Acta Neurol Belg DOI 10.1007/s13760-014-0393-9
LETTER TO THE EDITOR
Pseudotumor cerebri and pituitary apoplexy ¨ zc¸elik • Peren Perk A. Aysima O • Alper Dai Akif S¸irikc¸i
•
Received: 17 October 2014 / Accepted: 12 November 2014 Ó Belgian Neurological Society 2014
Keywords Children Pseudotumor cerebri Pituitary apoplexy Idiopathic intracranial hypertension Headache
Introduction Pseudotumor cerebri (PTC), also called idiopathic intracranial hypertension (IHH), is a disorder where intracranial pressure is increased neither by intracranial mass, infection
nor non-communicating hydrocephalus [1]. Spontaneous hemorrhage into the tumor, causing pituitary apoplexy (PA), is an acute clinical syndrome due to degenerative changes of vascular structures [2]. Here, we report a patient with neurological symptoms suggestive of pseudotumor cerebri (PTC) with the visual field cut due to pituitary adenoma. MRI features showed pituitary apoplexy (PA) during follow-up.
Case report
¨ zc¸elik (&) P. Perk A. Dai A. A. O Division of Pediatric Neurology, Gaziantep University Faculty of Medicine, 3th floor, 27310 S¸ ehitkamil/Gaziantep, Turkey e-mail: [email protected] P. Perk e-mail: [email protected] A. Dai e-mail: [email protected] A. S¸ irikc¸i Department of Neuroradiology, Gaziantep University Faculty of Medicine, Gaziantep, Turkey e-mail: [email protected]
A 15-year-old female was admitted to the pediatric neurology clinic because of headache that had lasted for 1 year. The headache was worse upon awakening, was localized in the frontal area and was resistant to analgesic treatment with sinusitis or migraine treatments. Over the previous month, her headache had worsened and was associated with blurred vision. She had no other clinical findings and no significant medical or family history. During fundoscopic examination, bilateral papilloedema was determined. Initial magnetic resonance images indicated no pathological findings (Fig. 1). Cerebrospinal fluid was measured as 290 mmH2O. Evaluation of the cavernous and dural sinuses by magnetic resonance venography (MRV) was normal. Scotomas, described as a partial loss of vision, were assessed in the inferior temporal visual field of the right eye and inferior and superior nasal visual field of the left eye with Humphrey Field Analyzer (HFA) 24-2 threshold perimetry for evaluating visual acuity. The patient received acetazolamide treatment and underwent serial lumbar punctures to reduce the increased intracranial pressure. The patient had no complaints after 6 months. Acetazolamide treatment was, therefore, gradually terminated. Headache and blurred vision worsened during the
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Acta Neurol Belg
pituitary hormones were normal. She continued to receive acetazolamide treatment during the follow-up and hypophysis MRI controls.
Discussion
Fig. 1 Sagittal T1-weighted brain MRI shows no hypophysis abnormality
second month following cessation of therapy. Visual field examination now showed a bitemporal hemianopia. Intracranial pressure, measured by lumbar puncture, was 300 mmH2O. A dynamic hypophysis MRI showed a 6 9 10 mm diffuse opacified lesion in the middle of the pituitary gland, termed a ‘filling defect’. The lesion, with hypointensity on T2-weighted images, and no opacified images on T1-weighted sagittal section, reflected a previous hemorrhage. There was a close relationship between the stalk of the pituitary gland and the optic chiasm on sagittal MRI images (Fig. 2). In addition, the suprasellar cistern was slightly obliterated. She reported regular menstrual cycles and no galactorrhea. An endocrinologic consultation showed that the levels of prolactin and other
Fig. 2 Left after contrast injection, sagittal T1-weighted MR image showing hypointense lesion compared to normal enhanced pituitary gland. Right coronal T2-weighted MR image showing dark masses, suggesting intrapituitary hemorrhage
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PA consists of sudden onset headache, vomiting, diplopia, altered mental status and failure of the hormonal axis. Patients with asymptomatic pituitary hemorrhage and infarct are known as subacute PA or subclinical PA. However, the effects of subclinical hemorrhage on pituitary glands remain unclear. In a review of 34 patients with pituitary adenomas, it was found that subclinical apoplexy was diagnosed in 6/34 subjects [3]. Annual cranial MRI monitoring and particular examinations were recommended by Lee JS et al. [3] for the diagnosis and follow-up of the disease. Bjerre et al. [4] evaluated 10 cases with PTC and established empty sella in 5 of 10 cases and pituitary adenoma in 2 of 10 cases. Based on this information, hypophysis MRI is essential for patients with pseudotumor cerebri, especially in whom bitemporal hemianopsia is dependent on compression to the optic chiasm detected during examination of the visual field. Our case was probably coincidental rather than an association, due to the absence of sinus venosus compression and a mass effect. To our knowledge, this is the first reported case with coexisting subclinical pituitary apoplexy and pseudotumor cerebri. We would like to emphasize that routine visual field evaluation is significantly important in patients with pseudotumor cerebri and would also recommend possible
Acta Neurol Belg
pituitary adenoma or pituitary apoplexy with reduced visual acuity to be considered during follow-up. Conflict of interest
None.
