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Fig. 1. Brain MRI brain and MRA demonstrating reversible cerebral vasoconstriction syndrome. (a) Brain MRI T2-weighted fluid attenuated inversion recovery axial image at presentation demonstrating convexity subarachnoid haemorrhage within a right central sulcus (arrow). (b) Brain MRA coronal image at presentation demonstrating moderate diffuse calibre variation of the right M1 segment and proximal middle cerebral artery branches and left A2 (arrows) consistent with cerebral vasoconstriction. (c) Brain MRA coronal image at 3 months follow-up with resolution of the segmental narrowing of the cerebral vessels.

following carotid endarterectomy [8]. No cause is found in approximately one third of patients [4]. There are no randomised controlled trials to guide treatment of RCVS. Aggressive management of hypertension and the removal of any precipitants are the mainstays of therapy. Nimodipine has been advocated but there is no firm evidence of benefit [5]. Our case demonstrates a rare complication of GBS and an unusual cause of RCVS. It also sheds light on the potential mechanism of RCVS. Neurologists should be aware that patients with GBS and autonomic dysfunction can develop RCVS.

Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.

References [1] Hughes RA, Cornblath DR. Guillain–Barré syndrome. Lancet 2005;366:1653–66. [2] van Doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain–Barré syndrome. Lancet Neurol 2008;7:939–50. [3] Ropper AH, Wijdicks EF. Blood pressure fluctuations in the dysautonomia of Guillain–Barré syndrome. Arch Neurol 1990;47:706–8. [4] Ducros A, Boukobza M, Porcher R, et al. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain 2007;130:3091–101. [5] Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol 2012;11:906–17. [6] Lenhard T, Grimm C, Ringleb PA. Renal salt wasting as part of dysautonomia in Guillain–Barré syndrome. J Neurol Neurosurg Psychiatry 2011;82:1051–3. [7] Edvardsson B, Persson S. Reversible cerebral vasoconstriction syndrome associated with autonomic dysreflexia. J Headache Pain 2010;11:277–80. [8] Wu TY, Anderson NE, Barber PA. Neurological complications of carotid revascularisation. J Neurol Neurosurg Psychiatry 2012;83:543–50.

http://dx.doi.org/10.1016/j.jocn.2015.01.020

Occult temporal lobe encephalocoele into the transverse sinus Hamed Asadi a,⇑, Andrew Morokoff b, Frank Gaillard a,c a

Department of Radiology, Royal Melbourne Hospital, Parkville, VIC, Australia Department of Neurosurgery, Royal Melbourne Hospital, Parkville, VIC, Australia c Department of Radiology, University of Melbourne, Parkville, VIC, Australia b

a r t i c l e

i n f o

Article history: Received 29 December 2014 Accepted 4 January 2015

Keywords: Encephalocoele Neuroradiology Neurosurgery

a b s t r a c t In this article we present a case of an incidental encephalocoele protruding through a dural defect into the transverse sinus. Encephalocoeles are usually described as a herniation of the meninges and brain parenchyma through a bony defect of the cranium or base of skull. To our knowledge, there are only a few patients reported in the literature of occult encephalocoeles through dural defects. Our case study highlights that encephalocoele should be a differential diagnosis for a filling defect in this location. Ó 2015 Elsevier Ltd. All rights reserved.

1. Introduction

⇑ Corresponding author. Tel.: +61 3 93427000. E-mail address: [email protected] (H. Asadi).

An encephalocoele is traditionally defined as a herniation of the meninges and brain parenchyma through a defect of the cranium or base of skull. However, they can also occur through a dural

Case Reports / Journal of Clinical Neuroscience 22 (2015) 1202–1204

defect, most commonly seen post-operatively or following trauma. Here we present a woman with an incidental encephalocoele protruding through a dural defect into the transverse sinus. 2. Case report A 51-year-old left handed woman presented with an unwitnessed collapse followed by confusion. This was preceded by a 3 week history of twitching of the left face and arm. There were no abnormalities detected on neurological examination and an electroencephalograph was normal. The woman had a background history of obesity, depression, anxiety and smoking, and her regular medications included duloxetine, quetiapine and alprazolam. She underwent a non-contrast CT scan (Fig. 1) followed by a contrast MRI scan (Fig. 2). On CT images, a small posterior filling defect within the left transverse sinus was noted with peripheral cerebrospinal fluid (CSF) density and central soft tissue attenuation isodense to brain. This structure was associated with scalloping of the overlying bone. MRI was able to confirm that the central soft tissue component was a direct continuation of the inferior temporal gyrus, just anterior to the preoccipital notch, surrounded by a small amount of CSF. An incidental left temporal arachnoid cyst was also noted. 3. Discussion Encephalocoeles are rare, with an incidence of approximately 1 in 35,000 reported in the literature [1], and they most commonly

