Spinal MRI findings in Spontaneous Intracranial Hypotension - Case Report

Transcription

1 Spinal MRI findings in Spontaneous Intracranial Hypotension - Case Report Poster No.: P-0105 Congress: ESSR 2014 Type: Scientific Poster Authors: M. Tzalonikou, D. KECHAGIAS, A. Plomaritoglou, A Agathonikou, N. Kritikos, A. Roussakis ; Athens/GR, 2 MAROUSI/GR Keywords: Cerebrospinal fluid, Education, MR, Musculoskeletal spine, CNS DOI: /essr2014/P-0105 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 39

2 Purpose Spontaneous intracranial hypotension (SIH) is a rather uncommon condition characterized by low CSF pressure in the intracranial cavity. The syndrome was originally (1,2) described by George Schaltenbrand in Its clinical presentation is with persistent postural-dependent headaches on a daily basis which resolve with recumbency. Patients with SIH may also present with nausea, vomiting, blurred vision or diplopia, photophobia or even tinnitus and vertigo. SIH typically has been described in young or middle(3-5) aged patients. No history of previous dural puncture or trauma is usually reported. Treatment options are conservative with bed rest and -if that fails- epidural blood (1,4,6-7) patch. Imaging studies, especially magnetic resonance imaging (MRI) have proven useful in making the diagnosis and ruling out other disorders. We report three cases of patients who presented with orthostatic headache and were diagnosed with SIH; spinal neuroimaging findings and review of the literature is presented. Page 2 of 39

3 Methods and Materials CASE 1: A 32 year old male patient visited his family doctor complaining vaguely about headaches and dizziness. The patient described that occasionally he would feel nauseated but he did not report vomiting. Physical exam was normal and the patient was sent home. Two weeks later and since his symptoms were not relieved, the patient decided to visit the local hospital and ask for an expert's consultation. Detailed history revieled that the symptoms aggravated during day time and while standing and were relieved in the evening with rest in the supine position. SIH was suspected and the patient was referred to the radiology department for imaging. MRI of the brain perfomed showed minimal meningeal enhancement and no other additional findings. MRI of the cervical spine showed very mild ventral dural enhancement (Fig. 1-5). Conservative treatment was recommended. The patient was free of symptoms about two months later and is still free of symptoms two years later. CASE 2: A 27 year old male patient visited a public hospital emergency department complaining about strong diffuse headache related to the standing and the sitting position. Neurologic examination showed a positive Romberg sign. The remainder of the physical exam was within normal. The patient refused lumbar puncture, so he was referred to the radiology department for an MRI of the brain and of the cervical spine. Contrast enhanced images showed mild pachymeningeal contrast enhancement in the endocranial cavity and mild ventral dural contrast enhancement in the upper cervical spine. Additional MRI scan of the thoracic spine showed fluid collection in the posterior epidural space. MRI of the lumbar spine was unremarkable. Based upon physical exam and the MRI findings, the diagnosis of SIH was suggested. The patient received conservative treatment and was relieved of symptoms. No recurrence was mentioned (Fig. 6-12). CASE 3: A 33-year old previously healthy female presented complaining about persistent headaches present on a daily basis for a period of time of about a month and a half. The patient described that she would feel her head 'heavy' shortly after coming out of bed and that the headache would become stronger during day time. Analgesic treatment did not prove helpful. The patient was relieved in the night and with bed-rest. No other symptoms were described. Physical exam was normal. Clinical suspicion of SIH was raised. A brain and a total spine MRI (cervical-thoracic-lumbar spine) as well as MR myelography were performed. MRI of the brain revealed intense pachymeningeal contrast enhancement. MRI of the cervical and the thoracic spine also showed meningeal enhancement. No definate CSF leak site was detected (Fig.13-25). The patient was treated concervatively with bed rest for 30 days. Page 3 of 39

4 Follow-up brain and spine MRI about two months after treatment were read as normal. Page 4 of 39

5 Images for this section: Page 5 of 39

6 Page 6 of 39

7 Fig. 1: Sag. T1 TSE. Cervical spine, midline section. No obvious findings. CT-MRI Dpt, Hygeia Hospital Athens, Greece. Page 7 of 39

8 Page 8 of 39

9 Fig. 2: Sag. T2 TSE. Cervical spine, midline section. No obvious abnormality. Page 9 of 39

10 Page 10 of 39

11 Fig. 3: Sag. contrast enhanced T1 TSE. Cervical spine, midline section. Mild ventral dural contral enhancement. Page 11 of 39

12 Fig. 4: Ax. T1 TSE. Cervical spine. Prominent epidural venous plexus. Page 12 of 39

13 Fig. 5: Ax. contrast enhanced T1 TSE. Cervical spine. Prominent epidural venous plexusmild festoned appearance.. Page 13 of 39

14 Fig. 12: Sag. contrast enhanced T1 TSE. MRI of the Lumbar Spine. Page 14 of 39

15 Fig. 6: Ax. contrast enhanced T1 TSE. MRI of the brain. Mild dural contrast enhancement. Page 15 of 39

16 Fig. 7: Sag. contrast enhancenced T1 TSE. MRI of the brain. Mild ventral dural contral enhancement in the upper cervical spine. Page 16 of 39

