704 Payer et al.
definition of the lesion useful for both diagnosis and surgical planning,17,18 and furthermore, to identify other associated intracranial anomalies. Meningoencephalocele can be associated with Chiari malformations, holoprosencephaly, Dandy-Walker syndrome, aqueductal stenosis, agenesis of corpus callosum, and other midline abnormalities.13,19 The treatment of intranasal basal meningoencephaloceles is solely surgical. Although the first successful operation for intranasal meningoencephalocele was performed via a transmaxillary route,20 an intracranial approach have been widely adopted more recently.1–6 Additionally, if the defect in the skull base is small, an endoscopic transnasal approach can be considered to minimize surgical complications.21 Before deciding on the operative approach, the size and site of the meningoencephalocele and the associated malformations should be properly determined by detailed neuroradiological examination.
19. Cohen Jr MM, Lemire RJ. Syndromes with cephaloceles. Teratology 1982; 25: 161–172. 20. Fenger C. Basal hernias of the brain. Am J Med Sci 1895; 62: 1–17. 21. Wormald PJ, McDonogh M. ‘Bath-plug’ technique for the endoscopic management of cerebrospinal fluid leaks. J Laryngol Otol 1997; 111: 1042–1046.
CONCLUSION
Summary We present a case of traumatic vertical atlantoaxial dislocation of 16 millimetres with a fatal outcome. We hypothesize that this extremely rare traumatic vertical atlantoaxial dislocation results from insufficiency of the C1/C2 facet capsules after rupture of the tectorial membrane and the alar ligaments. ª 2005 Elsevier Ltd. All rights reserved.
An adult case of intranasal transethmoidal meningoencephalocele with recurrent meningitis is reported. In a patient with recurrent intracranial infection but without history of immunodeficiency, cranial trauma or neurosurgical operation, a congenital anomaly of the skull base, including meningoencephalocele, should be considered even in an elderly patient. Complete neuroimaging studies and successful surgical repair are essential to avoid subsequent neurological complications.
Traumatic vertical atlantoaxial dislocation M Payer1
MD,
S Wetzel2
1
MD,
A Kelekis2
MD,
B Jenny1
MD
2
Department of Neurosurgery and Department of Neuroradiology, University Hospital of Geneva, Switzerland
Journal of Clinical Neuroscience (2005) 12(6), 704–706 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.03.043
Keywords: cervical spine trauma, atlantoaxial dislocation Accepted 19 March 2004 REFERENCES 1. Izquierdo JM, Gil-Carcedo LM. Recurrent meningitis and transethmoidal intranasal meningoencephalocele. Dev Med Child Neurol 1988; 30: 248–251. 2. Hayashi T, Utsunomiya H, Hashimoto T. Transethmoidal encephalomeningocele. Surg Neurol 1985; 24: 651–655. 3. Sekerci Z, Iyigun O, Baris S, et al. Intranasal (transethmoidal) encephalomeningocele. Case report. Neurosurg Rev 1995; 18: 123–126. 4. Dempsey PK, Harbaugh RE. Encephalomeningocele presenting with spontaneous cerebrospinal fluid rhinorrhea in an elderly man: case report. Neurosurgery 1988; 23: 637–640. 5. Copty M, Verret S, Langelier R, Contreras C. Intranasal meningoencephalocele with recurrent meningitis. Surg Neurol 1979; 12: 49–52. 6. Abiko S, Aoki H, Hiyoyoshi F. Intrasphenoidal encephalocele: Report of a case. Neurosurg 1988; 22: 933–936. 7. Smith DE, Murphy MJ, Hitchon PW, Babin RW, Abu-Yousef MM. Transsphenoidal encephaloceles. Surg Neurol 1983; 20: 471–480. 8. Donnenfeld AE, Hughes H, Weiner S. Prenatal diagnosis and perinatal management of frontoethmoidal meningoencephalocele. Am J Perinatol 1988; 5: 51–53. 9. Suwanwela C, Hongsaprabhas C. Fronto-ethmoidal encephalomeningocele. J Neurosurg 1966; 25: 172–182. 10. Pollock JA, Newton TH, Hoyt WF. Transsphenoidal encephalocele. Radiology 1968; 90: 442–453. 11. Mood GF. Congenital anterior herniations of brain. Ann Otorhinolaryngol 1938; 47: 391–401. 12. Lewin ML, Shuster MM. Transpalatal correction of basilar meningocele with cleft palate. Arch Surg 1965; 90: 687–693. 13. Sakoda K, Ishikawa S, Uozumi T, Hirakawa K, Okazaki H, Harada Y. Sphenoethmoidal meningoencephalocele associated with agenesis of the corpus callosum and median cleft lip and palate. Case report. J Neurosurg 1979; 51: 397–401. 14. Stuart EA. An otolaryngologic aspect of frontal meningocele. Arch Otolaryngol 1949; 40: 171–174. 15. Suwanwela C, Suwanwela N. A morphological classification of sincipital encephalomeningocele. J Neurosurg 1972; 36: 201–211. 16. Mazzola RF. Congenital malformations in the frontonasal area: Their pathogenesis and classification. Clin Plast Surg 1976; 3: 573–609. 17. Byrd SE, Harwood-Nash DC, Fitz CR, Rogovitz DM. Computed tomography in the evaluation of encephaloceles in infants and children. J Comput Assist Tomogr 1978; 2: 81–87. 18. Diebler C, Dulac O. Cephaloceles: clinical and neuroradiological appearance. Associated cerebral malformations. Neuroradiology 1983; 25: 199–216.
