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         CASE SNIPPET Year : 2023  |  Volume : 69  |  Issue : 3  |  Page : 179-181

The ebb and flow of headache: A clue to pathophysiology of sinus stenosis in idiopathic intracranial hypertension?

AV R Taallapalli, S Shivaram, M Gupta, GB Kulkarni
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Hosur Road, Bengaluru, Karnataka, India

Date of Submission07-Mar-2022Date of Decision14-May-2022Date of Acceptance25-May-2022Date of Web Publication28-Nov-2022

Correspondence Address:
Dr. G B Kulkarni
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Hosur Road, Bengaluru, Karnataka
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/jpgm.jpgm_238_22

How to cite this article:
R Taallapalli A V, Shivaram S, Gupta M, Kulkarni G B. The ebb and flow of headache: A clue to pathophysiology of sinus stenosis in idiopathic intracranial hypertension?. J Postgrad Med 2023;69:179-81
How to cite this URL:
R Taallapalli A V, Shivaram S, Gupta M, Kulkarni G B. The ebb and flow of headache: A clue to pathophysiology of sinus stenosis in idiopathic intracranial hypertension?. J Postgrad Med [serial online] 2023 [cited 2023 Jul 11];69:179-81. Available from: https://www.jpgmonline.com/text.asp?2023/69/3/179/362171

A 23-year-old lady presented with complaints of headache with episodes of blurring of vision for the past 45 days. Headache was dull aching, bi-frontal, lasting for two hours, aggravating in the supine and bending forward positions, and associated with intermittent episodes of blurring of vision in both eyes at the peak of the headache. Such episodes were occurring more than four times a day, with a score of 6-10 on the visual analog scale. Her body mass index (BMI) was 19.8 kg/m2. Visual acuity and eye movements were normal with bilateral grade four papilledema [Figure 1]. In another hospital she had been started on oral acetazolamide 250 mg, two times a day (from two weeks before admission), with which she had no relief from the headache. Hence she was referred to our center. Her routine evaluation, including hemogram, liver, and kidney function tests, erythrocyte sedimentation rate (ESR), thyroid profile, and lipid profile, was normal. Magnetic resonance imaging (MRI) brain [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d showed partial empty sella, perioptic sheath dilatation, vertically tortuous optic nerves, and right transverse (TS) and superior sagittal sinus stenosis suggestive of raised intracranial pressure (ICP). The left transverse sinus was hypoplastic and there was no evidence of cerebral venous sinus thrombosis. Her cerebrospinal fluid (CSF) opening pressure was 170 mm water, and analysis (2 ml) was normal (while she was on acetazolamide 500 mg per day). Visual field analysis (perimetry) showed an enlarged blind spot in both eyes. Based on the clinical and imaging parameters, idiopathic intracranial hypertension (IIH) was diagnosed. After the lumbar puncture, the headache pattern changed. She developed occipital headache, predominantly in the sitting position, which was relieved in the supine position. Repeat MRI brain [Figure 2]e, [Figure 2]f, [Figure 2]g, [Figure 2]h showed resolution of all raised intracranial pressure features, the reversal of stenosis, and features of intracranial hypotension appeared in the form of diffuse pachymeningeal enhancement and sagging of the brain.[1] Anti-edema measures were stopped temporarily. After three days, she developed her earlier headache, which subsided after restarting acetazolamide (500 mg three times a day). On follow-up after three months, she was asymptomatic. Follow-up MRI brain showed features of right sigmoid sinus stenosis without features of raised ICP [Figure 2]i, [Figure 2]j, [Figure 2]k, [Figure 2]l.