17 Fig. 8: Cor. contrast enhanced T1 TSE. MRi of the brain.very mild contrast enhancement in the upper cervicla spine. Page 17 of 39

18 Fig. 9: Ax. T2 TSE. MRI of the Thoracic Spine. Mild fluid collection in the posterior epidural space. Page 18 of 39

19 Fig. 10: Ax. T2 TSE. MRI of the Thoracic Spine. Fluid collection prominent in the posterior epidural space. Page 19 of 39

20 Fig. 11: Sag. contrast enhanced T1 TSE. MRI of the Thoracic Spine. Page 20 of 39

21 Fig. 13: Ax. Flair. MRI of the Brain. Subdural fluid collections. Page 21 of 39

22 Fig. 14: Ax. T1 TSE. MRI of the Brain. Pachymeningeal thickening. Page 22 of 39

23 Fig. 15: AX. contrast enhanced T1 TSE. MRI of the Brain. Diffuse pachymeningeal enhancement. Page 23 of 39

24 Fig. 16: Sag. T2 TSE. MRI of the Cervical Spine. Fluid collection in the ventral anterior epidural space. Notice of very mild downward displacement of the cerebral tonsils. Page 24 of 39

25 Fig. 17: Sag. contrast enhanced T1 TSE. MRI of the Cervical Spine. Diffuse dural enhancement. Notice of pituitary hyperemia is made. Page 25 of 39

26 Fig. 18: Ax. contrast enhanced T1 TSE. MRI of the Cervical Spine. Diffuse dural enhancement. Page 26 of 39

27 Fig. 19: Ax. contrast enhanced T1 fs. MRI of the Cervical Spine. Diffuse dural enhancement extending to the neural foramina. Page 27 of 39

28 Fig. 20: Ax. contrast enhanced T1 fs. MRI of the Cervical Spine. Diffuse dural enhancement extending to the neural foramina. Page 28 of 39

29 Fig. 21: Ax. contrast enhanced T1 fs. MRI of the Thoracic Spine. Diffuse dural enhancement. Page 29 of 39

30 Fig. 22: Ax. contrast enhanced T1 fs. MRI of the Lumbar Spine. Diffuse dural enhancement. Page 30 of 39

31 Fig. 23: Sag. contrast enhanced T1 TSE. MRI of the Thoracic Spine. Dural enhancement. Page 31 of 39

32 Fig. 24: Sag. contrast enhanced T1 fs. MRI of the Lumbar Spine. Page 32 of 39

33 Fig. 25: MR Myelogram. Thoracolumbar Spine. The exam was unremarkable but for a small cystic lesion in the soft tissues adjacent to the neckof a lower right rib. Page 33 of 39

34 Results Spontaneous intracranial hypotension is characterized by reduced CSF pressure, occurs without previous dural puncture or other previous inerventional procedure, surgery or trauma of the neural axis and is thought to be due to developmental CSF leak through small defects in the meninges in the spinal canal which result in decrease in CSF volume and CSF pressure. In a few cases, minor trauma may be reported. The most characteristic symptom of SIH is headache (frontal, vertex, occipital or of the whole head) aggravated by sitting or standing and relieved by recumbancy. Headache was the leading symptom in all our three patients. Other less commonly experienced symptoms include stiff neck, nausea, dizziness, vertigo, visual disturbances and deafness (1-4, ). CSF may be normal or with elevated protein content, xanthochromatic or with lymphocytic pleocytosis. CSF opening pressure on lumbar puncture in SIH is usually less than 60 (4,5 ) mm H20. Treatment options include conservative treatment with bed rest, analgesics and sedatives, fluid restoration with intake of oral fluids or intravenous hydration or epidural blood patch or epidural saline infusion. There is spontaneous resolution of SIH symptomatology over a period of weeks to months in most patients (1, 6-7 ). The most characteristic intracranial imaging finding of SIH is diffuse symmetrical bilateral dural thickening and intense pachymeningeal enhancement on contrast enhanced images. Other intracranial MR imaging findings assosiated with this entity are subdural fluid collections, slitlike ventricles, small sylvian fissures and basal cisterns, downward displacement of the cereberal tonsils, pituitary hyperemia and venous and dural sinus engorgement (8-11). Spinal imaging findings associated with spontaneous intracranial hypotension include: 1. Epidural fluid collections: this is the most frequent sign with a reported frequency varying from 67 up to 100% in different series. The collections tend to extend in more than 5 spinal segments. In one of our patients an anterior epidural space fluid collection was seen in the cervical spine and in a second patient epidural fluid collection was met in teh posterior epidural space in the thracic spine. Axial images are useful in the identification of epidural fluid collections because they allow visualization of the dura. Epidural fluid collections tend to be isointense to CSF on T1 and T2 weighted images. The use of both T1 and T2 images with and without fat saturation proves useful for the identification of the collection. These collections may or may not be symtomatic. Page 34 of 39

