The patient is a 19 y/o AAF who presented with pressure in retro-orbital and frontal regions bilaterally. She also reported blurry vision, wavy vertical lines in both eyes, and lightheadedness. All of her symptoms seemed to worsen with any change in posture.
Her PMH is significant for a seizure disorder for which she takes Topamax. Dr. Myers, could Topamax cause any of these symptoms?
On examination her Va was 20/20 and 20/25. She had no RAPD, her pressures were normal as was the rest of her anterior segment exam.
Her dilated exam was significant for bilateral swollen discs.
Here are her visual fields. Dr. Myers could you comment on these fields? Dr. Bilyk what further studies would you perform on this patient?
Here is her MRI. Dr. Flanders could you comment on these images?
Here is her MRV, Dr Flanders could you comment on these images?
Lumbar puncture was done. The opening pressure was elevated but CSF was otherwise normal. Dr. Savino what would your diagnosis be at this point? Dr. Savino, do you typically differentiate PTC from IIH?
The first line therapy for patients with IIH is weight loss. Greater that 6% weight loss is associated with resolution of papilledema. Dr. Savino, how long do you usually wait until starting medical therapy for patients? A recent study showed that 8/8 pts with IIH who had gastric bypass surgery had resolution of symptoms. Dr. Savino would you ever recommended this procedure for any of your patients? Diuretics, such as diamox are usually added next. Steroids are also used typically in the urgent setting prior to a procedure and in the PTC associated with SLE. Finally there are some interventional procedures. Dr. Savino, what dictates which procedure is recommended for these patients?
This patient was started on Diamox. She had a resolution of her symptoms and improvement of her papilledema. (Click) Nine months later she began having similar symptoms with headaches and visual disturbances. Dr. Savino, in patients with IIH and recurrent or persistent headache do you ever get further work up? (Click) The patient had no further work-up and was restarted on Diamox. (Click) She had minimal relief of symptoms on diamox and over the course of the next four months had two therapeutic lumbar punctures.
Four months later her mother returned home to find the patient hysterical. Pt was complaining of severe headache and said that she could not see. EMS was called and pt taken to ED at Cooper. She was sedated and intubated in the ED.
On hospital day #4 a MRI/MRV was done. Dr. Flanders could you comment on these images?
Here are the original images for comparison.
Here is the MRV. Dr. Flanders?
Again, the original images. Venous sinus thrombosis was diagnosed. Dr. Savino, to your knowledge have there been other cases of IIH progressing to VST? Dr. Bilyk, what further work up would you do on this patient?
The patient had a complicated hospital course. While her hypercoagulability work up was pending she developed renal failure, respiratory failure, liver failure, thrombocytopenia and anemia. (Click) She ultimately tested positive for Lupus Anticoagulant but did not fulfill criteria for SLE. Rest of hypercoagulability work-up was negative.
Pt was diagnosed with Venous Sinus Thrombosis secondary to Catastrophic Antiphospholipid Syndrome. Antiphospholipid syndrome is characterized by arterial and venous thrombosis, pregnancy loss, and thrombocytopenia in the presence of Antiphospholipid antibodies. One percent of patients with this syndrome have a fulminant course with high morbidity and mortality call Catastrophic Antiphospholipid Syndrome. This variant is characterized by involvement of 3 or more organs in less than one week.
Venous sinus thrombosis is the occlusion of one of the major dural venous sinuses. It causes increased intracranial pressure from venous hypertension leading to impaired CSF drainage. Many patients present with only symptoms of increased intracranial pressure. These patients present with PTC syndrome and are difficult to differentiate from IIH patients. Pts also can present with seizures and focal neurological deficits. These are usually the result of thrombosis extending to cortical veins and resulting in hemorrhagic venous infarcts.
Here I have compiled a list of conditions that have been reported to cause VST. The highlighted entities share an association with IIH. In one study of 38 IIH patients, 32% had coagulopathies, with antiphospholipid antibodies being the most common. Authors postulated that occult VST may be involved in the pathogenesis of IIH. Dr. Savino, given this information do you think that hypercoagulability work up should be done in all IIH patients? Lupus and oral contraceptive use is also more common in both. Interestingly 7% of pts with VST had recent lumbar puncture. Since IIH pts frequently have multiple LPs this may put them at increased risk for VST. IIH pts may have dehydration because of diuretics, and this may predispose them to VST as well.
Mainstay in treatment of VST is anticoagulation. Heparin followed by Coumadin is the typical regimen. Pts with severe symptoms in need of rapid intervention are treated with direct endovascular thrombolytics. There is also one case report of success with angioplasty and stenting. It is of course important to treat underlying cause if possible.
The patient was ultimately discharged after thirty days. Her main therapy consisted of treatment for CAPS with anticoagulation, high dose steroids and plasmaphoresis. The patient was discharged on Coumadin and prednisone. She has had no recurrences since discharge, however she remains bare LP in both eyes.
In summary this is a patient who presented on two separate occasions with PTC syndrome from two different causes. She demonstrates that it is difficult to distinguish the two entities clinically. This is a rare case but brings up the question, when do IIH patients require further work up?