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affect the anterior cranial fossa and occiput [2]. Temporal lobe encephalocoeles, in contrast, occur in the middle cranial fossa or the laterotemporal cranial vault and have been classified by Wilkins et al. (Table 1) [3]. Although encephalocoeles are most commonly encountered with acquired defects in the skull such as those seen post-operatively or post-trauma, they can also occur as congenital lesions due to failure of normal skull development [2,4]. Uncommonly, encephalocoeles can also occur in congenital dural defects, such as in the reported patient, which presumably was due to a large arachnoid granulation. The pathogenesis of spontaneous temporal lobe encephalocoele is unknown. Most theories, however, postulate a role for poorly understood embryological and congenital defects in the middle cranial fossa [2,3,5,6]. The congenital defect in the bone or dura is thought to be small in origin but with time and associated factors, such as increased CSF pressure or CSF pulsations, the defect enlarges, allowing for herniation of the intracranial contents [6–9]. Underlying benign intracranial hypertension may also be responsible [10]. The presenting symptoms of temporal lobe encephalocoeles are variable and somewhat dependent on age, the location and size of the encephalocoele, and any underlying condition. Symptoms may include CSF leak, seizures, headaches, recurrent meningitis, middle ear effusions, otitis, hearing impairment, rhinorrhoea, otorrhoea, and facial nerve palsy, or they may be asymptomatic. Management of large or symptomatic encephalocoeles, particularly with CSF leak, is surgical repair [2,6]. Surgery may be considered in temporal lobe encephalocoeles associated with epilepsy

Fig. 1. Non-contrast brain CT scan (A: axial, B: sagittal, C: coronal). The arrows indicate the small encephalocoele into the left transverse sinus.

Fig. 2. Brain MRI (A: axial T2-weighted, B: sagittal T1-weighted post-contrast, C: axial T1-weighted post-contrast, D: coronal T1-weighted post-contrast). The arrows indicate the small encephalocoele into the left transverse sinus.

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Table 1 Spontaneous temporal encephalocoeles and their typical features [3] Position

Features

Lateral

Defect at pterion or occasionally asterion. Apparent in infancy, especially in females, soft mass on side of head. Defect in sphenoid wing area, encephalocoele in posterior orbit. Association with neurofibromatosis in some patients. Apparent in infancy or youth, more often in females. Slowly progressive unilateral pulsating exophthalmos. Globe usually displaced inferolaterally. Defect in anteromedial middle fossa, especially on left. Encephalocoele in sphenoid sinus. Presents in adult life, especially in females. Cerebrospinal fluid rhinorrhea, with risk of meningitis. Defect in tegmen tympani, more often on left. Frequently more than one, in tympanic antrum or epitympanic recess. Presents in adult life, cerebrospinal fluid otorrhea or otorhinorrhea. Reduced auditory acuity, risk of meningitis. Defect in anteroinferior middle fossa. Encephalocoele in infratemporal region. Presents in young adulthood, especially in females. Complex or simple partial seizures.

Anterior (splieno-orbital, posterior orbital)

Anteromedial

Posteroinferior (aural)

Anteroinferior

[11]. However, in the woman presented here, the transverse sinus encephalocoele was a small incidental finding thought not to be accountable for her symptoms and was thus managed conservatively. 4. Conclusion To our knowledge, there are only a few patients reported in the literature with occult temporal encephalocoeles [2,11,12] with http://dx.doi.org/10.1016/j.jocn.2015.01.020

bulging into the transverse sinus, which have been linked to various clinical presentations, including epilepsy or dizziness. Encephalocoele should be a differential diagnosis for a filling defect in this location. Surgical management may need to be considered if there is a clear link to the presenting symptoms but many cases are likely to be incidental.

Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.

References [1] Vannouhuys JM, Bruyn GW. Nasopharyngeal transsphenoidal encephalocele, craterlike hole in the optic disc and agenesis of the corpus callosum. Pneumoencephalographic visualisation in a case. Psychiatr Neurol Neurochir 1964;67:243–58. [2] Wind JJ, Caputy AJ, Roberti F. Spontaneous encephaloceles of the temporal lobe. Neurosurg Focus 2008;25:E11. [3] Wilkins RH, Radtke RA, Burger PC. Spontaneous temporal encephalocele. Case report. J Neurosurg 1993;78:492–8. [4] Yang E, Yeo SB, Tan TY. Temporal lobe encephalocoele presenting with seizures and hearing loss. Singapore Med J 2004;45:40–2. [5] Hyson M, Andermann F, Olivier A, et al. Occult encephaloceles and temporal lobe epilepsy: developmental and acquired lesions in the middle fossa. Neurology 1984;34:363–6. [6] Kamiya K, Mori H, Kunimatsu A, et al. Two cases of spontaneous temporal encephalocele. J Neuroradiol 2012;39:360–3. [7] Hirvonen TP, Weg N, Zinreich SJ, et al. High-resolution CT findings suggest a developmental abnormality underlying superior canal dehiscence syndrome. Acta Otolaryngol 2003;123:477–81. [8] Leblanc R, Tampieri D, Robitaille Y, et al. Developmental anterobasal temporal encephalocele and temporal lobe epilepsy. J Neurosurg 1991;74:933–9. [9] Kaufman B, Yonas H, White RJ, et al. Acquired middle cranial fossa fistulas: normal pressure and nontraumatic in origin. Neurosurgery 1979;5:466–72. [10] Brainard L, Chen DA, Aziz KM, et al. Association of benign intracranial hypertension and spontaneous encephalocele with cerebrospinal fluid leak. Otol Neurotol 2012;33:1621–4. [11] Byrne RW, Smith AP, Roh D, et al. Occult middle fossa encephaloceles in patients with temporal lobe epilepsy. World Neurosurg 2010;73:541–6. [12] Coban G, Yildirim E, Horasanli B, et al. Unusual cause of dizziness: occult temporal lobe encephalocele into transverse sinus. Clin Neurol Neurosurg 2013;115:1911–3.

Occult temporal lobe encephalocoele into the transverse sinus.

In this article we present a case of an incidental encephalocoele protruding through a dural defect into the transverse sinus. Encephalocoeles are usu...
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