Downloaded from http://pn.bmj.com/ on October 8, 2017 - Published by group.bmj.com
A DIFFICULT CASE
Sagging brain causing postural loss of consciousness: a case of severe spontaneous intracranial hypotension Tjerk Joppe Lagrand, Richard Beukers ▸ To view the online supplementary video please visit the journal online (http://dx.doi. org/10.1136/practneurol-2015001183). Department of Neurology, Medical Centre Alkmaar, Alkmaar, The Netherlands Correspondence to Dr Tjerk Joppe Lagrand, Department of Neurology, Medical Centre Alkmaar, Wilhelminalaan 12, Alkmaar, 1815 JD, The Netherlands; [email protected] Accepted 5 July 2015 Published Online First 5 August 2015
To cite: Lagrand TJ, Beukers R. Pract Neurol 2015;15:471–473.
CASE REPORT A 69-year-old man presented with progressive headache and nausea. Ten days earlier, he had developed an acute neck pain after a workout on his home trainer. Three days later, he had hit his head on a door, and later developed a frontal headache that increased during exercise. His neurological examination was normal, but CT scan of the head showed bilateral subdural haematomas of mixed density. He was admitted for observation, and managed conservatively. An unenhanced MR scan of the brain confirmed subdural collections, but also showed an abnormal configuration of the midbrain, which was swollen and elongated with severe brain sagging (figure 1). Over the next few days, he became increasingly somnolent and bradyphrenic. After 3 days, he developed anisocoria. He was taken immediately to the operating room, and had two skull burr holes, releasing ‘crankcase oil-coloured’ fluid under low pressure. He improved immediately after surgery. However, when walking with the physiotherapist, he developed impaired arousal that resolved when he lay flat. We tested his mental status in both the Trendelenburg posture and upright posture (see figure 2 and online supplementary video S1); in just 5 min, his consciousness changed from full alertness to coma. A contrastenhanced MR myelogram (T1-weighted) showed a longitudinally extensive epidural collection of contrast-enhancing cerebrospinal fluid (CSF) in the thoracic and lumbar spine. There were two CSF leaks along the right-sided nerve roots at L2 (figure 3) and T6. He underwent an epidural blood patch, and within 24 h, his conscious level improved dramatically. After a week, he could go home. Repeat MR imaging at 6 months showed no
subdural collections or features of intracranial hypotension, and his midbrain morphology was completely normal. DISCUSSION In 1938, Schaltenbrand1 described ‘aliquorrhoea’, a syndrome of postural headache, meningism, nausea, vomiting, tinnitus and vertigo in patients with a low CSF pressure. Initially, the syndrome was thought to be caused by reduced fluid production in the intraventricular choroid plexus. However, more recent neuroimaging studies have shown CSF leakage to be the cause.2 3 Although spontaneous intracranial hypotension can present in many ways, they all share the criterion of orthostatic headache. Other rarer features include galactorrhoea, syringomyelia, seizures, parkinsonism and coma.4–7 This broad spectrum of presentations may not meet the 2004 International Headache Society diagnostic criteria for spontaneous intracranial hypotension, explaining why it is often misdiagnosed.8 Recently, Schievink et al9 proposed diagnostic criteria for spontaneous intracranial hypotension reflecting these variable manifestations, making them more useful in practice. Among these criteria are the characteristic MR imaging features, including pachymeningeal enhancement, subdural fluid collections, venous engorgement with dural sinus dilatation, pituitary gland enlargement and brain sagging.10 Most of these MR imaging features probably result from venous dilatation compensating for a reduced CSF pressure. This assumption is based on the Monro– Kellie hypothesis: in an incompressible compartment, as is the cranium, its constituents (blood, CSF and brain tissue) form a volume equilibrium. Thus, when one constituent decreases, there must be an
Lagrand TJ, Beukers R. Pract Neurol 2015;15:471–473. doi:10.1136/practneurol-2015-001183
471
Downloaded from http://pn.bmj.com/ on October 8, 2017 - Published by group.bmj.com
A DIFFICULT CASE
Figure 1 MR scan of brain (mid-sagittal T1-weighted) showing midbrain swelling (black arrow) and severe brain sagging (white arrows).
