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No areas of necrosis or pleomorphism were seen. The tumors were, thus, of the fibroblastic variety with no evidence of malignancy. Postoperatively the patient had no additional neurological deficit. He underwent radiotherapy, and 15 months after the surgery, the patient is doing well. 3. Discussion Meningiomas constitute 14% to 20% of all intracranial tumors. Usually, they are benign. The malignant group constitutes < 10% of all meningiomas. Meningiomas arise from the arachnoid villi, tela choroidea or leptomeninges and invariably abut the CSF.1,2 The reported incidence of meningiomas in the lateral ventricle varies between 0.7% and 2% of all intracranial meningiomas.2,3 Seeding of malignant intraventricular meningiomas through CSF pathways, although rare, has been reported.3–5 Histologically benign meningiomas are also reported to have metastasized extracranially through a hematogenous route.9–12 Benign intraventricular meningiomas with progressive anaplastic transformation and CSF metastases have been reported rarely.6,7 One patient with a transitional intraventricular meningioma with similarly benign metastases along the CSF pathways after surgery has also been reported.8 The patient reported here is unusual because he had an intraventricular fibroblastic meningioma in the trigone of the left lateral ventricle that developed metastases through implantation along the surgical path. The primary as well as the metastatic tumors were entirely benign. However, initially benign metastases may become progressively malignant with recurrence.6,7,9–11 Hence, radiation therapy in these patients might be justified, despite the total excision of the metastatic tumors.6–8,10,11 Simpson reported that recurrences develop either from initial inadequate resection or as a result of multifocal neoplastic change or accidental implantation of viable tumor cells during an otherwise successful resection.9 A microscopic residue along the surgical track is the most probable cause of recurrence in our patient. In our patient, the location of the tumors also confirms that surgery was probably the cause of the metastasis because multiple, discrete meningiomas were found in the gliotic plane of cleavage up to the dura in the path taken for the first surgery. All the tumors were located on the side of the surgery without any distant foci of metastasis. There is dispute on the biological significance of the histological appearance of meningiomas.9 Although the angioblastic and hemangiopericytic tumors are believed to metastasize more frequently than other histologic forms of meningioma, most investigators believe that neither the histologic pattern, local aggressiveness, size, nor the location of the tumor can be used accurately to predict which tumors will metastasize.5–7,9–12
Benign meningiomas of intraventricular location with CSF metastasis have been reported previously.6–8 Peh and Fan reported a patient with a benign fibroblastic meningioma that had progressive malignant transformation and CSF metastases after a quiescent period of 5 years,6 and McMaster et al. reported rhabdoid transformation in a recurrence of a fibroblastic intraventricular meningioma 5 years after initial surgery.7 The patient presented by Ramakrishnamurthy et al. had an intraventricular meningioma with a transitional histology that had CSF pathway metastasis with similar transitional features 4 years after the initial surgery.8 To our knowledge, this is the second report of a benign seeding metastasis of a benign intraventricular meningioma.8 We report a unique patient with benign fibroblastic intraventricular meningioma with implantation nodules that are also benign. Resection of fibroblastic intraventricular meningioma carries the risk of iatrogenic metastasis. Surgeons should wash out the operative field carefully and change surgical tools frequently. Recurrent meningiomas can be very aggressive and one should consider immediate postoperative radiotherapy. References 1. Russell DS, Rubinstein LJ. Pathology of tumours of the nervous system. Baltimore Williams and Wilkins; 1977; pp. 68–74, 89–91. 2. Fornari M, Savoiardo M, Morrello G, et al. Meningiomas of the lateral ventricles. Neuroradiological and surgical considerations in 18 cases. J Neurosurg 1981;54:64–74. 3. Shintaku M, Hashimoto K, Okamoto S. Intraventricular meningioma with anaplastic transformation and metastasis via the cerebrospinal fluid. Neuropathology 2007;27:448–52. 4. Darwish B, Munro I, Boet R, et al. Intraventricular meningioma with drop metastases and subgaleal metastatic nodule. J Clin Neurosci 2004;11:787–91. 5. Kamiya K, Inagawa T, Nagasako R. Malignant intraventricular meningioma with spinal metastasis through the cerebrospinal fluid. Surg Neurol 1989;32:213–8. 6. Peh WC, Fan YW. Case report: intraventricular meningioma with cerebellopontine angle and drop metastases. Br J Radiol 1995;68:428–30. 7. McMaster J, Ng T, Dexter M. Intraventricular rhabdoid meningioma. J Clin Neurosci 2007;14:672–5. 8. Ramakrishnamurthy TV, Murty AV, Purohit AK, et al. Benign meningioma metastasizing through CSF pathways: a case report and review of literature. Neurol India 2002;50:326–9. 9. Simpson D. The recurrence of intracranial meningiomas after surgical treatment. J Neurol Neurosurg Psychiatry 1957;20:22–39. 10. Miller DC, Ojemann RG, Proppe KH, et al. Benign metastasing meningioma. J Neurosurg 1985;62:763–6. 11. Som PM, Sacher M, Strenger SW, et al. Benign metastasizing meningiomas. AJNR 1987;8:127–30. 12. Celli P, Palma L, Domenincucci M, et al. Histologically benign recurrent meningioma metastasizing to the parotid gland; case report and review of literature. Neurosurgery 1992;31:1113–6.