Journal of Clinical Neuroscience (2005) 12(6)
Correspondence to: M. Payer, Department of Neurosurgery, University Hospital of Geneva, Rue Micheli-du-Crest 24, 1211 Gene`ve 14, Switzerland. Tel.: +41 22 372 82 24; Fax: +41 22 372 82 20; E-mail:
[email protected]
INTRODUCTION Traumatic vertical atlanto-occipital dislocation (AOD) is a rare and often fatal injury, most commonly observed in children involved in traffic accidents.1–7 Traumatic vertical atlanto-axial dislocation (AAD) is even less common. One case associated with atlanto-occipital coalition8 and one case with no cranio-cervical junction anomaly9 have been reported. We present another case of a purely traumatic vertical AAD without an associated cranio-cervical junction anomaly. CASE DESCRIPTION A 64-year old man riding a scooter and wearing a helmet collided head-on with a car. He was found unconscious with a GCS of 3 and intubated. Adrenaline was given during resuscitation for cardiac arrest and a rigid collar applied. High-dose prednisolone according to the NASCIS-2 study protocol10 was commenced. Five hours later he arrived at our institution for neurosurgical management. He was hypotensive and bradycardic despite intravenous adrenalin. Clinical examination revealed pinpoint pupils, absence of both corneal reflexes, a diminished gag reflex, paradoxical respiratory movements, a flaccid paralysis of arms and legs and absence of all tendon reflexes. There was no significant chest or abdominal trauma. Plain antero-posterior and lateral cervical spine X-rays showed an impressive vertical AAD of 16 millimetres with slight displacement of the atlas towards the right (Fig. 1). The anterior atlantoaxial interval measured 5 millimetres. ª 2005 Elsevier Ltd. All rights reserved.
Traumatic vertical atlantoaxial dislocation 705
Fig. 2 Sagittal T2-weighted cervical spine MRI. The position of the anterior arch of C1 is marked with a black arrow. Slight hyperintensity of the spinal cord at that level is noted (white arrow). Fig. 1 Plain antero-posterior (a) and lateral (b) cervical spine X-rays showing vertical AAD of 16 millimeters with slight displacement of the atlas towards the right.
Cervical CT scan revealed no other lesions of the cervical spine in addition to the findings on the plain radiographs. A cerebral CT scan showed marked posterior fossa oedema and no other craniocerebral lesions. Emergency cervical spine MRI showed a haemorrhage between the anterior arch of C1 and the dens extending to the anterior epidural space without spinal cord compression (Fig. 2). Discrete intramedullary oedema was seen at the level of C1-C2. No congenital, metabolic or inflammatory disorders of the cranio-cervical junction were seen on MRI. Despite high doses of adrenalin and noradrenalin the mean arterial blood pressure could not be maintained and the patient died after the MRI examination.
