JOCN-170.QXD 92 08/12/01 4:29 PM Page 92 Ng et al. CONCLUSION Trans-sphenoidal encephaloceles are rare entities and trans-sellar variety is rarest among these. We present one case of a male infant with trans-sellar trans-sphenoidal rhino-oral encephalocele and another case of an adolescent boy with a trans-sellar trans-sphenoidal rhinal encephalocele. Both patients were symptomatic and had associated anomalies. The first case had a cleft palate and the second case had cleft lip and hypertelorism. The diagnosis of these conditions has became relatively straightforward with the availability of imaging modalities, like CT scanning, MRI imaging and DSA studies. Ideal surgical approaches are still not clear due to the lack of adequate experience in the literature. We had performed trans-basal approaches and were satisfied with surgical techniques but in our first case the CSF leak resulting in fulminant meningitis was fatal. Management of these cases is a real challenge and must be meticulously planned on a case to case basis. A generalised approach may not be wise or appropriate in these cases. ACKNOWLEDGEMENT We sincerely thank Dr Paul, maxillofacial & dental surgeon, for the guidance and assistance in the management of case 2. REFERENCES Spinal epidural haematoma: MRI-aided diagnosis Wai Hoe Ng1 MBBS, C. C. Tchoyoson Lim2 FRCR, Puay Yong Ng1 FRACS, Kheng Kooi Tan1 FRCS 1 Department of Neurosurgery 2Department of Neuroradiology, National Neuroscience Institute, Singapore Summary Spinal epidural haematoma is a rare cause of spinal cord compression. It is most commonly attributed to trauma and coagulation disorders. We report a case of a 64 year-old man with thrombocytopaenia who presented with a traumatic epidural haematoma who presented with right hemiparesis which progressed to complete tetraplegia. MR of the cervical spine showed an epidural collection on the posterior aspect of the cord throughout the cervical spine. The patient underwent C1–C7 laminectomy and decompression. Postoperatively, the patient has improved gradually and has power of grade 3 (Medical Research Council classification of power) in all 4 limbs at 2-month follow-up. MRI is an important modality in the early diagnosis of spinal epidural haematomas and can facilitate emergent decompressive surgery which offers the best chance of neurological improvement. © 2002 Harcourt Publishers Ltd Journal of Clinical Neuroscience (2002) 9(1), 92–94 © 2002 Harcourt Publishers Ltd 1. Abiko S, Aoki H, Fudaba H. Trans sphenoidal encephaloceles-report of a case . Neurosurgery 1988; 27: 933–936. 2. Tsutsumi K, Asano T, Shigeno T, Matsui T, Ito S, Kaizulf. Transcranial approach for trans sphenoidal encephalocele: Report of two cases. Surg Neurol 1999; 51: 252–257. 3. Wiese GM, Kempe LG, Hammon WM . Trans sphenoidal meningoencephalocele: Case report. J Neurosurg 1972; 37: 475–478. 4. Kobayashi S, Miyazaki M, Miyagi O, Yodouaka M, Shimiza S. A case report of trans sphenoidal meningoencephalocele. Neurol Surg 1990; 18: 1671–1677 (In Japanese). 5. Itakura T, Miyamoto K, Uekatsa Y, Hayashi S, Kolval N. Bilateral morning glory syndrome associated with sphenoidal encephalocele. J Neurosurg 1992; 77: 949–951. 6. Ingraham FD, Matson DD. Spina bifida and cranium bifidum IV. An unusual nasopharyngeal encephalocele. New Eng J Med 1943; 228: 815–820. 7. Avazimi G, Crivelli G. A case of sphenopharyngeal encephalocele. Acta Neurochir 1970; 22: 205–212. 8. Ellyin F, Khatir AH, Sinyn SP. Hypothalamic–pituitary functions in patients with trans sphenoidal encephaloceles and midfacial anomalies. J Clin Endocrinol Metab 1980; 51: 854–856. 9. Shirataki K, Sakamoto K , Ohata Y. Diagnosis and treatment of trans spehenoidal encephalocele. Report of a case with review of the literature. Brain Nerve 1976; 28: 281–291 (In Japanese). 10. Takezawa N, Sato M, Yanagisawa T, Shimizu N. Pituitary dwarfism associated with morning glory syndrome and trans sphenoidal encephalocele – a case report. No To Hittatsu 1987; 19: 492–496 (In Japanese). 11. Lewin ML. Sphenoethmoidal encephalocele with cleft palate: transpalatal versus transcranial repair. Report of two cases. J Neurosurg 1983; 58: 924–931. 