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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 21  |  Issue : 2  |  Page : 186-188

Cerebrospinal fluid leak causing spontaneous intracranial hypotension


Department of Anaesthesiology, 165 Military Hospital, Rangapahar, Nagaland, India

Date of Submission21-Jul-2018
Date of Acceptance01-Jun-2019
Date of Web Publication07-Oct-2019

Correspondence Address:
Lt Col (Dr) Nihar Ameta
Department of Anaesthesiology, 165 Military Hospital, C/o 99 APO, Rangapahar, Nagaland
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jmms.jmms_46_18

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  Abstract 


Spontaneous intracranial hypotension (SIH) is one of the important causes of headache in young and middle-aged and is commonly misdiagnosed in the initial stages. SIH generally results from a spontaneous cerebrospinal fluid (CSF) leak usually in the spinal region subsequently leading to headache, usually postural in nature. SIH is characterized by a triad of postural headache, diffuse pachymeningeal enhancement diagnosed on magnetic resonance imaging, and low CSF opening pressure of <60 mm of water. Autologous epidural blood patch (EPB) is the treatment of choice in these patients. Here, we present a case of SIH with classical clinical and radiological findings managed appropriately with EBP, leading to complete resolution of the symptoms.

Keywords: Epidural blood patch, spontaneous cerebrospinal fluid leak, spontaneous intracranial hypotension


How to cite this article:
Mateen M A, Ameta N, Ghosh J. Cerebrospinal fluid leak causing spontaneous intracranial hypotension. J Mar Med Soc 2019;21:186-8

How to cite this URL:
Mateen M A, Ameta N, Ghosh J. Cerebrospinal fluid leak causing spontaneous intracranial hypotension. J Mar Med Soc [serial online] 2019 [cited 2019 Oct 17];21:186-8. Available from: http://www.marinemedicalsociety.in/text.asp?2019/21/2/186/268625




  Introduction Top


Although spontaneous intracranial hypotension (SIH) is rare, it has been increasingly recognized in recent years. SIH is characterized by the diagnostic triad of postural headache, magnetic resonance imaging (MRI) finding of diffuse pachymeningeal enhancement, and low cerebrospinal fluid (CSF) opening pressure of <60 mm of water.[1]

SIH may have an insidious onset leading to a delay in the diagnosis. Findings on imaging may be nonspecific, and because of insidious onset and nonspecific history, the diagnosis of SIH may be missed. Autologous epidural blood patch (EBP) is the treatment of choice in these patients.[2]

We present a case of SIH who presented with classical clinical features and radiological findings. He was appropriately managed with EBPs, in two sittings, at different levels, leading to the complete resolution of his symptoms.


  Case Report Top


A 30-year-old male patient presented to us with complaints of headache for 4 weeks. The headache was insidious in onset, generalized, heavy in character, got worsened on attaining sitting or standing posture, and got relieved within minutes on lying down. The patient also complained of double vision for the past 2 weeks.

The history was negative for fever, vomiting, altered sensorium, and loss of consciousness or seizures.

The patient is a professional boxer and had a match 2 weeks preceding the onset of the symptoms.

On examination, the patient was alert, conscious, and afebrile, and all vital parameters were normal. Central nervous system examination was suggestive of left-sided lateral rectus palsy and no other focal neurological deficits. The fundal examination was normal.

The patient was evaluated with MRI brain with gadolinium contrast, suggesting features of SIH (diffuse pachymeningeal thickening and enhancement, bilateral frontal subdural collections, and engorged venous sinuses).

Further evaluation with noncontrast MR myelography confirmed the findings and revealed expansion of CSF space around the nerve root sleeves, most significant at C2–C3 level. Other sites of CSF leak were T9–T12, L1–L4.

The patient was initially managed with conservative measures in the form of liberal oral fluids and analgesics with no relief. Subsequently, an EBP was performed at lumbar level (L3–L4), leading to immediate symptomatic relief in headache. However, since the LR palsy was still persisting, the patient was given second EBP in the cervical spine region after 1 week, which lead to complete resolution of all the symptoms.


  Discussion Top


SIH is one of the important causes of headache in young and middle-aged and is commonly misdiagnosed in the initial stages. It generally results from a spontaneous CSF leak usually in the region of the thoracic spine and cervicothoracic junction. The prevalence has been estimated to be 1/50,000/year, with a peak around 40 years of age. It is twice as common in women.[3]

The cause of spontaneous CSF leak remains unknown. The structural weakness of the spinal meninges may lead to it. Trivial trauma preceding the onset of symptoms may suggest the role of mechanical factors. Our patient had a bout of boxing preceding the illness which may have contributed to the development of SIH. The dural weakness may predispose the formation of a defect through which the CSF leaks into the epidural space.

Generalized connective tissue disorders may have a role in spontaneous CSF leaks. These disorders may affect different components of the dural extracellular matrix.[4] Approximately one-fifth of patients with SIH may have skeletal manifestation of Marfan Syndrome,[5],[6] our patient had none.