35 2. Dural enhancement: although the exact cause of dural enhancement is not known, it could be related either to dural vasodilatation and venous engorgement or to delayed CSF leak into the collection. It is associated with intracranial meningeal enhancement. Dural enhancement varying from mild to pronounced was met to all our three patients. 3. Dilated epidural veins: they have been described in both spontaneous intracranial hypotension as well as following lumbar puncture. 4. Dilatation of the anterior and/or of the posterior internal vertebral venous plexus: morphologic and/or size characteristics are used in the evaluation of the venous plexous. Chiapparini has described the 'festooned' or 'light bulb' appearance of the epidural venous pplexus in cases of SIH. 4. Abnormalities around the nerve root sleeve: they may point out to a possible CSF leakage site. We thought we have seen very mild signal intensity alterations in the paravertebral soft tissues at the level of the thoracic spine in our third patient. 5. C1-C2 sign: or false localising sign. It has been described by Schievink et al. in This refers to fluid signal intensity in the soft tissues between the C1-C2 spinous processes. This area should not be thought to indicate CSF leakage site. (10-17) Page 35 of 39

36 Conclusion MR imaging for the evaluation of headache continues to grow at an ever increasing rate. The recognition of the classic and less common findings of intracranial hypotension are important as the diagnosis is often not considered, leading to further workup and possibly more, unnecessary, invasive, and ineffective therapies. Page 36 of 39

37 References 1.Mokri B, Piepgras DG, Miller GM. Syndrome of orthostatic headache and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc 1997;72: Schalltenbrand G. Neuere anschauungen zur pathophysiologie der liquozirkulation. Zentralbl Neurochir 1938;3: Bell WE, Joynt RJ, Sahs AL. Low spinal fluid pressure syndromes. Neurology 1960;10: Schievink WI. Misdiagnosis of spontaneous intracranial hypotension. Arch Neurol 2003;60: Marcelis J, Silberstein SD. Spontaneous low cerebrospinal fluid pressure headache. Headache 1990;30: Turgut N, Unlü E, Hamamcio#glu MK, Güldiken B, Albayram S. Postural tremor as a manifestation of spontaneous intracranial hypotension. J Clin Neurosci 2010; 17: Rando TA, Fishman RA: Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology 42: , Wang SC, Lirng JF, Hseu SS, Chan KH. Spontaneous intracranial hypotension treated by epidural blood patches. Acta Anaesthesiol Taiwan 2008;46: Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 2006;295: Schievink WI. Spontaneous spinal cerebrospinal fluid leaks: a review. Neurosurg Focus 2000;9: Mokri B, Krueger BR, Miller GM, Piepgras DG. Rochester MN. Meningeal gadolinium enhancement in low pressure headache. Ann Neurol 1991;30: Chen HH, Huang CI, Hseu SS, Lirng JF. Bilateral subdural hematomas caused by spontaneous intracranial hypotension. J Chin Med Assoc 2008;71: Berroir S, Grabli D, Héran F, Bakouche P, Bousser MG. Cerebral sinus venous thrombosis in two patients with spontaneous intracranial hypotension. Cerebrovasc Dis 2004;17: Mokri B, Posner JB. Spontaneous intracranial hypotension: the broadening clinical and imaging spectrum of CSF leaks. Neurology 2000;55: Page 37 of 39

38 12.Metafratzi Z, Argyropoulou MI, Mokou-Kanta C, et al. Spontaneous intracranial hypotension: morphological findings and CSF flow dynamics studied by MRI. Eur Radiol 2004; 14: Messori A, Salvolini U. The spectrum of spinalmrfindings in SIH. Eur Radiol 2002; 14. Schoffer KL, Benstead TJ, Grant I. Spontaneous intracranial hypotension in the absence of magnetic resonance imaging abnormalities. Can J Neurol Sci. 2002;29: Chiapparini L, Farina L, D'Incerti L, et al. Spinal radiological findings in nine patients with spontaneous intracranial hypotension. Neuroradiology 2002;44: , discussion Messori A, Polonara G, Salvolini U. Dilation of cervical epidural veins in intracranial hypotension. AJNR Am J Neuroradiol 2001;22: Schievink WI, Maya MM, Tourje J. False localizing sign of C1/2 cerebrospinal fluid leak in spontaneous intracranial hypotension. J Neurosurg 2004; 100: Morgan JT, Scumpia AJ, Johnson AA, et al. Case report: spontaneous intracranial hypotension in association with the presence of a false localizing C1-2 CSF leak. Surg Neurol 2008; 70: Page 38 of 39

39 Personal Information Maria Tzalonikou: Radiologist, CT-MRI Dpts, Hygeia-Mitera Hospitals, Athens, Greece, D. Kechagias: Radiologist, CT-MRI Dpts, Hygeia-Mitera Hospitals, Athens, Greece, A. Plomaritoglou: neurologist, Hygeia-Mitera Hospitals, Athens, Greece, A. Agathonikou: neorologist, K.A.T. Hospital, Athens, Greece, N. Kritikos: Radiologist, CT-MRI Dpts, Hygeia-Mitera Hospitals, Athens, Greece, A. Roussakis: Radiologist, CT-MRI Dpts, Hygeia-Mitera Hospitals, Athens, Greece, Head of the Dpt. Page 39 of 39