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Bilateral Oculomotor Palsy Secondary to Pseudotumor Cerebri
Bilateral Oculomotor Palsy Secondary to Pseudotumor Cerebri Hu¨seyin Tan, MD Idiopathic intracranial hypertension is the syndrome of elevated intracranial pressure without clinical, laboratory, or imaging evidence of intracranial pathology. The classic symptoms include headache, nausea, and vomiting. It may also be associated with blurry vision, diplopia, stiff neck, increasing head size, photophobia, anorexia, retro-orbital pain, lightheadedness, myalgia, and head tilt. Sixth nerve palsy is documented in 1040% of patients with pseudotumor cerebri, in most series, but third nerve palsy is very rarely associated with pseudotumor cerebri. Described here is the novel case of a pseudotumor cerebri patient who had bilateral partial oculomotor palsy with sparing of the pupillary fibers. Ó 2010 by Elsevier Inc. All rights reserved. Tan H. Bilateral oculomotor palsy secondary to pseudotumor cerebri. Pediatr Neurol 2010;42:141-142.
of a patient with pseudotumor cerebri who had bilateral partial oculomotor palsy with sparing of the pupillary fibers. Case Report A 14-year-old girl was admitted for frontal headache and projectile vomiting of 12 days duration and diplopia, blurred vision, and pulsatile tinnitus of 6 days duration. She had been diagnosed with acute maxillary sinusitis at a local hospital and started on amoxicillin and acetaminophen 3 days previously, but no benefit was observed. Her medical history was unremarkable. There was no history of drug intake. On examination, the patient was at normal height and weight for her age. She was conscious and alert; vital signs were normal. She had bilateral medial rectus palsy and inferior rectus palsy with mild ptosis. Both pupils were equal and reactive to light and accommodation. Fundus examination revealed bilateral papilledema. Motor and sensory systems were normal. There were no signs of meningeal irritation. Findings from all her laboratory studies were normal for her age, including urinalysis, complete blood count, routine biochemical tests, C-reactive protein, erythrocyte sedimentation rate, thyroid function tests, parathyroid hormone, and serum copper and ceruloplasmin levels. Lumbar puncture revealed an opening pressure of 350 mm H2O and a closing pressure of 250 mm H2O. Cerebrospinal fluid findings were normal glucose (60 mg/dL) and protein (21 mg/dL), with no cells. Cranial magnetic resonance imaging with contrast enhancement revealed no abnormality, apart from maxillary sinusitis. Cerebral magnetic resonance venography revealed no obstruction in the venous system of the brain. A diagnosis of pseudotumor cerebri due to maxillary sinusitis was made on the basis of the history, clinical findings, and laboratory data. Her sinusitis treatment was continued. She was placed on oral acetazolamide (30 mg/ kg per day). All symptoms and signs except papilledema disappeared within 3 days after lumbar puncture and administration of oral acetazolamide. Tinnitus was decreased, but not completely improved. Tinnitus had improved completely at the end of 2 months, whereas papilledema disappeared after 3 months. Her visual acuity and visual field were normal at both initial and follow-up examinations.
Discussion Introduction Pseudotumor cerebri, now termed idiopathic intracranial hypertension, is a clinical syndrome defined by elevated intracranial pressure without clinical, laboratory, or imaging evidence of any responsible infection, vascular abnormality, space-occupying lesion, or hydrocephalus [1]. The most common symptoms in pseudotumor cerebri are headache, vomiting, and diplopia; its signs include those of raised intracranial pressure, including papilledema and absence of focal neurologic signs. Sixth nerve palsy is seen in 10-40% of patients with pseudotumor cerebri, in most series, but third nerve palsy is very rarely associated with pseudotumor cerebri [2]. Described here is the novel case
From the Department of Pediatric Neurology, Faculty of Medicine, Atatu¨rk University, Erzurum, Turkey.
Ó 2010 by Elsevier Inc. All rights reserved. doi:10.1016/j.pediatrneurol.2009.09.004 0887-8994/09/$—see front matter
Pseudotumor cerebri was first described in 1937 by Dandy [3]. It is a clinical syndrome characterized by elevated intracranial pressure with normal ventricular size, anatomy, and position. It is often seen in obese, young women, but it also affects children, including infants [4]. Pseudotumor cerebri is diagnosed by modified Dandy’s criteria: (1) the presence of signs or symptoms of increased intracranial pressure; (2) normal neurologic signs except for papilledema and abducens nerve palsy; (3) normal neuroimaging studies, or evidence of small, slit-like ventricles in some patients; (4) cerebrospinal fluid with normal composition and a raised opening pressure (above 200 mm H2O in patients older than 5 years of age, above 135 mm Communications should be addressed to: Dr. Tan; Atatu¨rk University, Faculty of Medicine; Yakutiye Research Hospital; Department of Pediatric Neurology; 25240 Erzurum, Turkey. E-mail: [email protected] Received February 23, 2009; accepted September 2, 2009.