Lumbar Puncture Opening pressure of 45 cm H2O Glucose WNL WBC WNL Protein WNL
Modified Dandy Criteria (Smith et al. J Clin Neuro-ophthalmol 1985)1. Signs and symptoms of increased ICP.2. No localizing neurological signs (except uni/ bilateral VI nerve palsy)3. No evidence of an intracranial mass lesion ( by CT )4. Normal CSF composition
Criteria for Idiopathic Intracranial Hypertension Friedman et al. Neurology 20021) If symptoms and signs are present, they should reflect only increased ICP.2) Documented increased ICP in the lateral decubitus position (> 250 cm/H2O).3) Normal CSF composition.4) No evidence of hydrocephalus, mass, structural or vascular lesion on CT with contrast for typical patients, and MRI/MRV for all others.5) No other cause of increased ICP found.
Spinal Tap Keep the patient’s head neutral and legs passively extended after needle in place. If the patient’s head is flexed and knees drawn up Increased cephalic venous pressure (Valsalva) compression of Jugular vein and hpoventilation (CO2 retention) Increased CSF pressure. An IIH patient needs only one spinal tap !
Is CT adequate ? Can detect hydrocephalus and most intra- cranial lesions. Misses venous sinus thrombosis, radiographic signs of meningeal infiltration, and isodense tumors. If only alternative (weight, availability), order contrast enhanced CT.
MRI and MRV Ideally for all patients. For atypical patients : (children with recent sinus/ear infection, fulminant course, non-responders to treatment).
Papilledema ? Blood vessels not obscured by NFL Anamolous trifurcationDrusen Drusen http://library.med.utah.edu/NOVEL/Hoyt
Papilledema ?Drusen Vessels not obscured by NFLhttp://library.med.utah.edu/NOVEL/Hoyt
Features of Anamolous discs (Peseuopapilledema)Psudopapilledema True PapilledemaDisc vessels obscured Disc margin vessels clearElevation of peripapillary Elevation confined to theNFL discSmall cupless disc Loss of cup lateAnamolous disc vessels Normal vessels(tri-, quadrifurcation)No hemorrhage or NFL hemorrhage, cottonexudates wool spots, exudate
IIH Treatment“ There is insufficient information to generate evidence-based management strategy for IIH. Of the Various treatments available, there is inadequate information regarding which are truly beneficial and which are potentially harmful”. Cochrane Review 2002
Idiopathic Intracranial Hypertension Treatment Trial Randomized clinical trial. In patients with mild visual loss ( MD -2 to -5 db), is Diamox better than placebo ? In patients with moderate visual loss (-5 to -14 db), is ONSF with Diamox better than Diamox alone ? All patients will be on low salt, fluid-restricted diet.
Venous Sinus Stenting Focal stenotic lesions in the lateral sinuses. Higgins et al. Journal of Neurology Neurosurgery and Psychiatry 2004;75:621-625
Venous Stenting Four patients underwent stenting had improved headache (Owler et al. J Neurosurg 2003). Direct retrograde cerebral venography (DRCV) is more sensitive than MRV in detecting venous thrombosis. Manometry showed a pressure gradient with raised proximal venous pressure.
Does venous stenting really work ? King et al. showed that in IIH patients, a pressure gradient across a venous stenotic lesion can be eliminated by removal of CSF through a cervical puncture . More longitudinal data are needed to evaluate the efficacy of venous stenting.
Which shunt is better ? 115 patients with IIH: 79 had LP and 36 had ventriculo- atrial (VAT) or ventriculoperitoneal (VP) shunts. Headache only and no visual deficit. LP shunts had higher revision rate (RR=2.5) and obstruction (RR=3.0). LP shunt 86% vs VAT/VP 44% revision rate after 24 months. Ventricular shunts can placed by stereo-tactic, MR- guided system. (McGirt et al. J Neurosug 101:627-632, 2004).
Case-Follow Up Improvement on Diamox Resolution of symptoms Papilledema improved Diamox tapered Nine months later Recurrence of symptoms No imaging done Diamox restarted Two therapeutic lumbar punctures
Four Months Later Altered Mental Status Combative Complaining of severe headache Screaming, “I can’t see” EMS called Sedated and intubated in ED Unable to attain VA
Treatment of VST Treatment of underlying cause Anticoagulation Systemic Heparin Coumadin (INR 2.0-3.0) Direct endovascular thrombolysis Urokinase tPA Rheolytic Endovascular recanalization
Follow Up Thirty day admission Treated for APS Anticoagulation (Heparin Coumadin) High dose steroids Plasmaphoresis Discharged Coumadin (INR 2.0-3.0) Prednisone No recurrence Bare LP OU
Case Two separate presentations of Pseudotumor cerebri syndrome in one patient. Idiopathic Intracranial Hypertension Venous Sinus Thrombosis
Summary IIH should be diagnosed using specific criteria. Pseudopapilledema should be ruled out. MRI/MRV is the neuro-imaging of choice. The treatment of IIH is medical and surgery is reserved for progressive visual loss and/or persistent headache. Consider repeat imaging in atypical cases ( fulminant course, poor response to treatment, focal neurologic signs).