increase in one of the other, to maintain homeostasis. According to this concept, CSF leakage may cause venous engorgement, extravasation of fluid from the dural vasculature into the innermost dural border cell (causing pachymeningeal enhancement on MRI), and in the worst cases, there is rupture of bridging veins, causing subdural haematomas. Brain sagging results when this compensation mechanism fails, and CSF cannot provide protective buoyancy any more. The downward displacement of the brain through the tentorial notch causes traction on pain-sensitive structures; also, brainstem compression may cause cranial nerve palsies and even depression of vital centres.11 12 Savoiardo et al described a few cases of brain sagging, with swelling of diencephalic–mesencephalic structures and increased diffusivity on MRI. They hypothesised that impaired venous drainage caused venous stagnation and, hence, vasogenic oedema. Thus, in applying the Monro–Kellie doctrine, the volume of the brain is not constant.13 In spontaneous intracranial hypotension, lumbar puncture may show a low opening pressure, often below 60 mm H2O. Sometimes, there is a ‘sucking noise’ after the stylet is withdrawn as air enters the subarachnoid space.14 CT and MR myelography after intrathecal contrast injection can detect CSF leaks, and offer the additional possibility to inject an epidural blood patch.15 The first treatment for spontaneous intracranial hypotension is for the patient to lie supine, relieving traction on the pain-sensitive structures. This typically resolves the headache and reduces CSF pressure in the
472
Figure 2 A still from the online supplementary video, neurologic examination in horizontal and after 5 minutes in upright position.
thecal sac, allowing meningeal defects to restore. Sufficient hydration, increased salt intake, caffeine and theophylline may be effective, whereas corticosteroids are of questionable value.16 17 Although over half of the patients recover with conservative management, those with persisting symptoms may be helped by an epidural blood patch, especially if there is a detected CSF leak. Injection of autologous blood replaces CSF volume and seals leaks in the meninges.18 Patients with persisting symptoms can have a repeat blood patch, or may be helped by percutaneous placement of a fibrin sealant.19 When all other treatments fail, surgical intervention must be considered. This option requires the demonstration of an exact site of CSF leakage; however, it may be effective with an almost 100% reduction of headache.20 Our case shows the variety of symptoms associated with spontaneous intracranial hypotension. We were initially misled by his head trauma in the week before his presentation. When he deteriorated, we assumed this was caused by his subdural haematomas; however, in retrospect, the initial MR scan showed all the characteristic features of spontaneous intracranial hypotension as well as mesencephalic swelling. The perioperative low CSF pressure and the persisting orthostatic symptoms suggested spontaneous intracranial hypotension, further corroborated by the MR myelogram showing two CSF leaks.
Lagrand TJ, Beukers R. Pract Neurol 2015;15:471–473. doi:10.1136/practneurol-2015-001183
Downloaded from http://pn.bmj.com/ on October 8, 2017 - Published by group.bmj.com
A DIFFICULT CASE
Figure 3 MR scan of lumbar spine (coronal and axial T1 contrast-enhanced) showing cerebrospinal fluid leakage along the right L2 nerve root (white arrows).
Key points ▸ Characteristic MRI features of intracranial hypotension include pachymeningeal enhancement, subdural fluid collections, venous engorgement with dural sinus dilatation, pituitary gland enlargement and brain sagging. ▸ Clinicians should suspect spontaneous intracranial hypotension in patients with bilateral subdural haematomas and orthostatic headaches. Contributors TJL wrote the first draft and prepared the manuscript. RB participated in the manuscript preparation and revisions, and helped to bring the manuscript to its final version. Competing interests None declared. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Mark Manford, Cambridge, UK.