Tan: Pseudotumor Cerebri With Oculomotor Palsy
141
H2O in patients aged 2-5 years, and above 75 mm H2O in patients <2 years of age); and (5) the absence of primary structural or systemic causes of increased intracranial venous sinus pressure [5]. The present case satisfied all the criteria. In addition, the patient had bilateral partial oculomotor palsy. In children, a secondary cause is identifiable in half of the cases; the most common predisposing conditions are infections (sinusitis, otitis media, mastoiditis, and roseola infantum), medications (nalidixic acid, prolonged corticosteroid intake or too rapid withdrawal, growth hormone treatment, excess vitamins A and D, tetracycline, and nitrofurantoin), hematologic disorders (polycythemia, hemolytic anemia, and iron-deficiency anemia), metabolic disorders (hypoparathyroidism, pseudohypoparathyroidism, hypophosphatasia, obesity, galactosemia, and Addison’s disease), and head injury [2]. The patient in the present case had sinusitis, resulting in pseudotumor cerebri. The pathogenesis of pseudotumor cerebri is still not completely understood. Several mechanisms have been suggested to explain its pathogenesis, including brain edema, decreased cerebrospinal fluid reabsorption or its increased production, abnormalities in vasomotor control and cerebral blood flow, and venous obstruction [2]. Karahalios et al. [6] hypothesized that increased intracranial venous pressure might play a role as a universal mechanism in pseudotumor cerebri of different etiologies. This increased venous pressure decreases cerebrospinal fluid reabsorption, leading to a rise in cerebrospinal fluid volume and thereby elevated intracranial pressure. All the posited mechanisms may have played a role in the present case, except brain edema, which was not observed in this patient. The most common symptoms of pseudotumor cerebri are headache, vomiting, and diplopia. Signs include those of raised intracranial pressure, including papilledema and absence of focal neurologic signs [1,2]. Oculomotor nerve palsy is rarely seen in children [2]. In terms of cause, cranial nerve palsy is most frequently congenital, followed by traumatic, neoplastic, vascular, and migrainous or parainfectious etiologies [7]. Oculomotor palsy in the present case may have been due to either the sinusitis or the pseudotumor cerebri, and a search of the related literature did reveal reports of oculomotor palsy due to maxillary sinusitis.
142 PEDIATRIC NEUROLOGY Vol. 42 No. 2
Sphenoid sinusitis may lead to partial oculomotor palsy because of the anatomic proximity of the sinus and the cranial nerve [8]. Thus, the present case suggests a possible association between oculomotor nerve palsy and pseudotumor cerebri. In patients with oculomotor nerve palsy, pseudotumor cerebri should be taken into consideration after other probable causes of oculomotor nerve palsy have been excluded. As for prognosis, spontaneous improvement in pseudotumor cerebri is frequent. The symptoms can usually be controlled by medical therapy until recovery occurs, generally within 2-3 months. In approximately one fourth of patients, however, the clinical course may be protracted. The most frequent complications in this disorder are disabling severe headache, cognitive dysfunction, and visual disturbances. The most serious complication is permanent loss of visual function. Treatment efficacy in pseudotumor cerebri should therefore be assessed with regular automated perimeter testing to ensure stable visual function. The evaluation should be performed every other week until the condition stabilizes, when it should be performed every third month [9].
References [1] Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002;59:1492-5. [2] Rangwala LM, Liu GT. Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 2007;52:597-617. [3] Dandy WE. Intracranial pressure without brain tumor: diagnosis and treatment. Ann Surg 1937;106:493-513. [4] Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri: population studies in Iowa and Louisiana. Arch Neurol 1988;45: 875-7. [5] Soler D, Cox T, Bullock P, Calver DM, Robinson RO. Diagnosis and management of benign intracranial hypertension. Arch Dis Child 1998;78:89-94. [6] Karahalios DG, Rekate HL, Khayata MH, Apostolides PJ. Elevated intracranial venous pressure as a universal mechanism in pseudotumor cerebri of varying etiologies. Neurology 1996;46:198-202. [7] Ng YS, Lyons CJ. Oculomotor nerve palsy in childhood. Can J Ophthalmol 2005;40:645-53. [8] Lee LA, Huang CC, Lee TJ. Prolonged visual disturbance secondary to isolated sphenoid sinus disease. Laryngoscope 2004;114:986-90. [9] Skau M, Brennum J, Gjerris F, Jensen R. What is new about idiopathic intracranial hypertension? An updated review of mechanism and treatment. Cephalalgia 2006;26:384-99.
