- Email: [email protected]
Thymoma metastatic to the extradural spine
824 Farin et al.
Tests for antibody to MVEV and the closely related Kunjin virus (KUNV) were then undertaken. The CSF collected on admission showed a positive IgM to MVEV by an indirect fluorescent antibody test (IFAT), and a negative IgM to KUNV. Repeat CSF examination on day 14, yielded the same results as well as a negative IgM to HSV by IFAT. Serum on day 14 showed a haemagglutination inhibition titre (HIT) of 1:1280 to MVE with a positive IgM to MVEV. Further testing by an epitope-blocking EIA4 confirmed specific antibody to MVEV, but not to KUNV in the serum. There was no acute serum sample available for testing in parallel. The presence of high titre MVEV antibody in the serum and MVEV IgM in the CSF confirmed encephalitis due to MVEV.5 The patient had been continued on intravenous acyclovir (10 mg/kg t.d.s.) until the diagnosis of MVE had been rigorously established as the cause of his illness. Repeat MRI at four weeks showed marked improvement with only subtle increase in signal within the subcortical white matter of the left temporal lobe. Repeat MRI at 13 months showed no significant interval change compared to the MRI at four weeks, with unilateral left hippocampal gliosis and atrophy, most prominently involving the hippocampal head. Serology on day 54 was not significantly different from the previous samples. The patient has been left with significant personality change, flattened affect and mild cognitive difficulties particularly with verbal processing. There are no motor disturbances. DISCUSSION This patient had serologically confirmed encephalitis due to MVEV. The absence of MVE-RNA in the CSF by PCR does not exclude infection, as it is found in less than 50% of patients, even within the first few days of illness (DW Smith, unpublished data). It is highly likely that he was infected when camping in the Kimberley as surveillance data indicated MVEV in that area, but not in the Northern Territory or Pilbara regions.6 His presentation was within the described incubation period of seven to 28 days.1,7 In a review of clinical cases in the Northern Territory,3 the presence of cranial nerve palsies, tremors or rigidity in adults was suggestive of MVE and reflected deep grey matter and brainstem involvement. In our patient the clinical features suggested HSE which is known to be the commonest identified cause of non-epidemic acute encephalitis in developed countries.8 Moreover CT and MRI showed changes that were typical of HSE. Reports of neuroradiological changes in MVE are limited, with approximately seven previous reports of MRI findings in MVE,3,9,10 showing mostly bilateral thalamic or grey matter involvement. Similar changes have been reported in MRI scans in encephalitis due to other mosquito-borne flavivirus including JEV11 and West Nile virus.12 The MRI scan findings in this case instead showed the typical temporal lobe changes of HSE, a pattern not been previously reported in MVE or related arboviruses. Accurate diagnosis of MVE is important in order to appropriately manage patients and in order to inform public health authorities so that appropriate warnings can be given to residents and travellers in the risk areas. Currently there is no specific treatment for MVE. Trials of dexamethasone and alpha-interferon for JEV encephalitis did not demonstrate any benefit,13,14 and limited experience with intravenous immunoglobulin for treatment of WNV encephalitis requires validation.15 Our case reports highlights that MVE can mimic HSE, both clinically and radiologically. Therefore it is important to collect an accurate and detailed travel history from patients where there is a risk of exposure to MVEV. If suspected, antibody testing of serum and CSF should be undertaken. Testing of CSF for MVEJournal of Clinical Neuroscience (2005) 12(7)
RNA, if available, may increase the likelihood of an early diagnosis. REFERENCES 1. Mackenzie JS, Smith DW, Broom AK, Bucens MR. Australian encephalitis in Western Australia 1978–1991. Med J Aust 1993; 158: 591–595. 2. Cordova SP, Smith DW, Broom AK, Lindsay MD, Dowse GK, Beers MY. Murray Valley encephalitis in Western Australia in 2000, with evidence of southerly spread. Commun Dis Intell (Australia) 2000; 24: 368–372. 3. Burrow JNC, Whelan PI, Kilburn CJ, Fisher DA, Currie BJ, Smith DW. Australian encephalitis in the Northern Territory: clinical and epidemiological features, 1987–1996. Aust N Z J Med 1998; 28: 590–596. 4. Hall RA, Broom AK, Harnett AC, Howard MJ, Mackenzie JS. Immunodominant epitopes on the NS1 protein of MVE and KUN viruses serve as targets for a blocking ELISA to detect virus-specific antibodies in sentinel animal serum. J Virol Methods 1995; 51: 201–210. 5. Mackenzie JS, Broom AK, Calisher CH, Cloonan MJ, Cunningham AL, Gibson L, Hueston L, Lindsay MD, Marshall ID, Phillips DA, Russell RC, Sheridan J, Smith DW, Vitarana T, Worswick D. Diagnosis and reporting of arbovirus infections in Australia. Comm Dis Intell 1993; 17: 202–206. 6. Broom AK, Whelan PI, Azuolas J, Dwyer D, Hueston L, Mackenzie JS, Melville L, Ritchie SA, Smith DW. Sentinel chicken surveillance programme in Australia, 1 July 2001 to 30 June 2002. Commun Dis Intell 2002; 26: 428–429. 7. Hawkes RA. Murray Valley encephalitis and related infections. In: Porterfield JS, editor. Exotic viral infections. London: Chapman and Hall; 1995. p. 175–181. 8. Whitely RJ, Gnann JW. Viral encephalitis: familiar infections and emerging pathogens. Lancet 2002; 359: 507–514. 9. Kienzle N, Boyes L. Murray Valley encephalitis: Case report and review of neuroradiological features. Australasian Radiology 2003; 47(1): 61–63. 10. Einsiedel L, Kat E, Ravindran J, Slavotinek J, Gordon DL. MR findings in Murray Valley encephalitis. American Journal of Neuroradiology 2003; 24(7): 1379–1382. 11. Kalita J, Misra UK. Comparison of CT scan and MRI findings in the diagnosis of Japanese encephalitis. J Neurol Sci 2000; 174: 3–8. 12. Agid R, Ducreux D, Halliday WC, Kucharczyk W, terBrugge KG, Mikulis DJ. MR diffusion-weighted imaging in a case of West Nile virus encephalitis. Neurology 2003; 61(12): 1821–1823. 13. Hoke CH, Vaughn DW, Nisalak A, Intralawan P, Poolsuppasit S, Jongsawas V, Titsyakorn U, Johnson RT. Effect of high-dose dexamethasone on the outcome of acute encephalitis due to Japanese encephalitis virus. J Infect Dis 1992; 165: 631–637. 14. Solomon T, Dung NM, Wills B, Kneen R, Gainsborough M, Diet TV, Thuy TT, Loan HT, Khanh VC, Vaughn DW, White NJ, Farrar JJ. Interferon alfa-2a in Japanese encephalitis: a randomised double-blind placebo-controlled trial. Lancet 2003; 361: 821–826. 15. Haley M, Retter AS, Fowler D, Gea-Banacloche J, O'Grady NP. The role for intravenous immunoglobulin in the treatment of West Nile virus encephalitis. Clin Infect Dis 2003; 37: e88–e90.
