Honavar PU*, Samant PY **, Bhosle SB***.
(* Assistant Professor, ** Additional Professor, *** First Year Resident, Department of Obstetrics and Gynecology, Seth G S Medical College and KEM Hospital, Mumbai, India.)
Idiopathic intracranial hypertension, also referred to as pseudotumor cerebri is a rare entity encountered more in obese women of reproductive age. In the western literature prevalence of around 10/100000 is reported which increases nearly 8 fold in obese women. We report a case of severe pregnancy induced hypertension that had idiopathic intracranial hypertension in the past. We would like to highlight possible autoimmune phenomenon as a causative factor for both the conditions.
Idiopathic intracranial hypertension (IIH) affects women more than men and especially young obese women of reproductive age. It is also called as pseudotumor cerebri (PTC) as it presents with severe headaches and other symptoms of raised intracranial tension in absence of organic mass lesion. Its association with systemic lupus erythematosus (SLE) has been reported. We report a case of severe preeclampsia that tested positive for antinuclear antibodies, and had idiopathic intracranial hypertension in the past. There could be a possible autoimmune causative factor linking both the conditions. It indicates the need for monitoring of her future pregnancies for severe hypertension and symptoms of SLE.
A 25 year old primigravida with 29 weeks gestation was referred from a peripheral health center to our center in view of uncontrolled antenatal blood pressure in spite of taking methyl dopa and nifedipine; for further management. She had no premonitory symptoms. She was diagnosed to have idiopathic intracranial hypertension one and a half year ago, following complaints of headache and diplopia. Her medical reports indicated that she had bilateral papilledema, but her magnetic resonance venogram of the brain was normal. She was treated with acetazolamide for 5 months after which her symptoms resolved.
Presently, her general condition was fair. Her blood pressure was 180/110 mm Hg. She had bilateral pedal edema and albuminuria. She had no other neurological symptoms.
The uterine size corresponded to 16 to 18 weeks. Her liver, renal and coagulation profiles were normal. Lupus anticoagulant and anti cardiolipin antibody tests were negative but antinuclear antibody (ANA) test was positive. Obstetric ultrasound revealed severe growth restriction with doppler changes suggestive of severe fetoplacental insufficiency. A decision to induce labor in maternal interest in view of severe preeclampsia was taken. Neuromedicine opinion was taken for induction of labor, as she had a past history of IIH. Magnesium sulphate was started and labor was induced with prostaglandins. She delivered a stillborn baby of 662 grams. After delivery, the antihypertensive drugs were tapered and stopped on the third day as her blood pressure reverted to normal.
Idiopathic intracranial hypertension is seen more commonly in women than in men, more so in obese women of reproductive age. In the United Kingdom, a survey showed annual incidence of 1.56/100,000/year and prevalence of 10.9/100000. The incidence of IIH is approximately 19.3/100,000 in obese women.[2,3] An Indian study reported the female to male ratio of 4:1.
Some authors prefer the term ‘Pseudo Tumor Cerebri Syndrome’ (PTCS) which includes intracranial hypertension that is idiopathic and also secondary to precipitating factors like drugs and medical conditions.[3,5] The symptoms of IIH/ PTCS include retrobulbar throbbing headache known to worsen with movements of eyes, photophobia, double vision, or even loss of vision. It may be associated with nausea, vomiting. Preexistent migraine may also worsen. Some patients complain of giddiness and tinnitus. Rarely, CSF rhinorrhea or otorrhea without any cause may occur in IIH/ PTCS.[6,7]
Till 2002, modified Dandy criteria were applied for diagnosis of IIH. As per these, the patient manifests symptoms of high intracranial tension, but there is no neurodeficit, or high opening cerebrospinal fluid (CSF) pressure on lumbar tap. CSF biochemistry is normal. There is empty sella, but no evidence of thrombosis on neuroimaging. Friedman described updated criteria taking into account advances in neuroimaging that point to additional causes of IIH. This criteria was further revised in 2013.They included venous thrombotic conditions as well as hypercoagulable conditions among others. These conditions may be of autoimmune origin. Rate of pregnancy wastage in IIH patients is similar to that in general pregnant population. Visual outcome is considered to be the same as that in the non pregnant IIH patients. Termination of pregnancy is not indicated for improving symptoms of IIH.
Acetazolamide, the treatment of choice in general population with IIH may be used in pregnancy with caution and after counseling if clinical situation warrants.[11,12] Epidural analgesia is safe in IIH patients in labor except in patients with shunts in place. Spinal anesthesia is considered safe in these patients and, is more preferred in cases of IIH/ PTCS than general anesthesia for cesarean section. Beta-blockers or hydralazine can be used to attenuate rise in ICP in these cases.
There are numerous reports of IIH presenting itself in men, women and children with autoimmune disorders especially lupus nephritis[13,14] and in other autoimmune conditions. In pregnancy related conditions, preeclampsia is known to unmask autoimmune pathology preexistent in a woman. Our patient had severe hypertension and albuminuria detected at 29 weeks and required induction of labor in maternal interest. On investigations she had a positive ANA which points to preexistent autoimmune condition. This indicates the need for further investigations for autoimmune disorder as well as monitoring for future successful obstetric outcome.
We believe that the term ‘pseudotumor cerebri syndrome’ is more inclusive than ‘idiopathic intracranial hypertension’ for the disorder. Severe preeclampsia and intracranial hypertension manifested by the patient in past may reflect autoimmune pathology.
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Honavar PU, Samant PY, Bhosle SB. Severe Preeclampsia and Idiopathic Intracranial Hypertension: Possible Autoimmune Connection. JPGO 2016. Volume 3 No. 7. Available from: http://www.jpgo.org/2016/07/severe-preeclampsia-and-idiopathic.html