Pseudotumor cerebri with transient oculomotor palsy
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《美国医学杂志》
Department of Pediatrics, NSCB Medical College, Jabalpur, India
Abstract
Pseudotumor cerebri is a clinical syndrome characterized by raised intracranial pressure with normal ventricular size, anatomy and position. Headache, vomiting and diplopia are the most common symptoms. Signs include those of raised intracranial pressure including papilledema and absence of focal neurological signs. A secondary cause is identifiable in 50% of children; the most common predisposing conditions are otitis media, viral infection and medications. Management is mainly directed towards identifying and treating the cause and measures to reduce the raised intracranial pressure. Though it is mostly a self limited condition, optic atrophy and blindness can occur. Oculomotor nerve palsy is very rarely associated with pseudotumor cerebri. We report a unique case of pseudotumor cerebri who had left Oculomotor palsy with sparing of the pupillary fibres, which resolved following treatment with oral acetazolamide.
Keywords: Pseudotumor cerebri; Raised intracranial pressure; Oculomotor nerve palsy
Pseudotumor cerebri is a perplexing syndrome of increased intracranial pressure without a space-occupying lesion.[1] More than 100 years after its original description by Quinckne, pseudotumor cerebri is still a poorly understood condition. We report a unique case of Pseudotumor cerebri which presented with left-sided oculomotor nerve palsy with sparing of pupillary fibers along with right nerve sixth nerve palsy and papilledema.
Case Report
A seven-year-old boy presented with diplopia of 10 days duration, frontal headache and projectile vomiting of seven days duration. There was no history of fever, convulsions or past history of headache. There was no proper history of drug intake.
On examination, the patient was conscious and alert; vitals were normal. Blood pressure was 116/82 taken in the left upper limb in the supine position with appropriate sized cuff. He had left ptosis with medial rectus palsy, right lateral rectus palsy. Both pupils were equal and reacting to light and accommodation. Fundus examination showed papilledema Stage 2 (Early papilledema as per Frisen's scale).[2] Motor and sensory systems were normal. There were no signs of meningeal irritation. Complete blood count and ESR were normal for his age. Lumbar puncture showed an opening pressure of 260 mm of water. Cerebrospinal fluid (CSF) findings were within normal limits. Both the CT and MRI Figure1 scans were normal. A diagnosis of pseudotumor cerebri was made.
The patient was put on oral acetazolamide following which the symptoms and signs of cranial nerve palsies (third and sixth) improved.
Discussion
The diagnostic criteria for pseudotumor cerebri have been revised and these include the following[3] :
1. Symptoms, if present, reflect only those of increased intracranial pressure or papilledema
2. Signs are attributable only to increased intracranial pressure or papilledema
3. Documented elevated intracranial pressure during lumbar puncture is measured in the lateral decubitus position
4. Cerebrospinal fluid composition is normal
5. There is no ventriculomegaly, mass, structural or vascular lesion on neuroimaging studies (MRI or contrast-enhanced CT for typical patients, and MRI and magnetic resonance venography for all others).
6. If no other cause (including medication) of intracranial hypertension is identified, the syndrome is termed "idiopathic intracranial hypertension".
The present patient satisfied all the above criteria. Unilateral or bilateral lateral rectus palsy is a nonlocalizing sign of increased intracranial pressure. Vertical diplopia due to fourth nerve palsy is an uncommon association. There has been only one reported case of oculomotor nerve palsy being associated with pseudotumor cerebri.[4] There are many conditions that are associated with pseudotumor cerebri in children, the most common being otitis media, viral infection, medication and closed head trauma, though weight gain and obesity are the only risk factors demonstrated in case control studies.[5],[6]
The reported abnormalities in neuroimaging in pseudotumor cerebri include dilated optic nerve sheaths, an empty sella and enlargement of subarachnoid space. The optic nerve can be clearly differentiated from the sheath on high-resolution orbital MRI with dilation of the perineuronal subarachnoid space. A study by Jacobson et al showed no difference in ventricular size between patients and controls.[7] MR studies in children have revealed no abnormality in periventricular water content.[8] Histologic evaluation of a brain specimen obtained during subtemporal decompression and evaluation of postmortem tissue of two patients who had PTC who died of other causes showed no histologic evidence of cerebral edema in patients with pseudotumor cerebri.[9]
Elevated cerebral venous sinus pressure has been proposed as the most probable cause for PTC and explains the lack of ventriculomegaly in PTC.[10]
The present case is unique due to the rare association of transient partial oculomotor palsy with pseudotumor cerebri.