Thymoma metastatic to the extradural spine Azadeh Farin1 MD, Henry E Aryan2 Bret B Abshire2 MD MS
MD,
1
Department of Neurosurgery, University of Southern California, Los Angeles, Division of Neurosurgery, University of California, San Diego Medical Center, San Diego; California, USA
2
Summary Background. Spinal epidural metastases are the most common spinal tumor, occurring in 10% of cancer patients. Malignant thymoma is a mediastinal tumor, with extrathoracic metastases occurring in 15% of patients to liver, kidney, and bone. Spinal metastasis is exceptionally rare. We present a case of thymoma with extradural metastasis and discuss the relevant literature. Case report. We describe a 45-year old man presenting with back pain and hypoesthesia twelve years after a diagnosis of thymoma. A
ª 2005 Elsevier Ltd. All rights reserved.
Thymoma metastatic to the extradural spine
review of the literature reveals few cases of thymoma metastatic to the extradural spine. We describe a novel surgical approach allowing ventral spinal cord decompression through a posterior incision. Conclusion. Spinal epidural metastases should be suspected in all cancer patients with back pain. Early detection of epidural metastases may enable improved pain control and preservation of spinal stability, ambulation and sphincter control. ª 2005 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2005) 12(7), 824–827 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.09.028
Keywords: spinal cord, metastasis, thymoma, extradural tumor, spinal neoplasm
825
logical examination disclosed intact cranial nerves and a normal motor exam. The sensory exam was significant for decreased light touch in the toes bilaterally. Deep tendon reflexes were reduced throughout the lower limbs, and the Babinski reflex was equivocal. Lower extremity joint position sense and proprioception were abnormal. Bladder and bowel function were normal. Imaging MRI of the entire spine was obtained. A lesion was identified at T11-12, which appeared to be extradural and involve the vertebral bodies, pedicles and posterior elements, as well as the paraspinal muscles on the right. The lesion enhanced brightly with gadolinium administration and was most suggestive of metastatic disease (Fig. 1).
Received 8 May 2004 Accepted 22 September 2004 Correspondence to: Azadeh Farin, Department of Neurosurgery, LAC-USC Medical Center, 1200 N. State St. #5046, Los Angeles CA 90033. Tel.: +1 323 226 7421; E-mail: [email protected]
INTRODUCTION Spinal epidural metastasis is the most common spinal tumor, occurring in approximately 10% of all cancer patients.1,2 Lung, breast, gastrointestinal and prostate cancer, as well as melanoma and lymphoma are the primary sites accounting for 80% of metastases.3 Most cause bony destruction, although metastatic prostate and breast cancer may cause osteoblastic changes. The most common route of metastasis is hematogenous spread to the vertebral body with erosion posteriorly through the pedicles and into the epidural space. The thoracic spine is the site of 50–60% of epidural metastases. Less than 5% of metastases are intradural or intramedullary.2 Malignant thymoma is a rare, slow-growing, anterior mediastinal tumor. Local invasion and intrathoracic spread are relatively common, and extrathoracic metastases are present in up to 15% of patients, often in the liver, kidney, and bone.4 Approximately one-third of patients with thymoma have myasthenia gravis. Invasive thymomas with a larger component of epithelial cells and association with myasthenia gravis have a poor prognosis.5 The authors describe an unusual case of a 45-year old man with a past history of thymoma presenting twelve years after primary diagnosis with back pain and sensory neuropathy. Review of the literature reveals only a few where thymoma is documented to metastasize to the extradural spine.4–13 This case is interesting as the symptoms presented twelve years after primary diagnosis. We also comment on the novel surgical approach of spondylectomy and anterolateral decompression for tumor resection.
Surgery After dissecting the paraspinal muscles from T11-L1, tumor was found within the laminae of T11 and T12, involving the facet complex at T11-12 and T12-L1, overlying the dura and encasing the T11 nerve roots bilaterally. The twelfth rib and paraspinal muscles were also involved. T11 and T12 laminectomies and partial corpectomies were performed to decompress the spinal cord and remove the invading tumor. All visualized tumor was resected. Lateral anterior interbody fusion of T10-L1 was performed after discectomy, an interbody cage was placed at T11-L1, and arthrodesis of T10-L1 was completed. Frameless stereotaxy was used for T10 to L1 pedicle screw fixation. Intraoperative biopsy showed a neoplasm consistent with metastatic thymoma. Additional biopsies were sent for permanent section examination.
CASE REPORT A 45-year old male was referred to the Neurosurgery Clinic in 2002. He complained of progressive back pain and decreased sensation in his toes bilaterally for several weeks. He denied trauma. He had been diagnosed with thymoma in 1990 after presenting with dyspnea. He was subsequently treated with chemotherapy, thymectomy, and local radiotherapy. His course was complicated by hemidiaphragm paralysis. In 1992, he developed myasthenia gravis, for which he was treated with gamma-globulin. He was subsequently stable without medication and with no evidence of disease for several years. Examination Physical examination revealed a thin, anxious male, alert and oriented, with a dry cough and a truncal maculopapular rash. Neuroª 2005 Elsevier Ltd. All rights reserved.
Fig. 1 Sagittal T1-weighted post-gadolinium MRI of the lower thoracic spine. A lesion is seen at the T11-T12 level involving the vertebral body and posterior elements with significant compression of the spinal cord, most suggestive of metastatic disease.