REFERENCES 1 Schaltenbrand VG. Neuere anschauungen zur pathophysiologie der liquorzirkulation. Zentralbl Neurochir 1938;3:290–9. 2 Bell W, Joynt R, Sahs A. Low spinal fluid pressure syndromes. Neurology 1960;10:512–21. 3 Mokri B, Posner JB. Spontaneous intracranial hypotension. Neurology 2000;55:1771–2. 4 Rahman M, Bidari SS, Quisling RG, et al. Spontaneous intracranial hypotension: dilemmas in diagnosis. Neurosurgery 2011;69:4–14. 5 Sharma P, Sharma A, Chacko AG. Syringomyelia in spontaneous intracranial hypotension. J Neurosurg 2001;95:905–8. 6 Hong M, Shah GV, Adams KM, et al. Spontaneous intracranial hypotension causing reversible frontotemporal dementia. Neurology 2002;58:1285–7.
7 Evan RW, Mokri B. Spontaneous intracranial hypotension resulting in coma. Headache 2002;42:159–60. 8 Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders: 2nd edition. Cephalgia 2004;24(Suppl 1):9–160. 9 Schievink WI, Dodick DW, Mokri B, et al. Diagnostic criteria for headache due to spontaneous intracranial hypotension: a perspective. Headache 2011;51:1442–4. 10 Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 2006;295:2286–96. 11 Pannullo SC, Reich JB, Krol G, et al. MRI changes in intracranial hypotension. Neurology 1993;43:919–26. 12 Paldino M, Mogilner AY, Tenner MS. Intracranial hypotension syndrome: a comprehensive review. Neurosurg Focus 2003;15: ECP2. 13 Savoiardo M, Minati L, Farina L, et al. Spontaneous intracranial hypotension with deep brain swelling. Brain 2007;130:1884–93. 14 Schievink WI, Reimer R, Folger WN. Surgical treatment of spontaneous intracranial hypotension associated with a spinal arachnoid diverticulum. J Neurosurg 1994;80:736–9. 15 Albes G, Weng H, Horvath D, et al. Detection and treatment of spinal CSF leaks in idiopathic intracranial hypotension. Neuradiology 2012;54:1367–73. 16 Marcelis J, Silberstein SD. Spontaneous low cerebrospinal fluid pressure headache. Headache 1990;30:192–6. 17 Mokri B, Piepgras DG, Miller GM. Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc 1997;72:400–13. 18 Sencakova D, Mokri B, McClelland RL. The efficacy of epidural blood path in spontaneous CSF leaks. Neurology 2001;57:1921–3. 19 Mehta B, Tarshis J. Repeated large-volume epidural blood patches for the treatment of spontaneous intracranial hypotension. Can J Anaesth 2009;56:609–13. 20 Schievink WI, Morreale V, Atkinson J, et al. Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. J Neurosurg 1998;88:243–6.
Lagrand TJ, Beukers R. Pract Neurol 2015;15:471–473. doi:10.1136/practneurol-2015-001183
473
Downloaded from http://pn.bmj.com/ on October 8, 2017 - Published by group.bmj.com
Sagging brain causing postural loss of consciousness: a case of severe spontaneous intracranial hypotension Tjerk Joppe Lagrand and Richard Beukers Pract Neurol 2015 15: 471-473 originally published online August 5, 2015
doi: 10.1136/practneurol-2015-001183 Updated information and services can be found at: http://pn.bmj.com/content/15/6/471
These include:
Supplementary Supplementary material can be found at: Material http://pn.bmj.com/content/suppl/2015/08/05/practneurol-2015-001183 .DC1
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This article cites 20 articles, 0 of which you can access for free at: http://pn.bmj.com/content/15/6/471#BIBL Receive free email alerts when new articles cite this article. Sign up in the box at the top right corner of the online article.
