case report

Oman Medical Journal [2024], Vol. 39, No. 1: e598 

Idiopathic Hypertension with Multiple Cranial Nerve Palsies: A Case Report and Literature Review

Shasthara Paneyala1*, Harsha Sundaramurthy1, Sahana Panambur1, Nemichandra S. C.1, Vimala Colaco2 and Sri Harsha Chalasani3

1Department of Neurology, JSS Medical College and Hospital, JSS Academy of Higher Education and Research,
Mysuru, India

2Department of Neurology, Father Muller Medical College, Mangalore, India

3Department of Pharmacy Practice, JSS College of Pharmacy, JSS Academy of Higher Education and Research,
Mysuru, India

article info


We report a rare case of idiopathic intracranial hypertension (IIH) with multiple cranial nerve palsies involving cranial nerves VI, VII, IX, and X in a 32-year-old female who had no prior comorbidities. Her condition improved rapidly on a ten-day regimen of acetazolamide and tablet topiramate. IIH should be considered in every patient presenting with persistent headache and multiple cranial nerve abnormalities. This paper also includes a literature review of similar cases.

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is identified by features of raised intracranial pressure (ICP), normal neuroimaging with elevated cerebrospinal fluid opening pressure (CSFOP) in the background of normal CSF components.1

Headache that worsens in the morning, pulsatile tinnitus, and vision abnormalities are among most common symptoms of IIH. Single or multiple cranial nerve palsies may also occur, as elevated ICP can lead to cranial nerve traction and compression. Sixth cranial nerve palsy, a marker of raised ICP, is seen in about 10–50% of children and 12% of adults with IIH. Rarely, cranial nerves (CNs) III, IV, VII, IX, and XII may also be involved.2 However, the occurrence of six CN palsies simultaneously (II, III, VI, VII, IX, and X) in the context of IIH—as in the current case—is extremely rare.

Case Report

A 32-year-old Indian woman with no known comorbidities and a body mass index of 20.8 kg/m2 presented to the neurology clinic with the complaint of a two-week-long headache that was dull, aching, holocranial, and continuous, accompanied by nausea, vomiting, photophobia and phonophobia, with no aggravating or relieving factors. A week after the headache began, she developed drooping of the left eyelid accompanied by horizontal double vision, especially for distant objects. She experienced difficulty in swallowing solids and liquids or sipping with a straw, though she could chew food without difficulty. The patient had no nasal regurgitation or hoarseness of voice. She had no history of migraine headache, oral contraceptive intake, recent illness, fever, seizures, altered sensorium, or trauma. She had regular menstrual cycles with stable hemodynamics at the time of the assessment.

During the neurological assessment, she was alert and had normal higher mental functions with no signs of meningeal irritation. Her muscle tone, motor system, reflexes, sensory system, coordination, and gait were normal. CN examination revealed partial palsy of the left third CN which manifested as partial ptosis of the left eyelid [Figure 1a]. The pupils were 3–4 mm in size and reacted sluggishly to light. Bilateral lateral gaze restriction of eye movements was noted with no nystagmus or skew deviation suggestive of bilateral sixth cranial nerve palsy [Figure 1 b and c]. Bilateral facial weakness was observed in the form of weak eye closure, reduced frontal creasing, nasolabial folds, and inability to hold air in the mouth or blow a whistle. Facial sensations were preserved on both sides with a normal jaw opening and a midline tongue. The uvula was deviated to the left [Figure 1d], with an absent gag reflex on the right side suggestive of right 9th and 10th CN palsies. The examination of the fundus revealed bilateral papilledema was also suggestive of 2nd CN palsy [Figure 2]. The shrugging of her shoulders was bilaterally symmetrical, and her hearing was normal.

Figure 1: (a) Partial ptosis of left eyelid in primary gaze. (b and c) Lateral gaze restriction. (d) Uvula deviated to left side suggestive of right 9th and 10th cranial nerve palsies.

Figure 2:Fundoscopy showing bilateral papilledema, suggestive of 2nd cranial nerve palsy.