A fall in CSF volume and not CSF pressure may cause SIH. Although loss of CSF volume occurs with CSF rhinorrhea and otorrhea, clinical and radiological features of SIH are rarely seen in these cases.[7] A patient of SIH typically presents with postural headache occurring immediately on assuming upright posture and resolves usually within a minute of lying down horizontally. However, in some cases, it may show a delayed response to change in posture. Postural headache sometimes occurs subsequent to spinal anesthesia or lumbar puncture (postdural puncture headache); however, in SIH, the headache may not be as dramatic or immediate.[8]

The headache may be diffuse or localized usually in the occipital region. The headache is due to the downward displacement of the brain due to loss of CSF, leading to traction on pain-sensitive structures, dura in particular. Neck pain and stiffness, nausea, and vomiting are other common symptoms. The downward displacement of the brain may lead to stretch on the cranial nerves. This probably explains symptoms of visual field defects, diplopia (abducens or rarely trochlear or oculomotor nerves), facial nerve symptoms, and hearing defects.

Cranial MRI with gadolinium and computed tomography (CT) myelography are the initial studies of choice in diagnosing SIH. Pachymeningeal enhancement is the most specific imaging abnormality on MRI. However, some patients with SIH may have normal MRI scans.[9]

Radionuclide cisternography may be used when the diagnosis is doubtful and myelography results are normal. With availability of MRI, CSF studies, including measurement of CSF pressures, are rarely required and may be performed in symptomatic patients with inconclusive MRI findings. We did not perform a CSF study on our patient as violation of the dura could have worsened the symptoms.

Various treatment options are available for SIH. Initial management is conservative with bed rest, adequate hydration, theophylline, caffeine, and steroids, but none is of proven efficacy.

EBP is the definitive treatment in patients who do not respond to conservative measures. The success rate of EBP is as high as up to 90%.[10] Ten to twenty milliliters of autologous blood is injected into the lumbar epidural space (usually under CT guidance), following which the patient is placed in the Trendelenburg position for approximately 2 h.[10] The blood ascends over several segments to seal the leak in the dura. EBP leads to volume replacement resulting from dural tamponade and may later also seal the dural rent. If the initial EBP is unsuccessful, it can be repeated using a larger volume of blood (20–100 mL) and a CT-guided directed EBP at the site of the leak, or percutaneous placement of fibrin sealant is recommended.

In patients who fail two to three EBPs, and where a focal CSF leak is identifiable, surgical repair of the defect may be considered. Ligation of the defect, a Gelfoam, or fibrin glue may be used to seal the CSF leak.[11],[12] Recently, greater occipital nerve block with corticosteroids and pulsed radiofrequency have also been attempted as treatment modalities.[13]

Recurrence of spinal CSF leaks may be seen in about 10% of patients;[3] and hence, our patient is on follow-up care.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mokri B, Krueger BR, Miller GM. Meningeal gadolinium enhancement in low pressure headache. Ann Neurol 1991;30:294-5.  Back to cited text no. 1
    
2.
Park ES, Kim E. Spontaneous intracranial hypotension: Clinical presentation, imaging features and treatment. J Korean Neurosurg Soc 2009;45:1-4.  Back to cited text no. 2
    
3.
Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 2006;295:2286-96.  Back to cited text no. 3
    
4.
Mokri B, Maher CO, Sencakova D. Spontaneous CSF leaks: Underlying disorder of connective tissue. Neurology 2002;58:814-6.  Back to cited text no. 4
    
5.
Davenport RJ, Chataway SJ, Warlow CP. Spontaneous intracranial hypotension from a CSF leak in a patient with Marfan's syndrome. J Neurol Neurosurg Psychiatry 1995;59:516-9.  Back to cited text no. 5
    
6.
Abu Libdeh A, Matsumoto JA, Dhamija R. Teaching neuroImages: Intracranial hypotension in a patient with Marfan syndrome. Neurology 2016;87:e40-1.  Back to cited text no. 6
    
7.
Mokri B. Spontaneous cerebrospinal fluid leaks: From intracranial hypotension to cerebrospinal fluid hypovolemia – Evolution of a concept. Mayo Clin Proc 1999;74:1113-23.  Back to cited text no. 7
    
8.
Headache Classification Committee of the International Headache Society (IHS). The international classification of headache disorders, 3rd ed.ition (beta version). Cephalalgia 2013;33:629-808.  Back to cited text no. 8
    
9.
Schoffer KL, Benstead TJ, Grant I. Spontaneous intracranial hypotension in the absence of magnetic resonance imaging abnormalities. Can J Neurol Sci 2002;29:253-7.  Back to cited text no. 9
    
10.
Ferrante E, Arpino I, Citterio A, Wetzl R, Savino A. Epidural blood patch in trendelenburg position pre-medicated with acetazolamide to treat spontaneous intracranial hypotension. Eur J Neurol 2010;17:715-9.  Back to cited text no. 10
    
11.
Schievink WI, Maya MM, Moser FM. Treatment of spontaneous intracranial hypotension with percutaneous placement of a fibrin sealant. Report of four cases. J Neurosurg 2004;100:1098-100.  Back to cited text no. 11
    
12.
Davidson B, Nassiri F, Mansouri A, Badhiwala JH, Witiw CD, Shamji MF, et al. Spontaneous intracranial hypotension: A review and introduction of an algorithm for management. World Neurosurg 2017;101:343-9.  Back to cited text no. 12
    
13.
Niraj G, Critchley P, Kodivalasa M, Dorgham M. Greater occipital nerve treatment in the management of spontaneous intracranial hypotension headache: A case report. Headache 2017;57:952-5.  Back to cited text no. 13
    




 

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