Figure 1: Fundus photograph at admission: (a) right eye showing grade IV papilledema; (b) left eye showing grade IV papilledema

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Figure 2: MRI brain at admission showing: (a) tapering and stenosis of right transverse and sigmoid sinuses in venogram, hypoplastic left transverse sinus; (b) patulous peri-optic sheath in axial T2 sequence; (c) empty sella in T2 sagittal image; (d) normal T1 post-contrast coronal image. MRI brain done after lumbar puncture showing: (e) normal venogram with opened right transverse and sigmoid sinuses; (f) normal optic nerves; (g) normal sella; and, (h) diffuse pachymeningeal enhancement, suggestive of intracranial hypotension. MRI brain done after three months showing: (i) mild stenosis of the right sigmoid sinus; (j) normal optic nerves; (k) normal sella, and (l) normal T1 post-contrast coronal image

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The present patient presented with clinical and MRI brain features of raised ICP with stenosis of right TS and sigmoid sinus with hypoplasia of left TS. After a lumbar puncture, headache changed into intracranial hypotension type, and repeat MRI brain showed reversal of raised ICP signs, with the opening of the right transverse sinus, suggesting that transverse sinus stenosis was probably due to the effect of raised ICP in IIH. Although only 2 ml CSF was drained, the patient developed features suggestive of decreased intracranial pressure. The possible explanation for this being a CSF leak, which is a common complication after lumbar puncture. In a study done by Wang et al.,[2] 65% of patients had evidence of CSF leak post lumbar puncture.

TS stenosis has been reported in more than 90% of IIH patients.[3] There are several conflicting studies, among which some favor the cause hypothesis, others favor the effect hypothesis, and few favor none.[3],[4],[5],[6] In 2002, King et al.[4] studied nine patients with IIH. After CSF drainage, pressure in the transverse sinus decreased and venous hypertension subsided. In 2015, Onder et al.[5] reported a young man with IIH whose stenotic sinus opened up after a lumbar puncture. Eventually he underwent a lumboperitoneal shunt (LPrS) due to IIH recurrences. In 2018, Bull et al.[6] observed that after high volume lumbar puncture (30 ml drainage) there was a transient resolution of venous sinus stenosis in a patient with IIH.

In 2011, Ahmed et al. reviewed sinus stenosis and stenting.[7] They observed two types of transverse sinus stenosis: extrinsic and intrinsic. Extrinsic stenosis has smooth, gradually narrowing tapered stenosis, which resolves after CSF drainage and it occurs secondary to raised ICP. Intrinsic stenosis has discrete obscurations and it is due to arachnoid granulations and fibrous septae. In this study, 52 patients were retrospectively studied. All were refractory to medical treatment. All underwent stenting and 49 were cured of IIH symptoms. In most patients, a collapsible transverse sinus, structurally vulnerable to extrinsic compression from intracranial hypertension, perpetuates the situation. It has been proposed that raised ICP compresses collapsible TS causing venous outflow obstruction, resulting in further venous hypertension, which then decreases CSF absorption and causes further increase in ICP. This positive feedback causes a further external compression and further stenosis of TS.

Although definitive conclusions cannot be made from a single report, we hypothesize that TS stenosis is an effect rather than a cause of IIH. The present case is unique as the same patient had raised ICP, low ICP features with corresponding MRI brain images, and resolution of TS stenosis following a lumbar puncture. The ebb and flow of headaches and the corresponding imaging findings suggest the dynamic nature of sinus stenosis in IIH.

Declaration of patient consent

The authors certify that appropriate patient consent was obtained.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 

 :: References 
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    6.Buell TJ, Raper DMS, Pomeraniec IJ, Ding D, Chen CJ, Taylor DG, et al. Transient resolution of venous sinus stenosis after high-volume lumbar puncture in a patient with idiopathic intracranial hypertension. J Neurosurg 2018;129:153-6.  
    7.Ahmed RM, Wilkinson M, Parker GD, Thurtell MJ, Macdonald J, McCluskey PJ, et al. Transverse sinus stenting for idiopathic intracranial hypertension: A review of 52 patients and of model predictions. AJNR Am J Neuroradiol 2011;32:1408-14.  
    
  [Figure 1], [Figure 2]