12. Pollock JA, Newton TH, Hoyt WF. Trans sphenoidal encephalocele. Radiology 1968; 90: 442–453. 13. Modesti LM, Glasaaer FE, Terplan KL. Sphenoethmoidal encephalocele. A case report and review of the literature. Child’s Brain 1977; 3: 140–153. 14. Yakota A, Matsukado Y, Fuwa I, Motoki K, Nagahira S. Anterior basal encephalocele of the neonatal and infantile period. Neurosurgery 1986; 19: 468–478. 15. Sakoda K, Liozumi T, Nishi Y. Basal meningoencephalocele. Proceedings of Japanese Congress of Neurological Surgeons, Tokyo, 1983 (In Japanese): 41–52. 16. Bruce DA. Anterior basal encephalocele of the neonatal and infantile period. Neurosurgery 1986; 19: 477–478. 17. Mahapatra AK. Anterior encephaloceles – a study of 65 cases. Ind J Pediatr 1997; 64: 699–704. 18. Monka M, Marubayashi T, Masamitsu T. Basal encephaloceles with morning glory syndrome and progressive hormonal and visual disturbances. Case report and review of the literature. Brain Dev 1995; 17: 196–201. Journal of Clinical Neuroscience (2002) 9(1) DOI: 10.1054/jocn.2000.0918, available online at http://www.idealibrary.com on Keywords: epidural haematoma, spinal cord compression, magnetic resonance imaging, spinal trauma Received 27 June 2000 Accepted 27 October 2000 Correspondence to: Dr Wai Hoe Ng, Department of Neurosurgery, National Neuroscience Institute, Singapore, 11 Jalan Tan Tock Seng, Singapore 308433. Tel.:;65 357 7153; Fax: ;65 256 4755. INTRODUCTION Spinal epidural haematoma is a rare condition which can present with acute spinal cord compression. Jackson first described a spontaneous case in 1869.1 Its description in the literature has mainly been confined to anecdotal reports or a small series of cases. Causation factors are related to trauma, anticoagulant therapy, blood dyscrasias, vascular malformations, epidural anaesthesia, surgery or lumbar puncture. There are also reported cases associated with cervical spondylosis and systemic lupus erythematosis. MR imaging can quite accurately characterize epidural haematomas and hence aid in early diagnosis and institution of definitive treatment. CASE REPORT A 64 year-old man presented with a history of assault. Post-injury, he was fully ambulant and had no neurological deficit. The next day, he sustained a fall at home and subsequently presented to the hospital with complaints of right-sided neck pain and right hemiparesis. On admission, there was evidence of right hemiparesis (power grade 2) and the bladder was distended. Approximately 8 h later, he developed left hemiparesis (power grade 2) and this rapidly progressed to complete tetraplegia 10 h later. © 2002 Harcourt Publishers Ltd JOCN-170.QXD 08/12/01 4:30 PM Page 93 Spinal epidural haematoma 93 High dose intravenous methylprednisolone was commenced. The patient was however noted to have thrombocytopenia with a platelet count of 62⫻109 – this was attributed to be secondary to alcoholic liver cirrhosis. The clotting profile was normal and plain radiographs of the cervical spine revealed no fracture. An urgent MRI of the cervical spine revealed an epidural collection compressing the posterior aspect of the cord throughout the cervical spinal. On T2-weighted images, the linear low signal representing the dura mater clearly delimited this collection at its superior extent from the bright signal of the CSF (Fig. 1). The signal intensity of the blood collection was slightly increased in both T1 and T2 compared to muscle, with collections of susceptibility at its inferior extent (Figs 1, 2 & 3). The prevertebral soft tissue exhibited generalized T2 prolongation, and the patient had evidence of a background of degenerative osteophytic disc disease. The platelet level was optimized and the patient subsequently underwent C1–C7 cervical laminectomy. In the immediate postoperative period, the motor strength in the left upper limb and right upper limb improved to grade 3 and grade 1 respectively. The postsurgical course was complicated by hypotension, atrial fibrillation, respiratory failure and sepsis. These were reversed by aggressive medical therapy. At 2-month follow-up, the patient has regained some neurological function with power of grade 3 in all 4 limbs. DISCUSSION Spinal epidural