Journal of Clinical Neuroscience (2005) 12(7)
826 Farin et al.
Post-operative course A post-operative chest X-ray showed right middle and lower lobe opacification. Bronchoscopy revealed a large fungating mass in the right bronchus that occluded the bronchi to the right middle and lower lobes. However, clinical examination, oxygen saturation and blood gas analysis were acceptable, and the patient was extubated without complications on post-operative day one. He began ambulation in a thoracolumbar brace with a front-wheel walker on post-operative day three. At six weeks he could ambulate well without assistance, had normal lower extremity strength and sensation, and had only occasional back pain requiring acetaminophen. At nine-month follow-up, he remains disease free. Histology Histopathologic analysis of the permanent sections revealed a metastatic thymoma. The tumor was composed of large sheets and lobules of densely cellular epithelium, associated with mature lymphocytes. The epithelial cells varied from round to polygonal to spindle-shaped, and nuclei ranged from round to ovoid to elongated. Chromatin was vesicular and nucleoli were prominent. Mitoses and single-cell necrosis was identified, but zonal necrosis was not identified. The neoplastic epithelial sheets infiltrated bone, dense fibrous connective tissue, fat and muscle. DISCUSSION Spinal extradural metastases are diagnosed preoperatively by radiological studies and clinical examination findings. On MRI, vertebral metastases are usually hypointense compared to normal bone marrow on T1-weighted images and hyperintense on T2weighted images. Involvement of the posterior vertebral body and pedicle may be apparent on axial views. Pain is the most common presenting symptom, particularly exacerbated by movement, recumbency or straining. Up to two-thirds of patients with spinal epidural metastases will also have motor weakness at the time of diagnosis.1 The more severe the neurologic deficit, the less the chance of complete recovery. Ability to walk at presentation is the most important prognostic factor. Loss of sphincter control is usually irreversible and is a poor prognostic factor.2 The Brice and McKissock classification is used to grade presenting spinal cord function in patients with spinal metastases and aids determination of prognosis.14 Group 1 patients are able to walk; Group 2 patients can move their legs but cannot oppose gravity; Group 3 patients have only slight residual motor function; Group 4 patients have no motor, sensory, or sphincter function below the level of the lesion. This patient's presentation in the reported case correlates to Group 1. He presented with pain, but no weakness or loss of sphincter control, thus recovery of his mild neurologic deficits after treatment was likely. Based on the severity and acuity of the neurologic deficits at presentation, patients can be managed according to an algorithm developed by Portenoy et al.15 Patients with mild and stable signs of cord compression, such as an isolated Babinski response, plexopathy or radiculopathy without evidence of cord compression, should be admitted and evaluated within 24 hours. Low-dose dexamethasone, which often reduces pain and may result in transient neurologic improvement, is administered unless radiographic evaluation shows compression of 80% or greater. With this finding on imaging, high-dose steroids and emergency management are indicated. Dexamethasone may also have an oncolytic effect on epidural metastases. One patient has been reported with an epidural thymoma causing obstruction on myelogram, who was commenced on dexamethasone. Within 24 hours, his pain and neurological deficits had improved and within 12 days, a second Journal of Clinical Neuroscience (2005) 12(7)
myelogram showed no block. The authors suggested that a rapid response to steroids may prevent surgical exploration.10 The objectives of treatment for spinal epidural metastases are control of pain, maintenance of spinal stability and preservation of sphincter control and ambulation. Therapy has not been shown to prolong life and chemotherapy has not been shown to play a major role in the treatment of spinal epidural metastases. Epidural tumor can be treated first with surgery or radiation. Surgery is less effective for pain control than fractionated radiation, which is administered through ports extending at least one vertebral level above and below the lesion.16 Laminectomy, which is the technique generally compared to radiation, is unlikely to preserve continence and ambulation compared to radiation alone or to surgery with radiation. Laminectomy has a 9% incidence of spinal instability when tumour involves the vertebral body and is suboptimal for most spinal metastases in which the pathology is anterior to the cord.17,18 Posterolateral approaches may better address anterior tumors, but spinal instability may still result.16,19,20 As radiation is as effective as laminectomy and has fewer complications overall, surgery is indicated only in cases where the primary diagnosis is unknown or where there is spinal instability, neurologic deficit due to spinal deformity (including a compression fracture), or presence of a radioresistant tumor, such as renal cell carcinoma or melanoma. Contraindications to surgery include complete paralysis for over 24 hours, expected survival of less than 4 months and/or multiple lesions at multiple levels. If the posterior elements are not involved, a transthoracic approach with corpectomy and stabilization followed by radiation improves neurologic function in 75% and pain in 85%.21 The literature predicts a mean survival of 18 months once thymoma metastasizes to the extrathoracic region.4 We expect the long-term prognosis for this patient to be poor given similar reported cases in the literature. CONCLUSION Spinal epidural metastases should be suspected in any cancer patient with back pain that persists in recumbency, even if the patient has been treated with systemic chemotherapy for the primary neoplasm and is believed to be free of disease for a prolonged period of time. Although thymoma is usually a benign tumor, it can be malignant, and therefore should be considered in the differential diagnosis of spinal cord lesions in patients with primary thymoma. Prognostic factors particular to the primary neoplasm should be considered and add to the index of suspicion for potentially aggressive metastatic disease when early neurologic deficits develop. In thymoma, invasive tumors with a greater component of epithelial cells and association with myasthenia gravis have a worse prognosis. Early detection of spinal epidural metastases may lead to better pain control and spinal stability and preservation of sphincter control and ambulation.
REFERENCES 1. Godersky JC, Smoker WRK, Knutzon R. Use of MRI in the evaluation of metastatic spinal disease. Neurosurgery 1987; 21: 676–680. 2. Stern WE. Localization and diagnosis of spinal cord tumors. Clin Neurosurg 1977; 25: 480–494. 3. Rodichok LD, Ruckdeschel JC, Harper GR. Early detection and treatment of spinal epidural metastases: the role of myelography. Ann Neurol 1986; 20: 696–702. 4. McLennan MK. Case report 657: Malignant epithelial thymoma with osteoplastic metastasis. Skeletal Radiol 1991; 20: 141–144. 5. Alafaci C, Salpietro FM, Grasso G, et al. Spinal cord compression by a metastasizing thymoma. Acta Neurochir (Wien) 1999; 141: 215–216. 6. Akamatsu H, Tsukuura T, Matsunaga H, Suzuki A. Study of combination therapy for thymoma: a case of stage IV which presented as total spinal block
ª 2005 Elsevier Ltd. All rights reserved.
