw our attention to the location of the Cerebral Venous Throm bosis HIGHER throm bosis throm bosis INTRACRANIAL of of PRESSURE CEREBRAL VEINS IC-SINUSES (liquor absorption disorders & venous flow disorders) CAVERNOUS sinus: OPHTHALMIC vein • chemosis, Thrombosis of • ophthalmoparesis pre Sagittal Superior Sinus • exophthalmos Retinal throm bosis trunk • retinal prethrombosis & branch • periferal eye-movement disorders Parietal veins : PAPILLEDEMA SAGITTAL SUPERIOR Sinus PAPILLEDEMA hemiparesis, epilepsy, coma TRANSVERSAL Sinus CORTICAL veins VISUAL FIELD defect (otitis purulent) PAPILLEDEMA paresis n.VI. +retroorbital pain

Which Background Illnesses can cause certain sinus throm boses? Throm bosis of Underlying illness as Causes venous sinuses Septic inflam m ation Aseptic diseases CAVERNOUS • nasal & paranasal sinusitis • cranial trauma, SINUS • • orbital infection facial operation, Dural Arteriovenous Cranial traum a Tum our SAGITTAL • parasagittal fistula (vertex ) SUPERIOR meningeoma (after recanalisation of sinus * ( recidivous) SINUS thrombosis) Prothrom bosis • meningeal cc. Gradenigo pr . Hem atology Mastoiditis TRANSVERSE Sy ndrom e • straight to the sinus syndrom e & SIGMOID ( purulent otitis, & • by vv. Emissaria abduction paresis SINUS sec. Coagulopathy retroorbital pain) Tum ours INTERNAL • surgical • intravascular JUGULAR • extravascular • traumatic VEIN