DISCUSSION We could only identify one case of traumatic vertical atlanto-axial dislocation without an associated cranio-cervical junction anomaly in the English literature.9 We thus investigated why our patient had a vertical atlanto-axial dislocation rather than the more common AOD.1–7 In traumatic vertical AOD, forced hyperextension combined with lateral flexion and/or hyperflexion is assumed to rupture the most important occipito-cervical ligaments; the tectorial membrane and the alar ligaments. Some or all of the other occipitocervical ligaments (apical dental ligament, vertical bands of the ª 2005 Elsevier Ltd. All rights reserved.
cruciate ligament) may also be ruptured.2–6 The relatively weak anterior, posterior and lateral atlanto-occipital membranes thereby become insufficient in such a way that vertical AOD occurs.2 Since all the above-mentioned occipito-cervical ligaments (tectorial membrane, alar ligaments, apical dental ligament, cruciate ligament with its vertical bands) connect C2 to C0, their rupture leads to instability of both the C0-C1 and C1-C2 segment. We propose that the C1/C2 facet capsules must be stronger than the atlanto-occipital membranes in order for AOD to occur after rupture of the tectorial membrane and the alar ligaments, although thus far, no biomechanical experiment has been demonstrated in the literature to prove this. On the other hand, if vertical AAD occurs, we hypothesize that the C1/C2 facet capsules may be less resistant to axial distraction than the atlanto-occipital membranes. Pre-existing pathology of the C1/C2 articulations, such as rheumatoid arthritis or ankylosing spondylitis,11,12 could explain their weakened function, but was not found in our patient. A rotatory atlanto-axial subluxation or dislocation during trauma, commonly observed in children, results in C1/C2 joint dysfunction.13–15 No signs of rotatory atlanto-axial subluxation were seen on CT in our patient, but theoretically could have occurred with spontaneous reduction prior to imaging. Our patient had no previous history of neck trauma potentially affecting C1/C2 stability. C0-C1 coalition would transfer all occipito-cervical junction forces to the C1/C2 joint, as reported in one case.8 Our patient had no occipito-cervical junction anomaly. We cannot fully explain vertical AAD in our patient rather than vertical AOD. We hypothesize that it resulted from insufficiency Journal of Clinical Neuroscience (2005) 12(6)
706 Short et al.
of the C1/C2 facet capsules after rupture of the tectorial membrane and alar ligaments. We cannot discuss the treatment of vertical AAD, as our patient died relatively early from cardio-vascular decompensation. The vertical AAD of 16 millimeters probably led to cardio-vascular decompensation from progressive oedema at the cervico-medullary junction or from progressive oedema in the posterior fossa due to dissection, thrombosis or spasm of the vertebral arteries. The other reported patient with vertical AAD survived with no spinal cord or brainstem injury. In this patient immediate external reduction by applying axial pressure on the head under fluoroscopy and secondary posterior occipito-cervical fixation were performed.9 We therefore think that after establishing the diagnosis on plain radiographs and CT, immediate reduction by applying axial pressure on the head under fluoroscopy followed by internal fixation and fusion probably offers the best chances for survival with this pathology.
CONCLUSION This is the second case of a purely traumatic vertical AAD without associated cranio-cervical junction pathology reported in the English literature. We hypothesize that after rupture of the tectorial membrane and the alar ligaments, the C1/C2 articular capsules become less resistant to axial distraction than the atlanto-occipital membranes. Due to the rarity of this pathology, no treatment guidelines can be established, but applying axial pressure on the head under fluoroscopy to reduce the AAD followed by internal fixation and fusion is appropriate.