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To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/
A DIFFICULT CASE
Sagging brain causing postural loss of consciousness: a case of severe spontaneous intracranial hypotension Tjerk Joppe Lagrand, Richard Beukers ▸ To view the online supplementary video please visit the journal online (http://dx.doi. org/10.1136/practneurol-2015001183). Department of Neurology, Medical Centre Alkmaar, Alkmaar, The Netherlands Correspondence to Dr Tjerk Joppe Lagrand, Department of Neurology, Medical Centre Alkmaar, Wilhelminalaan 12, Alkmaar, 1815 JD, The Netherlands; [email protected] Accepted 5 July 2015 Published Online First 5 August 2015
To cite: Lagrand TJ, Beukers R. Pract Neurol 2015;15:471–473.
CASE REPORT A 69-year-old man presented with progressive headache and nausea. Ten days earlier, he had developed an acute neck pain after a workout on his home trainer. Three days later, he had hit his head on a door, and later developed a frontal headache that increased during exercise. His neurological examination was normal, but CT scan of the head showed bilateral subdural haematomas of mixed density. He was admitted for observation, and managed conservatively. An unenhanced MR scan of the brain confirmed subdural collections, but also showed an abnormal configuration of the midbrain, which was swollen and elongated with severe brain sagging (figure 1). Over the next few days, he became increasingly somnolent and bradyphrenic. After 3 days, he developed anisocoria. He was taken immediately to the operating room, and had two skull burr holes, releasing ‘crankcase oil-coloured’ fluid under low pressure. He improved immediately after surgery. However, when walking with the physiotherapist, he developed impaired arousal that resolved when he lay flat. We tested his mental status in both the Trendelenburg posture and upright posture (see figure 2 and online supplementary video S1); in just 5 min, his consciousness changed from full alertness to coma. A contrastenhanced MR myelogram (T1-weighted) showed a longitudinally extensive epidural collection of contrast-enhancing cerebrospinal fluid (CSF) in the thoracic and lumbar spine. There were two CSF leaks along the right-sided nerve roots at L2 (figure 3) and T6. He underwent an epidural blood patch, and within 24 h, his conscious level improved dramatically. After a week, he could go home. Repeat MR imaging at 6 months showed no
subdural collections or features of intracranial hypotension, and his midbrain morphology was completely normal. DISCUSSION In 1938, Schaltenbrand1 described ‘aliquorrhoea’, a syndrome of postural headache, meningism, nausea, vomiting, tinnitus and vertigo in patients with a low CSF pressure. Initially, the syndrome was thought to be caused by reduced fluid production in the intraventricular choroid plexus. However, more recent neuroimaging studies have shown CSF leakage to be the cause.2 3 Although spontaneous intracranial hypotension can present in many ways, they all share the criterion of orthostatic headache. Other rarer features include galactorrhoea, syringomyelia, seizures, parkinsonism and coma.4–7 This broad spectrum of presentations may not meet the 2004 International Headache Society diagnostic criteria for spontaneous intracranial hypotension, explaining why it is often misdiagnosed.8 Recently, Schievink et al9 proposed diagnostic criteria for spontaneous intracranial hypotension reflecting these variable manifestations, making them more useful in practice. Among these criteria are the characteristic MR imaging features, including pachymeningeal enhancement, subdural fluid collections, venous engorgement with dural sinus dilatation, pituitary gland enlargement and brain sagging.10 Most of these MR imaging features probably result from venous dilatation compensating for a reduced CSF pressure. This assumption is based on the Monro– Kellie hypothesis: in an incompressible compartment, as is the cranium, its constituents (blood, CSF and brain tissue) form a volume equilibrium. Thus, when one constituent decreases, there must be an
Lagrand TJ, Beukers R. Pract Neurol 2015;15:471–473. doi:10.1136/practneurol-2015-001183
471
Downloaded from http://pn.bmj.com/ on October 8, 2017 - Published by group.bmj.com
A DIFFICULT CASE
Figure 1 MR scan of brain (mid-sagittal T1-weighted) showing midbrain swelling (black arrow) and severe brain sagging (white arrows).