Magnetic resonance imaging of the brain with magnetic resonance venography (MRV) revealed tortuous bilateral optic nerves with a mild bulge of the optic nerve head at the optic disc; these findings were consistent with raised ICP [Figure 3]. Lumbar puncture (LP) with the patient in the lateral decubitus position revealed a CSFOP of 320 mm H2O (standard reference interval: up to 250 mm H2O). The cytological and chemical results of LP were within the normal range: white blood cells 2 (lymphocytes 100%), protein 20.1 mg/dL, and glucose 79 mg/dL. No clinical or radiological features were suggestive of Addison’s or Cushing’s syndrome, and serology was negative. As the patient’s condition fulfilled modified Dandy criteria for definite IIH, she was started on a cerebral decongestant acetazolamide 250 mg twice daily along with topiramate 50 mg once at night for headache.

Figure 3: MRI of the brain showing tortuous bilateral optic nerves with a mild bulge of the optic nerve head at optic disc with hypoplastic left transverse sinus.

During her hospital stay, the patient showed dramatic improvement in headache and in palsies of the 7th, 9th, and 10th CNs. At a six-month routine follow-up, her papilledema and all the CN palsies were found to be completely resolved.


In the current case, after eliminating possible secondary causes, only the features suggestive of raised ICP remained. Thus, the most likely diagnosis for our patient’s presentation was IIH according to modified Dandy criteria.3 This diagnosis was further supported by the markedly elevated CSFOP and improvement upon receiving acetazolamide therapy.

IIH affects both children and adults, with a yearly incidence of approximately one to two cases per 100 000 population.4 While age is no bar to presentation of IIH, this condition is observed more among obese women of childbearing age with an estimated prevalence of four to 21 cases per 100 000 population.5

IIH can be described by various imaging findings such as perioptic nerve sheath distention, vertical buckling of the optic nerve, globe flattening, optic nerve head protrusion, and an empty sella tursica.2 Other differential diagnoses include neurosarcoidosis, Lyme disease, and Bell’s palsy. While the definite pathophysiology of IIH is not clear, it possibly involves CSF production and absorption and cerebral venous pressure elevation.2

Headache, pulsatile tinnitus, brief visual obscurations, and vision loss are the most typical symptoms of IIH.1 Also, in a case-control study, the most common symptoms reported by IIH patients were headache (94%), transient visual obscuration (68%), pulsatile tinnitus (58%), photopsia (54%), diplopia (38%), vision loss (30%), and retrobulbar pain on eye movements (22%).6 In addition to these classical signs of IIH, patients may present with multiple CN palsies as a false localizing sign, the most common being unilateral or bilateral 6th CN palsy.7 Rare cases of association of IIH with involvement of the right second, third, sixth, and left seventh CNs have been reported.8 However, the association of IIH with bilateral multifocal CN palsies as observed in our case report has not been published to date to the best of our knowledge. Awareness that signs may be false localizing has implications for diagnostic investigations.

Ophthalmological evaluation for IIH should include fundoscopy, optical coherence tomography, and perimetry as it aids in early diagnosis and in monitoring response to therapy on follow-up. Neuroimaging with LPCSFOP further aids in confirming the diagnosis.5

Cases of IIH with third cranial nerve palsy should be investigated for possible pupillary involvement.9 Compression in the subarachnoid space in the setting of elevated ICP appears to be the most likely mechanism of involvement of the third nerve (and other CNs). The reversal of oculomotor palsy after serial lumbar punctures establishes its link to elevated ICP.

A prospective study of 724 patients with recurrent headaches used MRV and found that 67.8% of migraine patients had IIH without papilledema and 6.7% had bilateral transverse sinus stenosis after LP. These findings suggest that migraine patients with bilateral transverse sinus stenosis on cerebral MRV should have a LP to rule out IIH without papilledema.10

The main goal of therapy is to protect vision and reduce disabling symptoms. IIH is commonly managed with acetazolamide, a carbonic anhydrase inhibitor. Vomiting, diarrhea, renal stones, and aplastic anemia are the most common adverse effects associated with acetazolamide. Topiramate, another carbonic anhydrase inhibitor, is used to manage headache and to reduce ICP. Topiramate additionally suppresses appetite and helps in promoting weight loss in obese patients. Furosemide, a loop diuretic can also be considered. Methyl prednisone, 1 g per day along with acetazolamide may be used in the short-term management of IIH, especially in fulminant IIH which threatens vision loss. Due to the adverse effects such as weight gain and fluid retention, methyl prednisone is not recommended for long-term management of IIH.