haematoma is a rare condition. Its true incidence is not known and there is no sexual predilection. Controversy exists Fig. 2 On T1-weighted (700/14) image, the epidural collection (arrow) is increased in signal compared to muscle. There is also evidence of degenerative spondylosis (arrowheads). Fig. 1 Fast spin-echo (FSE) T2-weighted repetition time (TR) msec/echo time (TE) msec, 4220/96 image shows an epidural fluid collection posterior to the spinal cord. The signal intensity is higher than muscle but lower than CSF, with a few collections of very low signal (arrowheads) from magnetic susceptibility within. The low intensity dura (arrow) is clearly seen. © 2002 Harcourt Publishers Ltd Fig. 3 This axial FSE T2-weighted (5040/84) image at C4-5 level shows an area of decreased signal in the haematoma with a focus of increased signal within (arrow). Note the swollen, prolonged T2 of the traumatized muscles on the right (arrowheads). Journal of Clinical Neuroscience (2002) 9(1) JOCN-170.QXD 94 08/12/01 4:30 PM Page 94 Reji Thomas et al. regarding the pathogenesis of spontaneous epidural haematoma and there is no agreement as to whether the bleeding is of arterial or venous origin.2 There exists a rich venous plexus in the epidural space and it is postulated that there is increased blood flow into the valveless epidural veins during straining.3 As such, it is believed that the epidural veins are the likely source of haemorrhage; their valveless nature also predisposes them to bleeding with minor trauma. With an associated fracture of the vertebra, cancellous bone bleeding would be an obvious source of haemorrhage. The causative agent may be anti-coagulant treatment, blood dyscrasias, vascular malformations, epidural anaesthesia,4 lumbar puncture and surgery. There has also been reports of spontaneous epidural haematomas occurring in patients with cervical spondylosis5 and systemic lupus erythematosis.6 The aetiological agent is unknown in many cases. Most cases reported in the literature have a history of anticoagulant therapy or platelet disorder; this was also the case in our patient who had thrombocytopenia secondary to alcoholic liver cirrhosis. Spinal epidural haematomas may present with back pain, radiculopathy, weakness or spinal cord compression. In rare cases, there may be a course of repeated spontaneous recovery and relapse.7 There is however no distinctive clinical sign pathognomonic for epidural haematoma. Johnston8 believes that modern radiological imaging modalities can overcome the shortfalls in clinical examination in the management of acute spinal cord compression. Since the introduction of MRI, the mean incidence of acute spontaneous spinal epidural haematoma reported in the literature has increased from 2.2 to 6.4 new cases per year.9 Indeed MRI can be considered as the imaging modality of choice. Sagittal MRI typically shows biconvex haematoma in the posterior epidural space with well-defined borders tapering superiorly and inferiorly. Axial sections would show a concave or convex mass. The dura mater separates the haematoma from the spinal cord on T1 and T2-weighted images. Boukobza et al.10 reviewed the MRI characteristics of 11 cases of spinal epidural haematomas and found that within 24 h of onset, the epidural haematoma is usually isointense on T1-weighted images. Occasionally, there is a mildly or markedly increased signal. On T2-weighted images, there may be a homogenous high signal or inhomogenous areas of mixed high and low signal. After 24 h, there is usually a high signal on T1-weighted images; T2-weighted images in most cases give the same signal as CSF. Important differential diagnosis to exclude are subdural haematoma, epidural metastasis and epidural abscess. Table 1 Grade A: complete loss of motor and sensory function below a given level Grade B: some preservation of sensation; complete motor function paralysis Grade C: sensory function useless; some motor function preserved, but insufficient to be useful Grade D: sensory function useful; weak but useful motor function Grade E: neurologically intact management by lumbar punctures.15 However, prompt surgical evacuation should remain the treatment of choice