increase in one of the other, to maintain homeostasis. According to this concept, CSF leakage may cause venous engorgement, extravasation of fluid from the dural vasculature into the innermost dural border cell (causing pachymeningeal enhancement on MRI), and in the worst cases, there is rupture of bridging veins, causing subdural haematomas. Brain sagging results when this compensation mechanism fails, and CSF cannot provide protective buoyancy any more. The downward displacement of the brain through the tentorial notch causes traction on pain-sensitive structures; also, brainstem compression may cause cranial nerve palsies and even depression of vital centres.11 12 Savoiardo et al described a few cases of brain sagging, with swelling of diencephalic–mesencephalic structures and increased diffusivity on MRI. They hypothesised that impaired venous drainage caused venous stagnation and, hence, vasogenic oedema. Thus, in applying the Monro–Kellie doctrine, the volume of the brain is not constant.13 In spontaneous intracranial hypotension, lumbar puncture may show a low opening pressure, often below 60 mm H2O. Sometimes, there is a ‘sucking noise’ after the stylet is withdrawn as air enters the subarachnoid space.14 CT and MR myelography after intrathecal contrast injection can detect CSF leaks, and offer the additional possibility to inject an epidural blood patch.15 The first treatment for spontaneous intracranial hypotension is for the patient to lie supine, relieving traction on the pain-sensitive structures. This typically resolves the headache and reduces CSF pressure in the
472
Figure 2 A still from the online supplementary video, neurologic examination in horizontal and after 5 minutes in upright position.
thecal sac, allowing meningeal defects to restore. Sufficient hydration, increased salt intake, caffeine and theophylline may be effective, whereas corticosteroids are of questionable value.16 17 Although over half of the patients recover with conservative management, those with persisting symptoms may be helped by an epidural blood patch, especially if there is a detected CSF leak. Injection of autologous blood replaces CSF volume and seals leaks in the meninges.18 Patients with persisting symptoms can have a repeat blood patch, or may be helped by percutaneous placement of a fibrin sealant.19 When all other treatments fail, surgical intervention must be considered. This option requires the demonstration of an exact site of CSF leakage; however, it may be effective with an almost 100% reduction of headache.20 Our case shows the variety of symptoms associated with spontaneous intracranial hypotension. We were initially misled by his head trauma in the week before his presentation. When he deteriorated, we assumed this was caused by his subdural haematomas; however, in retrospect, the initial MR scan showed all the characteristic features of spontaneous intracranial hypotension as well as mesencephalic swelling. The perioperative low CSF pressure and the persisting orthostatic symptoms suggested spontaneous intracranial hypotension, further corroborated by the MR myelogram showing two CSF leaks.
Lagrand TJ, Beukers R. Pract Neurol 2015;15:471–473. doi:10.1136/practneurol-2015-001183
Downloaded from http://pn.bmj.com/ on October 8, 2017 - Published by group.bmj.com
A DIFFICULT CASE
Figure 3 MR scan of lumbar spine (coronal and axial T1 contrast-enhanced) showing cerebrospinal fluid leakage along the right L2 nerve root (white arrows).
Key points ▸ Characteristic MRI features of intracranial hypotension include pachymeningeal enhancement, subdural fluid collections, venous engorgement with dural sinus dilatation, pituitary gland enlargement and brain sagging. ▸ Clinicians should suspect spontaneous intracranial hypotension in patients with bilateral subdural haematomas and orthostatic headaches. Contributors TJL wrote the first draft and prepared the manuscript. RB participated in the manuscript preparation and revisions, and helped to bring the manuscript to its final version. Competing interests None declared. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Mark Manford, Cambridge, UK.