References
1. Friedman DI. Pseudotumor cerebri. Neurol Clin N Am 2004;22 : 99
2. Frisen L. Swelling of the optic nerve head: a staging scheme. J Neurol Neurosurg Psychiatry 1982; 45: 13-18.
3. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002; 59:1492-5.
4. McCammon A, Kaufman HH, Sears ES. Transient Oculomotor paralysis in pseudotumor cerebri. Neurology 1981; 31(2): 182-184.
5. Dhiravibulya K, Ouvrier R, Johnston I et al. Benign intracranial hypertension in childhood; a review of 23 patients. J Pediatr Child Health 1991; 27 : 304-307.
6. Ireland B, Corbett JJ. The search for causes of idiopathic intracranial hypertension. Arch Neurol 1990; 47 : 315-320.
7. Jacobson DM, Karanjia PN, Olson KA et al. Computed tomography ventricular size has no predictive value in diagnosing pseudotumor cerebri. Neurology 1990; 40: 1454-1455.
8. Connolly MB, Farrell K, Hill A et al. Magnetic resonance imaging in pseudotumor cerebri. Dev Med Child Neurol 1992;34 : 1091-1094.
9. Wall M, Dollar JD, Sadun AA et al. Idiopathic intracranial hypertension. Lack of histologic evidence for cerebral edema. Arch Neurol 1995; 52: 141-145.
10. Corbett JJ, Digre KB. Idiopathic intracranial hypertension. An answer to, "the chicken or the egg" Neurology 2002; 58 : 5-6.(Chansoria Maya, Agrawal A)
Abstract
Pseudotumor cerebri is a clinical syndrome characterized by raised intracranial pressure with normal ventricular size, anatomy and position. Headache, vomiting and diplopia are the most common symptoms. Signs include those of raised intracranial pressure including papilledema and absence of focal neurological signs. A secondary cause is identifiable in 50% of children; the most common predisposing conditions are otitis media, viral infection and medications. Management is mainly directed towards identifying and treating the cause and measures to reduce the raised intracranial pressure. Though it is mostly a self limited condition, optic atrophy and blindness can occur. Oculomotor nerve palsy is very rarely associated with pseudotumor cerebri. We report a unique case of pseudotumor cerebri who had left Oculomotor palsy with sparing of the pupillary fibres, which resolved following treatment with oral acetazolamide.
Keywords: Pseudotumor cerebri; Raised intracranial pressure; Oculomotor nerve palsy
Pseudotumor cerebri is a perplexing syndrome of increased intracranial pressure without a space-occupying lesion.[1] More than 100 years after its original description by Quinckne, pseudotumor cerebri is still a poorly understood condition. We report a unique case of Pseudotumor cerebri which presented with left-sided oculomotor nerve palsy with sparing of pupillary fibers along with right nerve sixth nerve palsy and papilledema.
Case Report
A seven-year-old boy presented with diplopia of 10 days duration, frontal headache and projectile vomiting of seven days duration. There was no history of fever, convulsions or past history of headache. There was no proper history of drug intake.
On examination, the patient was conscious and alert; vitals were normal. Blood pressure was 116/82 taken in the left upper limb in the supine position with appropriate sized cuff. He had left ptosis with medial rectus palsy, right lateral rectus palsy. Both pupils were equal and reacting to light and accommodation. Fundus examination showed papilledema Stage 2 (Early papilledema as per Frisen's scale).[2] Motor and sensory systems were normal. There were no signs of meningeal irritation. Complete blood count and ESR were normal for his age. Lumbar puncture showed an opening pressure of 260 mm of water. Cerebrospinal fluid (CSF) findings were within normal limits. Both the CT and MRI Figure1 scans were normal. A diagnosis of pseudotumor cerebri was made.