of a patient with pseudotumor cerebri who had bilateral partial oculomotor palsy with sparing of the pupillary fibers. Case Report A 14-year-old girl was admitted for frontal headache and projectile vomiting of 12 days duration and diplopia, blurred vision, and pulsatile tinnitus of 6 days duration. She had been diagnosed with acute maxillary sinusitis at a local hospital and started on amoxicillin and acetaminophen 3 days previously, but no benefit was observed. Her medical history was unremarkable. There was no history of drug intake. On examination, the patient was at normal height and weight for her age. She was conscious and alert; vital signs were normal. She had bilateral medial rectus palsy and inferior rectus palsy with mild ptosis. Both pupils were equal and reactive to light and accommodation. Fundus examination revealed bilateral papilledema. Motor and sensory systems were normal. There were no signs of meningeal irritation. Findings from all her laboratory studies were normal for her age, including urinalysis, complete blood count, routine biochemical tests, C-reactive protein, erythrocyte sedimentation rate, thyroid function tests, parathyroid hormone, and serum copper and ceruloplasmin levels. Lumbar puncture revealed an opening pressure of 350 mm H2O and a closing pressure of 250 mm H2O. Cerebrospinal fluid findings were normal glucose (60 mg/dL) and protein (21 mg/dL), with no cells. Cranial magnetic resonance imaging with contrast enhancement revealed no abnormality, apart from maxillary sinusitis. Cerebral magnetic resonance venography revealed no obstruction in the venous system of the brain. A diagnosis of pseudotumor cerebri due to maxillary sinusitis was made on the basis of the history, clinical findings, and laboratory data. Her sinusitis treatment was continued. She was placed on oral acetazolamide (30 mg/ kg per day). All symptoms and signs except papilledema disappeared within 3 days after lumbar puncture and administration of oral acetazolamide. Tinnitus was decreased, but not completely improved. Tinnitus had improved completely at the end of 2 months, whereas papilledema disappeared after 3 months. Her visual acuity and visual field were normal at both initial and follow-up examinations.
Discussion Introduction Pseudotumor cerebri, now termed idiopathic intracranial hypertension, is a clinical syndrome defined by elevated intracranial pressure without clinical, laboratory, or imaging evidence of any responsible infection, vascular abnormality, space-occupying lesion, or hydrocephalus [1]. The most common symptoms in pseudotumor cerebri are headache, vomiting, and diplopia; its signs include those of raised intracranial pressure, including papilledema and absence of focal neurologic signs. Sixth nerve palsy is seen in 10-40% of patients with pseudotumor cerebri, in most series, but third nerve palsy is very rarely associated with pseudotumor cerebri [2]. Described here is the novel case
From the Department of Pediatric Neurology, Faculty of Medicine, Atatu¨rk University, Erzurum, Turkey.
Ó 2010 by Elsevier Inc. All rights reserved. doi:10.1016/j.pediatrneurol.2009.09.004 0887-8994/09/$—see front matter
Pseudotumor cerebri was first described in 1937 by Dandy [3]. It is a clinical syndrome characterized by elevated intracranial pressure with normal ventricular size, anatomy, and position. It is often seen in obese, young women, but it also affects children, including infants [4]. Pseudotumor cerebri is diagnosed by modified Dandy’s criteria: (1) the presence of signs or symptoms of increased intracranial pressure; (2) normal neurologic signs except for papilledema and abducens nerve palsy; (3) normal neuroimaging studies, or evidence of small, slit-like ventricles in some patients; (4) cerebrospinal fluid with normal composition and a raised opening pressure (above 200 mm H2O in patients older than 5 years of age, above 135 mm Communications should be addressed to: Dr. Tan; Atatu¨rk University, Faculty of Medicine; Yakutiye Research Hospital; Department of Pediatric Neurology; 25240 Erzurum, Turkey. E-mail: [email protected] Received February 23, 2009; accepted September 2, 2009.