Temporosphenoidal encephalocele with CSF rhinorrhoea
7. 8.
9.
10.
11.
12. 13.
14. 15. 16. 17.
18. 19.
20.
21.
caused by epidural metastasis and which preoperative combination therapy was effective for minimizing the tumor. Kyobu Geka 1993; 46: 1156–1160. Butterworth ST, Newell JE, Stack BH. Malignant thymoma with central nervous system metastases. Br J Dis Chest 1973; 67: 141–145. Emile J, Bertrand G, Truelle JL. Spinal paraplegia revealing metastatic malignant lymphoepithelial thymoma: apropos of a case. Ann Med Interne (Paris) 1975; 126: 211–215. Kuroda R, Tanaka N, Iwata Y, Yamada R, Kanai N. Case of malignant thymoma with symptoms of spinal compression and metastases to the skull. No To Shinkei 1969; 21: 512–516. Posner JB, Howieson J, Cvitkovic E. Disappearing spinal cord compression: oncolytic effect of glucocorticoids and other chemotherapeutic agents on epidural metastases. Ann Neurol 1977; 5: 409–413. Theile U, Kutzner J, Mertsch H, Scholmerich P. Diagnostic problems in malignant thymomas with skeletal metastasizing. Med Welt 1972; 23: 1685–1688. Walther E. Thymoma: therapeutic and diagnostic aspects. Schweiz Med Wochenschr 1980; 110: 1764–1771. Ydrach AA, Marcial VA, Mercado H. Spinal cord compression by metastases-a neurosurgical and oncological emergency. Bol Assoc Med P R 1979; 71: 411–416. Brice J, McKissock W. Surgical treatment of malignant extradural spinal tumors. Br Med J 1965; 1: 1341–1344. Portenoy RK, Lipton RB, Foley KM. Back pain in the cancer patient: an algorithm for evaluation and management. Neurology 1987; 37: 134–138. Findlay GFG. Adverse effects of the management of malignant spinal cord compression. J Neurol Neurosurg Psychiatry 1984; 47: 761–768. Cooper PR, Errico TJ, Martin R, Crawford B, DiBartolo T. A systematic approach to spinal reconstruction after anterior decompression for neoplastic disease of the thoracic and lumbar spine. Neurosurgery 1993; 32: 1–8. Onimus M, Schraub S, Bertin D. Surgical treatment of vertebral metastasis. Spine 1986; 11: 883–891. Overby MC, Rothman AS. Anterolateral decompression for metastatic epidural spinal cord tumors: results of modified costotransversectomy approach. J Neurosurg 1985; 6: 344–348. Shaw B, Mansfield FL, Borges L. One-stage posterolateral decompression and stabilization for primary and metastatic vertebral tumors in the thoracic and lumbar spine. J Neurosurg 1989; 70: 405–410. Sundaresan N, Galicich JH, Lane JM. Treatment of neoplastic epidural cord compression by vertebral body resection and stabilization. J Neurosurg 1985; 63: 676–684.
Spontaneous cerebrospinal fluid rhinorrhoea due to temporosphenoidal encephalocele
827
Keywords: Cerebrospinal fluid rhinorrhoea, Encephalocele, Surgical treatment Received 26 July 2004 Accepted 22 September 2004 Correspondence to: Address: Murat Cosar MD, Bulgurlu mah. Sogutlucayir cad. Milli Sok. No:1/3 Camlica 81190 Uskudar, Istanbul, Turkey. Fax: +90 212 2217800; E-mail: [email protected]
INTRODUCTION Anteromedial temporosphenoidal encephaloceles are the least common type of temporal encephalocele. The other major types of temporal encephalocele are anterior (spheno-orbital, posterior orbital), lateral, posteroinferior (aural) and anteroinferior. We present the 13th case of anteromedial temporosphenoidal encephalocele reported in the literature. The etiology, clinical features and surgical treatment of this rare entity are discussed. CASE REPORT A 57-year old man was referred to our hospital with spontaneous CSF rhinorrhoea of 6 years duration. He had an episode of meningitis 2 months previously and was hospitalized for 20 days. There was no history of previous trauma and headaches. On examination, he had CSF leaking from the right nostril, particularly on standing. Neurological examination was otherwise normal and he had no signs of meningism on admission. CT scan showed a bony defect in the lateral wall of the right sphenoid sinus (Fig. 1). MRI revealed a right anteromedial intrasphenoidal mass protruding through the bony defects on the sphenoid wall (Fig. 2). CT cisternography confirmed a CSF leak into the right sphenoid sinus (Fig. 3). The patient was operated on via a right pterional approach. The temporal pole was mobilized and three dural defects in the lateral wall of the sphenoid sinus were seen. The largest defect was 1.5 cm in diameter with prolapse of the anteromedial temporal lobe through the underlying smooth bony defect. The temporal encephalocele was amputated, the sphenoid sinus obliterated with muscle
Kerem Bikmaz MD, Murat Cosar MD, A Celal Iplikcioglu MD, Cem Dinc MD, M Aziz Hatiboglu MD Social Security Okmeydani Training Hospital, Istanbul, Turkey
Summary A 57-year old man was referred to our hospital with spontaneous cerebrospinal fluid (CSF) rhinorrhoea of 6 years duration. He had an episode of meningitis 2 months previously. CT cisternography and cranial MRI revealed a defect in the lateral wall of the sphenoid sinus, with an anteromedial temporosphenoidal encephalocele. Surgery was performed transcranially through a pterional approach. The temporal encephalocele was amputated, the sphenoid sinus obliterated and the dural defect repaired. Lumbar drainage was used for 5 days after surgery. Spontaneous CSF rhinorrhoea is only infrequently due to temporal encephalocele. Anteromedial temporosphenoidal encephaloceles are the least common type of temporal encephalocele, with only 12 reported in the literature. ª 2005 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2005) 12(7), 827–829 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.09.030
ª 2005 Elsevier Ltd. All rights reserved.