The patient was put on oral acetazolamide following which the symptoms and signs of cranial nerve palsies (third and sixth) improved.
Discussion
The diagnostic criteria for pseudotumor cerebri have been revised and these include the following[3] :
1. Symptoms, if present, reflect only those of increased intracranial pressure or papilledema
2. Signs are attributable only to increased intracranial pressure or papilledema
3. Documented elevated intracranial pressure during lumbar puncture is measured in the lateral decubitus position
4. Cerebrospinal fluid composition is normal
5. There is no ventriculomegaly, mass, structural or vascular lesion on neuroimaging studies (MRI or contrast-enhanced CT for typical patients, and MRI and magnetic resonance venography for all others).
6. If no other cause (including medication) of intracranial hypertension is identified, the syndrome is termed "idiopathic intracranial hypertension".
The present patient satisfied all the above criteria. Unilateral or bilateral lateral rectus palsy is a nonlocalizing sign of increased intracranial pressure. Vertical diplopia due to fourth nerve palsy is an uncommon association. There has been only one reported case of oculomotor nerve palsy being associated with pseudotumor cerebri.[4] There are many conditions that are associated with pseudotumor cerebri in children, the most common being otitis media, viral infection, medication and closed head trauma, though weight gain and obesity are the only risk factors demonstrated in case control studies.[5],[6]
The reported abnormalities in neuroimaging in pseudotumor cerebri include dilated optic nerve sheaths, an empty sella and enlargement of subarachnoid space. The optic nerve can be clearly differentiated from the sheath on high-resolution orbital MRI with dilation of the perineuronal subarachnoid space. A study by Jacobson et al showed no difference in ventricular size between patients and controls.[7] MR studies in children have revealed no abnormality in periventricular water content.[8] Histologic evaluation of a brain specimen obtained during subtemporal decompression and evaluation of postmortem tissue of two patients who had PTC who died of other causes showed no histologic evidence of cerebral edema in patients with pseudotumor cerebri.[9]
Elevated cerebral venous sinus pressure has been proposed as the most probable cause for PTC and explains the lack of ventriculomegaly in PTC.[10]
The present case is unique due to the rare association of transient partial oculomotor palsy with pseudotumor cerebri.
References
1. Friedman DI. Pseudotumor cerebri. Neurol Clin N Am 2004;22 : 99
2. Frisen L. Swelling of the optic nerve head: a staging scheme. J Neurol Neurosurg Psychiatry 1982; 45: 13-18.
3. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002; 59:1492-5.
4. McCammon A, Kaufman HH, Sears ES. Transient Oculomotor paralysis in pseudotumor cerebri. Neurology 1981; 31(2): 182-184.
5. Dhiravibulya K, Ouvrier R, Johnston I et al. Benign intracranial hypertension in childhood; a review of 23 patients. J Pediatr Child Health 1991; 27 : 304-307.
6. Ireland B, Corbett JJ. The search for causes of idiopathic intracranial hypertension. Arch Neurol 1990; 47 : 315-320.
7. Jacobson DM, Karanjia PN, Olson KA et al. Computed tomography ventricular size has no predictive value in diagnosing pseudotumor cerebri. Neurology 1990; 40: 1454-1455.
8. Connolly MB, Farrell K, Hill A et al. Magnetic resonance imaging in pseudotumor cerebri. Dev Med Child Neurol 1992;34 : 1091-1094.
9. Wall M, Dollar JD, Sadun AA et al. Idiopathic intracranial hypertension. Lack of histologic evidence for cerebral edema. Arch Neurol 1995; 52: 141-145.
10. Corbett JJ, Digre KB. Idiopathic intracranial hypertension. An answer to, "the chicken or the egg" Neurology 2002; 58 : 5-6.(Chansoria Maya, Agrawal A)