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Orthostatic headache with or without associated symptoms (neck or intrascapular pain, nausea and vomiting, change in hearing, diplopia, visual blurring, bitemporal hemianopsia, upper limb paresthesias, parkinsonism,[1] stupor, and coma[2]) is indicative of intracranial hypotension that can occur either after active cerebrospinal fluid (CSF) removal (eg, after a lumbar puncture) or spontaneously (spontaneous intracranial hypotension [SIH]) as a result of a spinal meningeal CSF leak.[3, 4] Spontaneous CSF leaks are attributed to the underlying fragility of the spinal meninges (sometimes associated with connective-tissue disorders) that easily tear when exposed to a mechanical factor, such as a minor trauma.[3] A trivial trauma such as coughing, pulling, pushing, and lifting is reported in a minority of the patients.[3] Diagnosis is based on clinical presentation and typical brain magnetic resonance imaging (MRI): thickening of the dura with diffuse pachymeningeal enhancement, sometimes brain sagging, subdural fluid collections, dilatation of the venous compartment with dural sinuses, and pituitary gland enlargement. When these aspects are present, it is not necessary to measure the CSF opening pressure.[4] The first-line treatment of this disorder is conservative, including bed rest, oral hydration, analgesics, nonsteroidal anti-inflammatory drugs, and caffeine or theophylline intake.[3] Spinal MRI, computed tomography or MRI myelography and radionuclide cisternography should be used to identify the site of the CSF leak[3] if conservative treatment fails. Treatment is usually conservative, but autologous epidural blood patch (EBP) has emerged as the most important nonsurgical management.[5] Some resistant cases underwent percutaneous injection of fibrin glue[6] and surgical repair of the dural tear is reserved for refractory cases when the site of the CSF leak is located.[3]

From among 214 patients referred to one of us (E.F.) over a 21-year period between April 1992 and May 2013, for evaluation of orthostatic headache (OH) and suspected SIH, 10 patients with negative head and spinal MRI and normal CSF opening pressure (CSF-OP) were identified. Nine patients were women. Mean age at the time of evaluation was 37 years (range 16-65). All patients also had anxiety-depressive disorder (mild grade in 7 patients and moderate grade in 3 patients), one of them was also suffering from conversion disorder, another from pseudoseizures, and one from mild hyperlaxity joints. Median duration of orthostatic headache prior to evaluation at our institution was 9.5 months (range 3-36). Cochleovestibular symptoms were present in 4 patients. Eight patients performed the lumbar puncture in sideways (mean CSF-OP was 140.2 mmH2O [range 80-240]), while 2 in a sitting position (mean CSF-OP was 490 mmH2O [range 440-540]). On the top of best psychiatric treatment, 9 patients performed EBP in Trendelenburg position[2] ex juvantibus criterium. One patient was treated with bed rest and overhydration for a short time. After mean follow-up of 21.6 months (range 6-74), 3 patient experienced a complete recovery, and 3 patients improved after EBP; the one treated with only conservative therapy improved with a low dose of aripiprazole (1 mg/day). Three patients with moderate psychiatric disorder had persistent OH. A small series of 6 similar patients has been published,[7] in which 5 patients remained severely symptomatic and work disabled at an average follow-up of 4 years. The most likely explanation for these cases is the existence of an intermittent or very slow flow leak that would evade identification by existing imagining techniques. Alternative etiological hypotheses are of increased compliance of the lower spinal CSF space shifting the hydrostatic indifferent point downward in the orthostatic position (inducing compensatory dilation of pain-sensitive intracranial venous structures without changing CSF pressure at the lumbar level[8] or of orthostatic CSF leakage to the epidural venous network.[9] In this small series, it is not described whether or not the patients had psychiatric disorders in their medical history. A similar case has been described[10] in which a progressive improvement occurred simultaneously to the introduction of vitamin A supplementation. A beneficial effect of vitamin A supplementation was speculated from this report. This patient was also suffering from chronic fatigue treated with bupropion 150 mg qd, amitriptyline 35 mg qd. Orthostatic headache can occur without evidence of intracranial hypotension or detectable CSF leak despite extensive diagnostic testing. Clinical features alone are unlikely to differentiate between orthostatic headache with and without identifiable CSF leak.

We think that in our series, as also in the case report (the patient was taking antidepressant drugs), the underlying psychiatric disorder was the major cause of orthostatic headache that might be considered as a new type of headache attributed to psychiatric disorder. Further studies are needed to confirm these data.

Acknowledgments

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We acknowledge Dr. Daria Roccatagliata for having kindly reviewed the manuscript.

References

  1. Top of page
  2. Acknowledgments
  3. References