doi:10.1016/j.jocn.2009.05.025
Postural tremor as a manifestation of spontaneous intracranial hypotension Nilda Turgut a,*, Ercüment Ünlü b, Mustafa Kemal Hamamcıog˘lu c, Babürhan Güldiken a, Sait Albayram d a
Department of Neurology, School of Medicine, Trakya University, Edirne 22030, Turkey Department of Radiology, School of Medicine, Trakya University, Edirne, Turkey c Department of Neurosurgery, School of Medicine, Trakya University, Edirne, Turkey d _ Department of Radiology, Division of Neuroradiology, Istanbul University Cerrahpasßa School of Medicine, Istanbul, Turkey b
* Corresponding author. Tel.: +90 2842357641; fax: +90 2842357652. E-mail address:
[email protected] (N. Turgut).
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Article history: Received 28 October 2008 Accepted 22 May 2009
Keywords: Epidural blood patching Diffuse pachymeningeal enhancement Spontaneous intracranial hypotension Tremor
a b s t r a c t Spontaneous intracranial hypotension (SIH) is a syndrome caused by low cerebrospinal fluid (CSF) pressure due to leakage of CSF. Clinically, orthostatic headache, neck pain, nausea, emesis, interscapular pain, diplopia, dizziness, changes in hearing, visual blurring and radicular upper extremity symptoms are most frequently observed. We describe a 57-year-old man with SIH who presented with postural tremor. CSF leakage was revealed by cranial MRI. Lumbar puncture identified low CSF pressure and intrathecal gadolinium enhanced MR cisternography showed diffuse CSF leakage in the thoracolumbar region. The patient underwent epidural blood patching, which resulted in complete resolution of postural tremor within 2 months. Ó 2009 Elsevier Ltd. All rights reserved.
1. Introduction
dural blood patch (EBP) at the thoracolumbar junction. He reported a decrease in tremors 2 weeks after the EBP and complete resolu-
Intracranial hypotension is a syndrome that may occur spontaneously, following rupture of an arachnoid cyst. It can also be precipitated by a lumbar puncture, spinal trauma, or cranial or spinal surgery. Orthostatic headache, neck pain, nausea, emesis, interscapular pain, diplopia, dizziness, changes in hearing, visual blurring and radicular upper extremity symptoms are common clinical features of spontaneous intracranial hypotension (SIH). These symptoms may result from traction and brain compression. A typical cranial MRI finding is diffuse pachymeningeal gadolinium enhancement.1,2 In this report, we describe a patient with SIH who presented with postural tremor. 2. Case report A 57-year-old, right-handed man was admitted to our clinic with a 5-year history of tremor. He reported that the tremor had started in his right hand and gradually increased; it then progressed to his left hand. His tremor was aggravated when he tried to do simple tasks such as drinking a glass of water or writing. In addition, he described having interscapular pain and episodic fatigue for the previous 2 years. There was no history of drug usage, head trauma, lumbar puncture, head or spinal surgery, or orthostatic headache and there was no family history of tremor. Neurological examination revealed prominent postural tremor in his hands when he held his arms outstretched, and it increased with voluntary movements. Finger and wrist joints were affected. His tremor was not orthostatic. There were no head or voice tremors, nor was there resting tremor, bradykinesia, rigidity, dystonia or speech disorder. His cognition and cranial nerve examination were normal. There was no limb weakness or pathological reflexes. Muscle stretch reflexes were normal. Laboratory blood tests, and sensory and cerebellar examinations were also within normal limits. A 7 Hz tremor activity was recorded from wrist flexors and extensors during surface electromyographic recording. Cranial MRI showed diffuse pachymeningeal enhancement overlying both cerebral hemispheres, downward displacement of cranial contents, and pituitary hyperemia (Fig. 1). A lumbar puncture (LP) showed an opening pressure of 2 cm H2O. CSF cell counts, glucose and protein levels were within normal limits. His results did not reveal infection, inflammation nor neoplasia. The patient experienced a severe headache, which was orthostatic, after LP, which decreased gradually. After assessing the MRI and his LP results, SIH was considered. One month after admission, intrathecal gadolinium enhanced MR cisternography was performed at another institute. This test identified the CSF leak at the left thoracolumbar region, and that it was due to a ruptured meningeal diverticula. Also, many bilateral thoracic and lumbar meningeal diverticula were observed (Fig. 2). During this investigation, the patient received an autologous epi-
Fig. 1. (A) Post-gadolinium coronal T1-weighted MRI showing diffuse uniform continuous dural thickening with contrast enhancement overlying both cerebral hemispheres. (B) Sagittal contrast enhanced T1-weighted MRI showing brain sagging in the patient before treatment. Effacement of perichiasmatic cisternae (arrow), pituitary hyperaemia (star), effacement of prepontine cistern with flattening of pons against the clivus, and clival epidural venous engorgement (arrowhead) are indicated.