One case in the literature where methyl prednisone was used in an obese patient was that of a 40-year-old Hispanic woman with hypertension.2 She presented with typical IIH symptoms including those indicative of a right-sided CN VII palsy. The fundus examination revealed bilateral grade II papilledema. Imaging studies ruled out structural and obstructive lesions and LP results were unremarkable except for increased OP. Prednisone and acetazolamide were administered. Two days later, both her headache and facial nerve palsy were reported a significant improvement .2 As our patient showed a significant response to acetazolamide alone, steroids could be avoided.

Fourteen case reports of IIH with facial nerve palsy involvement are shown in Table 1.

Table 1: Cases of idiopathic hypertension with multiple cranial palsies selected from the literature.

Author, year



Cranial nerve(s) involved



Hagberg and Sillanpää,11 1970



Unilateral CN VII and CN V

Headache and vomiting

Lumbar puncture and dexamethasone

Chutorian et al,12 1977



Unilateral CN VII

Bifrontal headache, drooping of the left side of the face, tearing from the left eye, nausea, and vomiting

Lumbar puncture



Transfrontal headache, facial asymmetry,



intermittent bitemporal headache, right side face drooping, and right brow could not elevate

Snyder and Frenkel,13 1979



Unilateral CN VII and ophthalmoplegia

Not available

Lumbar puncture and dexamethasone

Couch et al,14 1984

M & F

≤ 14

Unilateral CN VII and CN V

Headache, nausea and vomiting, visual disturbances, neck pain, unsteady gait, intermittent paresthesia, and swishing

noise in both ears which correlated with an audible generalized cranial bruit

Lumbar puncture

Kiwak and Levine,15 1984



Diplegic CN VII

Pressure like bifrontal headaches and

decreased peripheral vision

Lumbar puncture, prednisone, acetazolamide, lumboperitoneal shunt

Agarwal et al,16 1989



Unilateral CN VII, CN VI, and partial CN III

Not available

Lumbar puncture,


glycerol syrup

Zachariah et al,17 1990



Unilateral CN VII

Headaches, numbness of the right

side of the face, altered taste sensation, and tingling and weakness of the right arm and right leg

Lumbar puncture, prednisone, and acetazolamide

Bakshi et al,18 1992



Diplegia CN VII and CN V

Bifrontal headache, vomiting, and loss of vision

Lumboperitoneal shunt

Davie et al,19 1992




Bifrontal headaches, nausea, and


Lumbar puncture

Capobianco et al,20 1997



Unilateral CN VII

Bifrontal headaches and right-sided facial drooping




Unilateral CN VII

Severe throbbing headaches, pulsatile tinnitus, blurred vision in the left eye, painless horizontal diplopia, and

right-sided facial pain

Optic nerve sheath


Brackmann and Doherty,21 2007



Unilateral CN VII

Right-sided facial palsy

Acetazolamide, prednisolone, and serial lumbar punctures

Soroken et al,22 2015



Unilateral CN VI and contralateral CN VII

Diplopia and horizontal strabismus of the left eye

Lumbar puncture and acetazolamide

Samara et al,2 2019



Unilateral CN VII

Bifrontal headache

Prednisone and acetazolamide

CN: cranial nerve.

Bilateral papilledema is a feature of IIH, and optical coherence tomography imaging is an objective, noninvasive, and reproducible technique for diagnosing and monitoring it. The benefits of octreotide and somatostatin analogues in IIH are still being evaluated. Patients who do not tolerate or improve with medical management may need surgical management such as optic nerve sheath fenestration and CSF diversions (lumboperitoneal or ventriculoperitoneal shunting). Transverse venous sinus stenting is one of the cutting-edge surgical treatments for IIH with promising outcomes.8


For women of reproductive age who present with headache and multiple cranial nerve palsies, IIH should be included in the differential diagnosis. IIH is an exclusionary diagnosis and neuroimaging should be conducted to rule out any secondary causes for raised ICP. In this paper, we have sought to raise clinicians’ awareness of the potential for IIH in presentations of multiple cranial nerve palsies, as it is rarely described in the pathophysiology of IIH.


The authors declared no conflicts of interest. Written consent has been obtained from the patient.


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