Fig. 1 Coronal CT scan with bony windows shows the defect in the wall of the sphenoid sinus (arrow), which is partially filled with tissue density.
Journal of Clinical Neuroscience (2005) 12(7)
Tests for antibody to MVEV and the closely related Kunjin virus (KUNV) were then undertaken. The CSF collected on admission showed a positive IgM to MVEV by an indirect fluorescent antibody test (IFAT), and a negative IgM to KUNV. Repeat CSF examination on day 14, yielded the same results as well as a negative IgM to HSV by IFAT. Serum on day 14 showed a haemagglutination inhibition titre (HIT) of 1:1280 to MVE with a positive IgM to MVEV. Further testing by an epitope-blocking EIA4 confirmed specific antibody to MVEV, but not to KUNV in the serum. There was no acute serum sample available for testing in parallel. The presence of high titre MVEV antibody in the serum and MVEV IgM in the CSF confirmed encephalitis due to MVEV.5 The patient had been continued on intravenous acyclovir (10 mg/kg t.d.s.) until the diagnosis of MVE had been rigorously established as the cause of his illness. Repeat MRI at four weeks showed marked improvement with only subtle increase in signal within the subcortical white matter of the left temporal lobe. Repeat MRI at 13 months showed no significant interval change compared to the MRI at four weeks, with unilateral left hippocampal gliosis and atrophy, most prominently involving the hippocampal head. Serology on day 54 was not significantly different from the previous samples. The patient has been left with significant personality change, flattened affect and mild cognitive difficulties particularly with verbal processing. There are no motor disturbances. DISCUSSION This patient had serologically confirmed encephalitis due to MVEV. The absence of MVE-RNA in the CSF by PCR does not exclude infection, as it is found in less than 50% of patients, even within the first few days of illness (DW Smith, unpublished data). It is highly likely that he was infected when camping in the Kimberley as surveillance data indicated MVEV in that area, but not in the Northern Territory or Pilbara regions.6 His presentation was within the described incubation period of seven to 28 days.1,7 In a review of clinical cases in the Northern Territory,3 the presence of cranial nerve palsies, tremors or rigidity in adults was suggestive of MVE and reflected deep grey matter and brainstem involvement. In our patient the clinical features suggested HSE which is known to be the commonest identified cause of non-epidemic acute encephalitis in developed countries.8 Moreover CT and MRI showed changes that were typical of HSE. Reports of neuroradiological changes in MVE are limited, with approximately seven previous reports of MRI findings in MVE,3,9,10 showing mostly bilateral thalamic or grey matter involvement. Similar changes have been reported in MRI scans in encephalitis due to other mosquito-borne flavivirus including JEV11 and West Nile virus.12 The MRI scan findings in this case instead showed the typical temporal lobe changes of HSE, a pattern not been previously reported in MVE or related arboviruses. Accurate diagnosis of MVE is important in order to appropriately manage patients and in order to inform public health authorities so that appropriate warnings can be given to residents and travellers in the risk areas. Currently there is no specific treatment for MVE. Trials of dexamethasone and alpha-interferon for JEV encephalitis did not demonstrate any benefit,13,14 and limited experience with intravenous immunoglobulin for treatment of WNV encephalitis requires validation.15 Our case reports highlights that MVE can mimic HSE, both clinically and radiologically. Therefore it is important to collect an accurate and detailed travel history from patients where there is a risk of exposure to MVEV. If suspected, antibody testing of serum and CSF should be undertaken. Testing of CSF for MVEJournal of Clinical Neuroscience (2005) 12(7)
RNA, if available, may increase the likelihood of an early diagnosis. REFERENCES 1. Mackenzie JS, Smith DW, Broom AK, Bucens MR. Australian encephalitis in Western Australia 1978–1991. Med J Aust 1993; 158: 591–595. 2. Cordova SP, Smith DW, Broom AK, Lindsay MD, Dowse GK, Beers MY. Murray Valley encephalitis in Western Australia in 2000, with evidence of southerly spread. Commun Dis Intell (Australia) 2000; 24: 368–372. 3. Burrow JNC, Whelan PI, Kilburn CJ, Fisher DA, Currie BJ, Smith DW. Australian encephalitis in the Northern Territory: clinical and epidemiological features, 1987–1996. Aust N Z J Med 1998; 28: 590–596. 4. Hall RA, Broom AK, Harnett AC, Howard MJ, Mackenzie JS. Immunodominant epitopes on the NS1 protein of MVE and KUN viruses serve as targets for a blocking ELISA to detect virus-specific antibodies in sentinel animal serum. J Virol Methods 1995; 51: 201–210. 5. Mackenzie JS, Broom AK, Calisher CH, Cloonan MJ, Cunningham AL, Gibson L, Hueston L, Lindsay MD, Marshall ID, Phillips DA, Russell RC, Sheridan J, Smith DW, Vitarana T, Worswick D. Diagnosis and reporting of arbovirus infections in Australia. Comm Dis Intell 1993; 17: 202–206. 6. Broom AK, Whelan PI, Azuolas J, Dwyer D, Hueston L, Mackenzie JS, Melville L, Ritchie SA, Smith DW. Sentinel chicken surveillance programme in Australia, 1 July 2001 to 30 June 2002. Commun Dis Intell 2002; 26: 428–429. 7. Hawkes RA. Murray Valley encephalitis and related infections. In: Porterfield JS, editor. Exotic viral infections. London: Chapman and Hall; 1995. p. 175–181. 8. Whitely RJ, Gnann JW. Viral encephalitis: familiar infections and emerging pathogens. Lancet 2002; 359: 507–514. 9. Kienzle N, Boyes L. Murray Valley encephalitis: Case report and review of neuroradiological features. Australasian Radiology 2003; 47(1): 61–63. 10. Einsiedel L, Kat E, Ravindran J, Slavotinek J, Gordon DL. MR findings in Murray Valley encephalitis. American Journal of Neuroradiology 2003; 24(7): 1379–1382. 11. Kalita J, Misra UK. Comparison of CT scan and MRI findings in the diagnosis of Japanese encephalitis. J Neurol Sci 2000; 174: 3–8. 12. Agid R, Ducreux D, Halliday WC, Kucharczyk W, terBrugge KG, Mikulis DJ. MR diffusion-weighted imaging in a case of West Nile virus encephalitis. Neurology 2003; 61(12): 1821–1823. 13. Hoke CH, Vaughn DW, Nisalak A, Intralawan P, Poolsuppasit S, Jongsawas V, Titsyakorn U, Johnson RT. Effect of high-dose dexamethasone on the outcome of acute encephalitis due to Japanese encephalitis virus. J Infect Dis 1992; 165: 631–637. 14. Solomon T, Dung NM, Wills B, Kneen R, Gainsborough M, Diet TV, Thuy TT, Loan HT, Khanh VC, Vaughn DW, White NJ, Farrar JJ. Interferon alfa-2a in Japanese encephalitis: a randomised double-blind placebo-controlled trial. Lancet 2003; 361: 821–826. 15. Haley M, Retter AS, Fowler D, Gea-Banacloche J, O'Grady NP. The role for intravenous immunoglobulin in the treatment of West Nile virus encephalitis. Clin Infect Dis 2003; 37: e88–e90.