References 1. Corbett JJ, Savino PJ, Thompson HS et al (1982) Visual loss in pseudotumor cerebri. Follow-up of 57 patients from five to 41 year
sand a profile of 14 patients with permanent severe visual loss. Arch Neurol 39:461–474 2. Wakai S, Fukushima T, Teramoto A, Sano K (1981) Pituitary apoplexy: its incidence and clinical significance. J Neurosurg 55:187–193 3. Lee JS, Park YS, Kwon JT, Nam TK, Lee TJ, Kim JK (2011) Radiological apoplexy and its correlation with acute clinical presentation, angiogenesis and tumor microvascular density in pituitary adenomas. J Korean Neurosurg Soc 50:281–287 4. Bjerre P, Lindholm J, Gyldensted C (1982) Pseudotumour cerebri. A theory on etiology and pathogenesis. Acta Neurol Scand 66:472–481
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LETTER TO THE EDITOR
Pseudotumor cerebri and pituitary apoplexy ¨ zc¸elik • Peren Perk A. Aysima O • Alper Dai Akif S¸irikc¸i
•
Received: 17 October 2014 / Accepted: 12 November 2014 Ó Belgian Neurological Society 2014
Keywords Children Pseudotumor cerebri Pituitary apoplexy Idiopathic intracranial hypertension Headache
Introduction Pseudotumor cerebri (PTC), also called idiopathic intracranial hypertension (IHH), is a disorder where intracranial pressure is increased neither by intracranial mass, infection
nor non-communicating hydrocephalus [1]. Spontaneous hemorrhage into the tumor, causing pituitary apoplexy (PA), is an acute clinical syndrome due to degenerative changes of vascular structures [2]. Here, we report a patient with neurological symptoms suggestive of pseudotumor cerebri (PTC) with the visual field cut due to pituitary adenoma. MRI features showed pituitary apoplexy (PA) during follow-up.
Case report
¨ zc¸elik (&) P. Perk A. Dai A. A. O Division of Pediatric Neurology, Gaziantep University Faculty of Medicine, 3th floor, 27310 S¸ ehitkamil/Gaziantep, Turkey e-mail: [email protected] P. Perk e-mail: [email protected] A. Dai e-mail: [email protected] A. S¸ irikc¸i Department of Neuroradiology, Gaziantep University Faculty of Medicine, Gaziantep, Turkey e-mail: [email protected]
A 15-year-old female was admitted to the pediatric neurology clinic because of headache that had lasted for 1 year. The headache was worse upon awakening, was localized in the frontal area and was resistant to analgesic treatment with sinusitis or migraine treatments. Over the previous month, her headache had worsened and was associated with blurred vision. She had no other clinical findings and no significant medical or family history. During fundoscopic examination, bilateral papilloedema was determined. Initial magnetic resonance images indicated no pathological findings (Fig. 1). Cerebrospinal fluid was measured as 290 mmH2O. Evaluation of the cavernous and dural sinuses by magnetic resonance venography (MRV) was normal. Scotomas, described as a partial loss of vision, were assessed in the inferior temporal visual field of the right eye and inferior and superior nasal visual field of the left eye with Humphrey Field Analyzer (HFA) 24-2 threshold perimetry for evaluating visual acuity. The patient received acetazolamide treatment and underwent serial lumbar punctures to reduce the increased intracranial pressure. The patient had no complaints after 6 months. Acetazolamide treatment was, therefore, gradually terminated. Headache and blurred vision worsened during the
123
Acta Neurol Belg
pituitary hormones were normal. She continued to receive acetazolamide treatment during the follow-up and hypophysis MRI controls.