REFERENCES 1 Schaltenbrand VG. Neuere anschauungen zur pathophysiologie der liquorzirkulation. Zentralbl Neurochir 1938;3:290–9. 2 Bell W, Joynt R, Sahs A. Low spinal fluid pressure syndromes. Neurology 1960;10:512–21. 3 Mokri B, Posner JB. Spontaneous intracranial hypotension. Neurology 2000;55:1771–2. 4 Rahman M, Bidari SS, Quisling RG, et al. Spontaneous intracranial hypotension: dilemmas in diagnosis. Neurosurgery 2011;69:4–14. 5 Sharma P, Sharma A, Chacko AG. Syringomyelia in spontaneous intracranial hypotension. J Neurosurg 2001;95:905–8. 6 Hong M, Shah GV, Adams KM, et al. Spontaneous intracranial hypotension causing reversible frontotemporal dementia. Neurology 2002;58:1285–7.
7 Evan RW, Mokri B. Spontaneous intracranial hypotension resulting in coma. Headache 2002;42:159–60. 8 Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders: 2nd edition. Cephalgia 2004;24(Suppl 1):9–160. 9 Schievink WI, Dodick DW, Mokri B, et al. Diagnostic criteria for headache due to spontaneous intracranial hypotension: a perspective. Headache 2011;51:1442–4. 10 Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 2006;295:2286–96. 11 Pannullo SC, Reich JB, Krol G, et al. MRI changes in intracranial hypotension. Neurology 1993;43:919–26. 12 Paldino M, Mogilner AY, Tenner MS. Intracranial hypotension syndrome: a comprehensive review. Neurosurg Focus 2003;15: ECP2. 13 Savoiardo M, Minati L, Farina L, et al. Spontaneous intracranial hypotension with deep brain swelling. Brain 2007;130:1884–93. 14 Schievink WI, Reimer R, Folger WN. Surgical treatment of spontaneous intracranial hypotension associated with a spinal arachnoid diverticulum. J Neurosurg 1994;80:736–9. 15 Albes G, Weng H, Horvath D, et al. Detection and treatment of spinal CSF leaks in idiopathic intracranial hypotension. Neuradiology 2012;54:1367–73. 16 Marcelis J, Silberstein SD. Spontaneous low cerebrospinal fluid pressure headache. Headache 1990;30:192–6. 17 Mokri B, Piepgras DG, Miller GM. Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc 1997;72:400–13. 18 Sencakova D, Mokri B, McClelland RL. The efficacy of epidural blood path in spontaneous CSF leaks. Neurology 2001;57:1921–3. 19 Mehta B, Tarshis J. Repeated large-volume epidural blood patches for the treatment of spontaneous intracranial hypotension. Can J Anaesth 2009;56:609–13. 20 Schievink WI, Morreale V, Atkinson J, et al. Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. J Neurosurg 1998;88:243–6.
Lagrand TJ, Beukers R. Pract Neurol 2015;15:471–473. doi:10.1136/practneurol-2015-001183
473
Downloaded from http://pn.bmj.com/ on October 8, 2017 - Published by group.bmj.com
Sagging brain causing postural loss of consciousness: a case of severe spontaneous intracranial hypotension Tjerk Joppe Lagrand and Richard Beukers Pract Neurol 2015 15: 471-473 originally published online August 5, 2015
doi: 10.1136/practneurol-2015-001183 Updated information and services can be found at: http://pn.bmj.com/content/15/6/471
These include:
Supplementary Supplementary material can be found at: Material http://pn.bmj.com/content/suppl/2015/08/05/practneurol-2015-001183 .DC1
References Email alerting service
Topic Collections
This article cites 20 articles, 0 of which you can access for free at: http://pn.bmj.com/content/15/6/471#BIBL Receive free email alerts when new articles cite this article. Sign up in the box at the top right corner of the online article.
Articles on similar topics can be found in the following collections Ophthalmology (130)
Notes
To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/