Thymoma metastatic to the extradural spine Azadeh Farin1 MD, Henry E Aryan2 Bret B Abshire2 MD MS
MD,
1
Department of Neurosurgery, University of Southern California, Los Angeles, Division of Neurosurgery, University of California, San Diego Medical Center, San Diego; California, USA
2
Summary Background. Spinal epidural metastases are the most common spinal tumor, occurring in 10% of cancer patients. Malignant thymoma is a mediastinal tumor, with extrathoracic metastases occurring in 15% of patients to liver, kidney, and bone. Spinal metastasis is exceptionally rare. We present a case of thymoma with extradural metastasis and discuss the relevant literature. Case report. We describe a 45-year old man presenting with back pain and hypoesthesia twelve years after a diagnosis of thymoma. A
ª 2005 Elsevier Ltd. All rights reserved.
Thymoma metastatic to the extradural spine
review of the literature reveals few cases of thymoma metastatic to the extradural spine. We describe a novel surgical approach allowing ventral spinal cord decompression through a posterior incision. Conclusion. Spinal epidural metastases should be suspected in all cancer patients with back pain. Early detection of epidural metastases may enable improved pain control and preservation of spinal stability, ambulation and sphincter control. ª 2005 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2005) 12(7), 824–827 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.09.028
Keywords: spinal cord, metastasis, thymoma, extradural tumor, spinal neoplasm
825
logical examination disclosed intact cranial nerves and a normal motor exam. The sensory exam was significant for decreased light touch in the toes bilaterally. Deep tendon reflexes were reduced throughout the lower limbs, and the Babinski reflex was equivocal. Lower extremity joint position sense and proprioception were abnormal. Bladder and bowel function were normal. Imaging MRI of the entire spine was obtained. A lesion was identified at T11-12, which appeared to be extradural and involve the vertebral bodies, pedicles and posterior elements, as well as the paraspinal muscles on the right. The lesion enhanced brightly with gadolinium administration and was most suggestive of metastatic disease (Fig. 1).
Received 8 May 2004 Accepted 22 September 2004 Correspondence to: Azadeh Farin, Department of Neurosurgery, LAC-USC Medical Center, 1200 N. State St. #5046, Los Angeles CA 90033. Tel.: +1 323 226 7421; E-mail: [email protected]
INTRODUCTION Spinal epidural metastasis is the most common spinal tumor, occurring in approximately 10% of all cancer patients.1,2 Lung, breast, gastrointestinal and prostate cancer, as well as melanoma and lymphoma are the primary sites accounting for 80% of metastases.3 Most cause bony destruction, although metastatic prostate and breast cancer may cause osteoblastic changes. The most common route of metastasis is hematogenous spread to the vertebral body with erosion posteriorly through the pedicles and into the epidural space. The thoracic spine is the site of 50–60% of epidural metastases. Less than 5% of metastases are intradural or intramedullary.2 Malignant thymoma is a rare, slow-growing, anterior mediastinal tumor. Local invasion and intrathoracic spread are relatively common, and extrathoracic metastases are present in up to 15% of patients, often in the liver, kidney, and bone.4 Approximately one-third of patients with thymoma have myasthenia gravis. Invasive thymomas with a larger component of epithelial cells and association with myasthenia gravis have a poor prognosis.5 The authors describe an unusual case of a 45-year old man with a past history of thymoma presenting twelve years after primary diagnosis with back pain and sensory neuropathy. Review of the literature reveals only a few where thymoma is documented to metastasize to the extradural spine.4–13 This case is interesting as the symptoms presented twelve years after primary diagnosis. We also comment on the novel surgical approach of spondylectomy and anterolateral decompression for tumor resection.
Surgery After dissecting the paraspinal muscles from T11-L1, tumor was found within the laminae of T11 and T12, involving the facet complex at T11-12 and T12-L1, overlying the dura and encasing the T11 nerve roots bilaterally. The twelfth rib and paraspinal muscles were also involved. T11 and T12 laminectomies and partial corpectomies were performed to decompress the spinal cord and remove the invading tumor. All visualized tumor was resected. Lateral anterior interbody fusion of T10-L1 was performed after discectomy, an interbody cage was placed at T11-L1, and arthrodesis of T10-L1 was completed. Frameless stereotaxy was used for T10 to L1 pedicle screw fixation. Intraoperative biopsy showed a neoplasm consistent with metastatic thymoma. Additional biopsies were sent for permanent section examination.
CASE REPORT A 45-year old male was referred to the Neurosurgery Clinic in 2002. He complained of progressive back pain and decreased sensation in his toes bilaterally for several weeks. He denied trauma. He had been diagnosed with thymoma in 1990 after presenting with dyspnea. He was subsequently treated with chemotherapy, thymectomy, and local radiotherapy. His course was complicated by hemidiaphragm paralysis. In 1992, he developed myasthenia gravis, for which he was treated with gamma-globulin. He was subsequently stable without medication and with no evidence of disease for several years. Examination Physical examination revealed a thin, anxious male, alert and oriented, with a dry cough and a truncal maculopapular rash. Neuroª 2005 Elsevier Ltd. All rights reserved.
Fig. 1 Sagittal T1-weighted post-gadolinium MRI of the lower thoracic spine. A lesion is seen at the T11-T12 level involving the vertebral body and posterior elements with significant compression of the spinal cord, most suggestive of metastatic disease.