Tan: Pseudotumor Cerebri With Oculomotor Palsy
141
H2O in patients aged 2-5 years, and above 75 mm H2O in patients <2 years of age); and (5) the absence of primary structural or systemic causes of increased intracranial venous sinus pressure [5]. The present case satisfied all the criteria. In addition, the patient had bilateral partial oculomotor palsy. In children, a secondary cause is identifiable in half of the cases; the most common predisposing conditions are infections (sinusitis, otitis media, mastoiditis, and roseola infantum), medications (nalidixic acid, prolonged corticosteroid intake or too rapid withdrawal, growth hormone treatment, excess vitamins A and D, tetracycline, and nitrofurantoin), hematologic disorders (polycythemia, hemolytic anemia, and iron-deficiency anemia), metabolic disorders (hypoparathyroidism, pseudohypoparathyroidism, hypophosphatasia, obesity, galactosemia, and Addison’s disease), and head injury [2]. The patient in the present case had sinusitis, resulting in pseudotumor cerebri. The pathogenesis of pseudotumor cerebri is still not completely understood. Several mechanisms have been suggested to explain its pathogenesis, including brain edema, decreased cerebrospinal fluid reabsorption or its increased production, abnormalities in vasomotor control and cerebral blood flow, and venous obstruction [2]. Karahalios et al. [6] hypothesized that increased intracranial venous pressure might play a role as a universal mechanism in pseudotumor cerebri of different etiologies. This increased venous pressure decreases cerebrospinal fluid reabsorption, leading to a rise in cerebrospinal fluid volume and thereby elevated intracranial pressure. All the posited mechanisms may have played a role in the present case, except brain edema, which was not observed in this patient. The most common symptoms of pseudotumor cerebri are headache, vomiting, and diplopia. Signs include those of raised intracranial pressure, including papilledema and absence of focal neurologic signs [1,2]. Oculomotor nerve palsy is rarely seen in children [2]. In terms of cause, cranial nerve palsy is most frequently congenital, followed by traumatic, neoplastic, vascular, and migrainous or parainfectious etiologies [7]. Oculomotor palsy in the present case may have been due to either the sinusitis or the pseudotumor cerebri, and a search of the related literature did reveal reports of oculomotor palsy due to maxillary sinusitis.
142 PEDIATRIC NEUROLOGY Vol. 42 No. 2
Sphenoid sinusitis may lead to partial oculomotor palsy because of the anatomic proximity of the sinus and the cranial nerve [8]. Thus, the present case suggests a possible association between oculomotor nerve palsy and pseudotumor cerebri. In patients with oculomotor nerve palsy, pseudotumor cerebri should be taken into consideration after other probable causes of oculomotor nerve palsy have been excluded. As for prognosis, spontaneous improvement in pseudotumor cerebri is frequent. The symptoms can usually be controlled by medical therapy until recovery occurs, generally within 2-3 months. In approximately one fourth of patients, however, the clinical course may be protracted. The most frequent complications in this disorder are disabling severe headache, cognitive dysfunction, and visual disturbances. The most serious complication is permanent loss of visual function. Treatment efficacy in pseudotumor cerebri should therefore be assessed with regular automated perimeter testing to ensure stable visual function. The evaluation should be performed every other week until the condition stabilizes, when it should be performed every third month [9].
References [1] Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002;59:1492-5. [2] Rangwala LM, Liu GT. Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 2007;52:597-617. [3] Dandy WE. Intracranial pressure without brain tumor: diagnosis and treatment. Ann Surg 1937;106:493-513. [4] Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri: population studies in Iowa and Louisiana. Arch Neurol 1988;45: 875-7. [5] Soler D, Cox T, Bullock P, Calver DM, Robinson RO. Diagnosis and management of benign intracranial hypertension. Arch Dis Child 1998;78:89-94. [6] Karahalios DG, Rekate HL, Khayata MH, Apostolides PJ. Elevated intracranial venous pressure as a universal mechanism in pseudotumor cerebri of varying etiologies. Neurology 1996;46:198-202. [7] Ng YS, Lyons CJ. Oculomotor nerve palsy in childhood. Can J Ophthalmol 2005;40:645-53. [8] Lee LA, Huang CC, Lee TJ. Prolonged visual disturbance secondary to isolated sphenoid sinus disease. Laryngoscope 2004;114:986-90. [9] Skau M, Brennum J, Gjerris F, Jensen R. What is new about idiopathic intracranial hypertension? An updated review of mechanism and treatment. Cephalalgia 2006;26:384-99.