except in cases where operative morbidity and mortality is high and in cases with no neurological deficit. We also consider that the risk of injury to blood vessels and nerve roots during lumbar puncture can be high and the likelihood of adequate decompression is low. REFERENCES 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. Management Prompt surgical evacuation is the treatment of choice in spinal epidural haematomas. Rapid diagnosis and emergent surgical treatment maximises neurological recovery. Lawton et al.11 showed that patients who underwent decompression within 12 h had better neurological outcomes than patients with identical Frankel grades (Table 1) whose decompression was delayed beyond 12 h. Groen et al.12 reviewed 330 cases of spontaneous spinal epidural haematomas. They conclude that incomplete preoperative sensorimotor deficit and early surgery (within 36h) were correlated with better outcome. Mortality was higher in patients with cervical or cervicothoracic haematomas, especially in patients with cardiovascular disease and those on anti-coagulant therapy. There have been reported cases of spontaneous resolution of spinal epidural haematomas with good neurological recovery.13,14 It is postulated that this occurs from the spreading of the haematoma in the epidural space upwards and downwards resulting in decompression. Some authors have proposed conservative Journal of Clinical Neuroscience (2002) 9(1) Frankel grading 15. Jackson R. Case of spinal apoplexy. Lancet 1896; 2: 5–6. Groen RJM, Ponssen H. Vascular anatomy of the spinal epidural space: considerations on the etiology of spontaneous epidural hematoma. Clin Anat 1991; 4: 413–429. Bruyn GW, Bosma NJ. Spinal extradural haematoma. In: Vinken PJ, Bruyn GW, eds. Handbook of clinical neurology. Vol. 13 Amsterdam: Elsevier, 1976: 1–30. Gingrich TF. Spinal epidural hematoma following epidural anesthesia. Anesthesiology 1968; 29: 162–163. Sannoh N, Kubokura T, Nishimura T, Koyama S, Tsubone K. A case of spontaneous cervical hematoma associated with cervical spondylosis. NO Chinkei Geka 1988; 16(Suppl 5): 539–543. Satoh S, Hirakata M, Kameda H, Hama N, Yoshida T, Mimori T, Akizuki M, Ikeda Y. A case of systemic lupus erythematosus associated with spinal epidural hematoma. Nihon Rinsho Meneki Gakka Kaishi 1998; 21(4): 166–171. Chen CJ, Fang W, Chen CM, Wan YL. Spontaneous spinal epidural haematomas with repeated remission and relapse. Neuroradiology 1997; 39(10): 737–740. Johnston RA. The management of acute spinal cord compression. J Nurol Neurosurg Psychiatry 1993; 56:1046–1054. Jamjoom ZA. Acte spontaneous spinal epidural hematoma: the influence of magnetic resonance imaging on diagnosis and treatment. Surg Neurol 1996; 46(4): 345–349. Boukobza M, Guichard JP, Boissonet M, George B, Reizine D, Gelbert F, Meland JJ. Spinal epidural haematoma: report of 11 cases and review of the literature. Neuroradiology 1994; 36: 456–459. Lawton MT, Porter RW, Heiserman JE, Jacobowitz R, Sonntag VK, Dickman CA. Surgical management of spinal epidural hematoma: relationship between surgical timing and neurological outcome. J Neurosurg 1995; 83(1): 1–7. Groen RJ, van Alphen HA. Opeative treatment of spontaneous spinal epidural hematomas: a study of factors determining postoperative outcome. Neurosurgery 1996; 39(3): 494–508. Wagner S, Forsting M, Hacke W. Spontaneous resolution of a lage spinal epidural hematoma: case report. Neurosurgery 1996; 38(4): 816–818. Giovanni La Rosa, Domenico D’Avella, Alfredo Conti, Salvatore Cardeli, Domenico La Torre, Fabio Cacciola, Marcello Longo, Francesco Tomasello. Magnetic resonance imaging-monitored conservative management of traumatic epidural hematomas. J Neurosurg (Spine 1) 1999; 91: 128–132. Lee JI, Hong SC, Shin HJ, Eoh W, Byun HS, Kim JH. Traumatic spinal subdural hematoma: rapid resolution after repeated lumbar spinal puncture and drainage. J Trauma 1996; 40(4): 654–655. An unusual case of paroxysmal kinesigenic dyskinesia Reji Thomas1 MD, M. Behari1 MD DM , S.B. Gaikwad2 MD, K. Prasad1 MD DM 1 Departments of Neurology and 2Neuro Radiology, All India Institute of Medical Sciences, New Delhi-110 029, India © 2002 Harcourt Publishers Ltd