Journal of Clinical Neuroscience (2005) 12(7)
826 Farin et al.
Post-operative course A post-operative chest X-ray showed right middle and lower lobe opacification. Bronchoscopy revealed a large fungating mass in the right bronchus that occluded the bronchi to the right middle and lower lobes. However, clinical examination, oxygen saturation and blood gas analysis were acceptable, and the patient was extubated without complications on post-operative day one. He began ambulation in a thoracolumbar brace with a front-wheel walker on post-operative day three. At six weeks he could ambulate well without assistance, had normal lower extremity strength and sensation, and had only occasional back pain requiring acetaminophen. At nine-month follow-up, he remains disease free. Histology Histopathologic analysis of the permanent sections revealed a metastatic thymoma. The tumor was composed of large sheets and lobules of densely cellular epithelium, associated with mature lymphocytes. The epithelial cells varied from round to polygonal to spindle-shaped, and nuclei ranged from round to ovoid to elongated. Chromatin was vesicular and nucleoli were prominent. Mitoses and single-cell necrosis was identified, but zonal necrosis was not identified. The neoplastic epithelial sheets infiltrated bone, dense fibrous connective tissue, fat and muscle. DISCUSSION Spinal extradural metastases are diagnosed preoperatively by radiological studies and clinical examination findings. On MRI, vertebral metastases are usually hypointense compared to normal bone marrow on T1-weighted images and hyperintense on T2weighted images. Involvement of the posterior vertebral body and pedicle may be apparent on axial views. Pain is the most common presenting symptom, particularly exacerbated by movement, recumbency or straining. Up to two-thirds of patients with spinal epidural metastases will also have motor weakness at the time of diagnosis.1 The more severe the neurologic deficit, the less the chance of complete recovery. Ability to walk at presentation is the most important prognostic factor. Loss of sphincter control is usually irreversible and is a poor prognostic factor.2 The Brice and McKissock classification is used to grade presenting spinal cord function in patients with spinal metastases and aids determination of prognosis.14 Group 1 patients are able to walk; Group 2 patients can move their legs but cannot oppose gravity; Group 3 patients have only slight residual motor function; Group 4 patients have no motor, sensory, or sphincter function below the level of the lesion. This patient's presentation in the reported case correlates to Group 1. He presented with pain, but no weakness or loss of sphincter control, thus recovery of his mild neurologic deficits after treatment was likely. Based on the severity and acuity of the neurologic deficits at presentation, patients can be managed according to an algorithm developed by Portenoy et al.15 Patients with mild and stable signs of cord compression, such as an isolated Babinski response, plexopathy or radiculopathy without evidence of cord compression, should be admitted and evaluated within 24 hours. Low-dose dexamethasone, which often reduces pain and may result in transient neurologic improvement, is administered unless radiographic evaluation shows compression of 80% or greater. With this finding on imaging, high-dose steroids and emergency management are indicated. Dexamethasone may also have an oncolytic effect on epidural metastases. One patient has been reported with an epidural thymoma causing obstruction on myelogram, who was commenced on dexamethasone. Within 24 hours, his pain and neurological deficits had improved and within 12 days, a second Journal of Clinical Neuroscience (2005) 12(7)
myelogram showed no block. The authors suggested that a rapid response to steroids may prevent surgical exploration.10 The objectives of treatment for spinal epidural metastases are control of pain, maintenance of spinal stability and preservation of sphincter control and ambulation. Therapy has not been shown to prolong life and chemotherapy has not been shown to play a major role in the treatment of spinal epidural metastases. Epidural tumor can be treated first with surgery or radiation. Surgery is less effective for pain control than fractionated radiation, which is administered through ports extending at least one vertebral level above and below the lesion.16 Laminectomy, which is the technique generally compared to radiation, is unlikely to preserve continence and ambulation compared to radiation alone or to surgery with radiation. Laminectomy has a 9% incidence of spinal instability when tumour involves the vertebral body and is suboptimal for most spinal metastases in which the pathology is anterior to the cord.17,18 Posterolateral approaches may better address anterior tumors, but spinal instability may still result.16,19,20 As radiation is as effective as laminectomy and has fewer complications overall, surgery is indicated only in cases where the primary diagnosis is unknown or where there is spinal instability, neurologic deficit due to spinal deformity (including a compression fracture), or presence of a radioresistant tumor, such as renal cell carcinoma or melanoma. Contraindications to surgery include complete paralysis for over 24 hours, expected survival of less than 4 months and/or multiple lesions at multiple levels. If the posterior elements are not involved, a transthoracic approach with corpectomy and stabilization followed by radiation improves neurologic function in 75% and pain in 85%.21 The literature predicts a mean survival of 18 months once thymoma metastasizes to the extrathoracic region.4 We expect the long-term prognosis for this patient to be poor given similar reported cases in the literature. CONCLUSION Spinal epidural metastases should be suspected in any cancer patient with back pain that persists in recumbency, even if the patient has been treated with systemic chemotherapy for the primary neoplasm and is believed to be free of disease for a prolonged period of time. Although thymoma is usually a benign tumor, it can be malignant, and therefore should be considered in the differential diagnosis of spinal cord lesions in patients with primary thymoma. Prognostic factors particular to the primary neoplasm should be considered and add to the index of suspicion for potentially aggressive metastatic disease when early neurologic deficits develop. In thymoma, invasive tumors with a greater component of epithelial cells and association with myasthenia gravis have a worse prognosis. Early detection of spinal epidural metastases may lead to better pain control and spinal stability and preservation of sphincter control and ambulation.