Discussion
Fig. 1 Sagittal T1-weighted brain MRI shows no hypophysis abnormality
second month following cessation of therapy. Visual field examination now showed a bitemporal hemianopia. Intracranial pressure, measured by lumbar puncture, was 300 mmH2O. A dynamic hypophysis MRI showed a 6 9 10 mm diffuse opacified lesion in the middle of the pituitary gland, termed a ‘filling defect’. The lesion, with hypointensity on T2-weighted images, and no opacified images on T1-weighted sagittal section, reflected a previous hemorrhage. There was a close relationship between the stalk of the pituitary gland and the optic chiasm on sagittal MRI images (Fig. 2). In addition, the suprasellar cistern was slightly obliterated. She reported regular menstrual cycles and no galactorrhea. An endocrinologic consultation showed that the levels of prolactin and other
Fig. 2 Left after contrast injection, sagittal T1-weighted MR image showing hypointense lesion compared to normal enhanced pituitary gland. Right coronal T2-weighted MR image showing dark masses, suggesting intrapituitary hemorrhage
123
PA consists of sudden onset headache, vomiting, diplopia, altered mental status and failure of the hormonal axis. Patients with asymptomatic pituitary hemorrhage and infarct are known as subacute PA or subclinical PA. However, the effects of subclinical hemorrhage on pituitary glands remain unclear. In a review of 34 patients with pituitary adenomas, it was found that subclinical apoplexy was diagnosed in 6/34 subjects [3]. Annual cranial MRI monitoring and particular examinations were recommended by Lee JS et al. [3] for the diagnosis and follow-up of the disease. Bjerre et al. [4] evaluated 10 cases with PTC and established empty sella in 5 of 10 cases and pituitary adenoma in 2 of 10 cases. Based on this information, hypophysis MRI is essential for patients with pseudotumor cerebri, especially in whom bitemporal hemianopsia is dependent on compression to the optic chiasm detected during examination of the visual field. Our case was probably coincidental rather than an association, due to the absence of sinus venosus compression and a mass effect. To our knowledge, this is the first reported case with coexisting subclinical pituitary apoplexy and pseudotumor cerebri. We would like to emphasize that routine visual field evaluation is significantly important in patients with pseudotumor cerebri and would also recommend possible
Acta Neurol Belg
pituitary adenoma or pituitary apoplexy with reduced visual acuity to be considered during follow-up. Conflict of interest
None.
References 1. Corbett JJ, Savino PJ, Thompson HS et al (1982) Visual loss in pseudotumor cerebri. Follow-up of 57 patients from five to 41 year
sand a profile of 14 patients with permanent severe visual loss. Arch Neurol 39:461–474 2. Wakai S, Fukushima T, Teramoto A, Sano K (1981) Pituitary apoplexy: its incidence and clinical significance. J Neurosurg 55:187–193 3. Lee JS, Park YS, Kwon JT, Nam TK, Lee TJ, Kim JK (2011) Radiological apoplexy and its correlation with acute clinical presentation, angiogenesis and tumor microvascular density in pituitary adenomas. J Korean Neurosurg Soc 50:281–287 4. Bjerre P, Lindholm J, Gyldensted C (1982) Pseudotumour cerebri. A theory on etiology and pathogenesis. Acta Neurol Scand 66:472–481
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