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Fig. 3. Sagittal contrast enhanced T1-weighted MRI showing resolution of brain sagging in the patient after treatment.
Fig. 2. (A) Intrathecal MR cisternography (axial view) showing cerebrospinal fluid leak (arrow) from ruptured meningeal cyts on the left side. (B) Coronal view of the thoracolumbar area showing multiple meningeal cysts.
tion was noted 2 months later. In the month after application of the EBP, a cranial MRI revealed an obvious decrease in meningeal thickening and resolution of brain sagging (Fig. 3). 3. Discussion We report a patient with SIH with postural tremor as the only symptom. Patients with SIH usually have orthostatic headaches, neck pain, nausea, emesis, interscapular pain, diplopia, dizziness, changes in hearing, visual blurring and radicular upper extremity symptoms. SIH patients with atypical features have been reported, including Parkinsonism, ataxia and bulbar weakness. Headaches can be absent in some patients with SIH.1–3 The pathogenic mechanism of SIH is a decrease in CSF volume, and targeted EBP usually leads to the resolution of symptoms. Potential pathophysiological mechanisms contributing to dysfunction in SIH include traction and brain compression.4,5 The patient described here presented with a previously unreported movement disorder – postural tremor. Brain regions responsible for postural tremor include the primary motor and sensory areas, cerebellum, globus pallidus, red nucleus, thalamus and inferior olivary nudoi:10.1016/j.jocn.2009.05.024
cleus.6,7 A CSF leak can cause loss of hydrostatic support of the brain in the cranial vault, resulting in compression of the cerebellum, brainstem, deep midline structures, basal ganglia, and/or their connections. Therefore, we speculated that the postural tremor observed in our patient could be explained by such effects. Traction of cranial nerves and nerve roots precedes brain compression. Although our patient did not present with typical clinical signs, his MRI findings were typical for SIH, and the CSF opening pressure was low. Complete resolution of the postural tremor occurred after EBP, and confirmed that it was due to CSF leakage. There are several reports of tremors in patients caused by posterior fossa tumors, cranial trauma, Arnold Chiari malformations or hypertrophic cranial pachymeningitis that resolved after treatment or posterior fossa decompression.8–10 We demonstrated that postural tremor observed in SIH can be improved with EBP. Thus, SIH can cause reversible postural tremors. Therefore, patients with postural tremors should have MRI scans performed followed by careful examination for signs of CSF leakage. References 1. Mokri B, Posner JB. Spontaneous intracranial hypotension: the broadening clinical and imaging spectrum of CSF leaks. Neurology 2000;55:1771–2. 2. Mokri B, Ahlskog JE, Luetmer PH. Chorea as a manifestation of spontaneous CSF leak. Neurology 2006;67:1490–1. 3. Pakiam AS, Lee C, Lang AE. Intracranial hypotension with parkinsonism, ataxia, and bulbar weakness. Arch Neurol 1999;56:869–72. 4. Hong M, Shah GV, Adams KM, et al. Spontaneous intracranial hypotension causing reversible frontotemporal dementia. Neurology 2002;58:1285–7. 5. Mokri B. Spontaneous low cerebrospinal pressure/volume headaches. Curr Neurol Neurosci Rep 2004;4:117–24. 6. Bucher SF, Seelos KC, Dodel RC, et al. Activation mapping in essential tremor with functional magnetic resonance imaging. Ann Neurol 1997;41:32–40. 7. Hesselmann V, Maarouf M, Hunsche S, et al. Functional MRI for immediate monitoring stereotactic thalamotomy in a patient with essential tremor. Eur Radiol 2006;16:2229–33. 8. Kinfe TM, Capelle HH, Krauss JK. Impact of surgical treatment on tremor due to posterior fossa tumors. J Neurosurg 2008;108:692–7. 9. Vanhatalo S, Paetau R, Mustonen K, et al. Posttraumatic tremor and Arnold Chiari malformation: no sign of compression, but cure after surgical decompression. Mov Disord 2000;15:581–3. 10. Okimura Y, Tanno H, Karasudani H, et al. A case of hypertrophic cranial pachymeningitis. No Shinkei Geka 1991;19:259–62.