REFERENCES 1. Godersky JC, Smoker WRK, Knutzon R. Use of MRI in the evaluation of metastatic spinal disease. Neurosurgery 1987; 21: 676–680. 2. Stern WE. Localization and diagnosis of spinal cord tumors. Clin Neurosurg 1977; 25: 480–494. 3. Rodichok LD, Ruckdeschel JC, Harper GR. Early detection and treatment of spinal epidural metastases: the role of myelography. Ann Neurol 1986; 20: 696–702. 4. McLennan MK. Case report 657: Malignant epithelial thymoma with osteoplastic metastasis. Skeletal Radiol 1991; 20: 141–144. 5. Alafaci C, Salpietro FM, Grasso G, et al. Spinal cord compression by a metastasizing thymoma. Acta Neurochir (Wien) 1999; 141: 215–216. 6. Akamatsu H, Tsukuura T, Matsunaga H, Suzuki A. Study of combination therapy for thymoma: a case of stage IV which presented as total spinal block
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Temporosphenoidal encephalocele with CSF rhinorrhoea
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caused by epidural metastasis and which preoperative combination therapy was effective for minimizing the tumor. Kyobu Geka 1993; 46: 1156–1160. Butterworth ST, Newell JE, Stack BH. Malignant thymoma with central nervous system metastases. Br J Dis Chest 1973; 67: 141–145. Emile J, Bertrand G, Truelle JL. Spinal paraplegia revealing metastatic malignant lymphoepithelial thymoma: apropos of a case. Ann Med Interne (Paris) 1975; 126: 211–215. Kuroda R, Tanaka N, Iwata Y, Yamada R, Kanai N. Case of malignant thymoma with symptoms of spinal compression and metastases to the skull. No To Shinkei 1969; 21: 512–516. Posner JB, Howieson J, Cvitkovic E. Disappearing spinal cord compression: oncolytic effect of glucocorticoids and other chemotherapeutic agents on epidural metastases. Ann Neurol 1977; 5: 409–413. Theile U, Kutzner J, Mertsch H, Scholmerich P. Diagnostic problems in malignant thymomas with skeletal metastasizing. Med Welt 1972; 23: 1685–1688. Walther E. Thymoma: therapeutic and diagnostic aspects. Schweiz Med Wochenschr 1980; 110: 1764–1771. Ydrach AA, Marcial VA, Mercado H. Spinal cord compression by metastases-a neurosurgical and oncological emergency. Bol Assoc Med P R 1979; 71: 411–416. Brice J, McKissock W. Surgical treatment of malignant extradural spinal tumors. Br Med J 1965; 1: 1341–1344. Portenoy RK, Lipton RB, Foley KM. Back pain in the cancer patient: an algorithm for evaluation and management. Neurology 1987; 37: 134–138. Findlay GFG. Adverse effects of the management of malignant spinal cord compression. J Neurol Neurosurg Psychiatry 1984; 47: 761–768. Cooper PR, Errico TJ, Martin R, Crawford B, DiBartolo T. A systematic approach to spinal reconstruction after anterior decompression for neoplastic disease of the thoracic and lumbar spine. Neurosurgery 1993; 32: 1–8. Onimus M, Schraub S, Bertin D. Surgical treatment of vertebral metastasis. Spine 1986; 11: 883–891. Overby MC, Rothman AS. Anterolateral decompression for metastatic epidural spinal cord tumors: results of modified costotransversectomy approach. J Neurosurg 1985; 6: 344–348. Shaw B, Mansfield FL, Borges L. One-stage posterolateral decompression and stabilization for primary and metastatic vertebral tumors in the thoracic and lumbar spine. J Neurosurg 1989; 70: 405–410. Sundaresan N, Galicich JH, Lane JM. Treatment of neoplastic epidural cord compression by vertebral body resection and stabilization. J Neurosurg 1985; 63: 676–684.
Spontaneous cerebrospinal fluid rhinorrhoea due to temporosphenoidal encephalocele
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Keywords: Cerebrospinal fluid rhinorrhoea, Encephalocele, Surgical treatment Received 26 July 2004 Accepted 22 September 2004 Correspondence to: Address: Murat Cosar MD, Bulgurlu mah. Sogutlucayir cad. Milli Sok. No:1/3 Camlica 81190 Uskudar, Istanbul, Turkey. Fax: +90 212 2217800; E-mail: [email protected]
INTRODUCTION Anteromedial temporosphenoidal encephaloceles are the least common type of temporal encephalocele. The other major types of temporal encephalocele are anterior (spheno-orbital, posterior orbital), lateral, posteroinferior (aural) and anteroinferior. We present the 13th case of anteromedial temporosphenoidal encephalocele reported in the literature. The etiology, clinical features and surgical treatment of this rare entity are discussed. CASE REPORT A 57-year old man was referred to our hospital with spontaneous CSF rhinorrhoea of 6 years duration. He had an episode of meningitis 2 months previously and was hospitalized for 20 days. There was no history of previous trauma and headaches. On examination, he had CSF leaking from the right nostril, particularly on standing. Neurological examination was otherwise normal and he had no signs of meningism on admission. CT scan showed a bony defect in the lateral wall of the right sphenoid sinus (Fig. 1). MRI revealed a right anteromedial intrasphenoidal mass protruding through the bony defects on the sphenoid wall (Fig. 2). CT cisternography confirmed a CSF leak into the right sphenoid sinus (Fig. 3). The patient was operated on via a right pterional approach. The temporal pole was mobilized and three dural defects in the lateral wall of the sphenoid sinus were seen. The largest defect was 1.5 cm in diameter with prolapse of the anteromedial temporal lobe through the underlying smooth bony defect. The temporal encephalocele was amputated, the sphenoid sinus obliterated with muscle
Kerem Bikmaz MD, Murat Cosar MD, A Celal Iplikcioglu MD, Cem Dinc MD, M Aziz Hatiboglu MD Social Security Okmeydani Training Hospital, Istanbul, Turkey
Summary A 57-year old man was referred to our hospital with spontaneous cerebrospinal fluid (CSF) rhinorrhoea of 6 years duration. He had an episode of meningitis 2 months previously. CT cisternography and cranial MRI revealed a defect in the lateral wall of the sphenoid sinus, with an anteromedial temporosphenoidal encephalocele. Surgery was performed transcranially through a pterional approach. The temporal encephalocele was amputated, the sphenoid sinus obliterated and the dural defect repaired. Lumbar drainage was used for 5 days after surgery. Spontaneous CSF rhinorrhoea is only infrequently due to temporal encephalocele. Anteromedial temporosphenoidal encephaloceles are the least common type of temporal encephalocele, with only 12 reported in the literature. ª 2005 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2005) 12(7), 827–829 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.09.030
ª 2005 Elsevier Ltd. All rights reserved.
Fig. 1 Coronal CT scan with bony windows shows the defect in the wall of the sphenoid sinus (arrow), which is partially filled with tissue density.
Journal of Clinical Neuroscience (2005) 12(7)