REFERENCES 1. Chaljub G, Singh H, Gunito Jr FC, Crow WN. Traumatic atlanto-occipital dislocation: MRI and CT. Neuroradiology 2001; 43: 41–44. 2. Cohen A, Hirsch M, Katz M, Sofer S. Traumatic atlanto-occipital dislocation in children: review and report of five cases. Pediatr Emerg Care 1991; 7: 24–27. 3. Hladky JP, Lejeune JP, Leclercq F, Dhellemmes P, Christiaens JL. La dislocation occipito-atloidienne traumatique. (French) Neurochirurgie 1991; 37: 312–317. 4. Houle P, McDonnell DE, Vender J. Traumatic atlanto-occipital dislocation in children. Pediatr Neurosurg 2001; 34: 193–197. 5. Kenter K, Worley G, Griffin T, Fitch RD. Pediatric traumatic atlanto-occipital dislocation: five cases and a review. J Pediatr Orthop 2001; 21: 585–589. 6. Labbe JL, Leclair O, Duparc B. Traumatic atlanto-occipital dislocation with survival in children. J Pediatr Orthop Part B 2001; 10: 319–327. 7. Takayasu M, Hara M, Suzuki Y, Yoshida J. Treatment of atlanto-occipital dislocation in chronic phase. Neurosurg Rev 1999; 22: 135–137. 8. Weiner BK, Brower RS. Traumatic vertical atlantoaxial instability in a case of atlanto-occipital coalition. Spine 1997; 22: 1033–1035. 9. Ramar S, Lazennec JY, Camelot C, Saillant G, Hansen S, Trabelsi R. Vertical atlantoaxial dislocation. Eur Spine J 1999; 8: 241–243. 10. Bracken MB, Shepard MJ, Collins WF, et al. A randomized, controlled trial of methylprednisolone or naloxone in the treatment of acute spinal-cord injury. Results of the Second National Acute Spinal Cord Injury Study. N Engl J Med 1990; 322: 1405–1411. 11. Henderson DR. Vertical atlanto-axial subluxation in rheumatoid arthritis. Rheumatol Rehabil 1975; 14: 31–38. 12. Ramos-Remus C, Gomez-Vargas A, Hernandez-Chavez A, Gamez-Nava JI, Gonzalez-Lopez L, Russell AS. Two year followup of anterior and vertical atlantoaxial subluxation in ankylosing spondylitis. J Rheumatol 1997; 24: 507–510. 13. Crockard HA, Rogers MA. Open reduction of traumatic atlanto-axial rotatory dislocation with use of the extreme lateral approach. A report of two cases. J Bone Joint Surg (Am) 1996; 78A: 431–436. 14. Weisskopf M, Mittlmeier T, Hoffmann R. Fixed rotatory subluxation of the atlanto-axial joint in polytrauma patients. Chirurg 1999; 70: 818–822. 15. Wise JJ, Cheney R, Fischgrund J. Traumatic bilateral rotatory dislocation of the atlanto-axial joints: a case report and review of the literature. J Spinal Disord 1997; 10: 451–453.
Journal of Clinical Neuroscience (2005) 12(6)
A case of presumptive primary lateral sclerosis with upper and lower motor neurone pathology Cathy L. Short1 MBBS BSC FRACP, Grace Scott2 Peter C. Blumbergs2 MBBS FRACP FRCPA, Simon A. Koblar1,3 BM BS PHD FRACP
BSC MBCHB,
1
Department of Neurology, The Queen Elizabeth Hospital, South Australia, Department of Neuropathology, Institute of Medical and Veterinary Science, South Australia, 3University Department of Medicine, University of Adelaide, The Queen Elizabeth Hospital, South Australia 2
Summary Motor Neurone Disease (MND) is one of the commonest neurodegenerative disorders of adulthood. MND characteristically presents with a combination of both upper and lower motor neurone features. Primary Lateral Sclerosis (PLS) is thought to be a variant of MND presenting with purely upper motor neurone signs. Debate continues over whether PLS constitutes a distinct pathological entity or whether it is part of the spectrum of motor neurone diseases that present as an upper motor neurone-predominant form of MND. We present a case of MND with purely upper motor neurone features and a prominent pain component. A pre-mortem diagnosis of PLS was made, however autopsy findings demonstrated both upper and lower motor neurone involvement. We believe these findings support the view that PLS is not a discrete pathological entity, but that it is a part of the range of motor neurone diseases that present with predominant but not exclusive upper motor neurone involvement. This case also highlights the feature that pain may be associated with MND even though it is not appreciated to have a sensory pathology. ª 2005 Published by Elsevier Ltd. Journal of Clinical Neuroscience (2005) 12(6), 706–709 0967-5868/$ - see front matter ª 2005 Published by Elsevier Ltd. doi:10.1016/j.jocn.2004.08.032
Keywords: primary lateral sclerosis, motor neurone disease, pain Received 17 July 2004 Accepted 25 August 2004 Correspondence to: Dr. S.A. Koblar, University Department of Medicine, University of Adelaide, The Queen Elizabeth Hospital, Woodville Rd, Woodville, South Australia, 5011, Australia. Tel.: +618 8222612; Fax: 618-82226042; E-mail:
[email protected]
INTRODUCTION Motor Neurone Disease (MND) or Amyotrophic Lateral Sclerosis (ALS) is one of the most common neurodegenerative disorders occurring in adult life.1 The clinical hallmarks of this disease are a combination of both upper and lower motor neurone features including muscle wasting and weakness, fasciculations, spasticity, brisk reflexes and extensor plantar responses.2 Although the diagnosis is essentially a clinical one, a variety of investigations are usually carried out to exclude other potentially reversible causes of such a presentation. Electrophysiological tests can be undertaken to help support the diagnosis of MND. In particular, electroª 2005 Published by Elsevier Ltd.
