Paradoxical presentation of orthostatic headache associated with increased intracranial pressure in patients with cerebral venous thrombosis

Authors: Kim JB, Kwon DY, Park MH, Kim BJ, Park KW.

Headache is the most common symptom of cerebral venous thrombosis (CVT); however, the detailed underlying mechanisms and characteristics of headache in CVT have not been well described. Here, we report two cases of CVT whose primary and lasting presentation was orthostatic headache, suggestive of decreased intracranial pressure. Contrary to our expectations, the headaches were associated with elevated cerebrospinal fluid (CSF) pressure. Magnetic resonance imaging and magnetic resonance venography showed characteristic voiding defects consistent with CVT. We suggest that orthostatic headache can be developed in a condition of decreased intracranial CSF volume in both intracranial hypotensive and intracranial hypertensive states. In these cases, orthostatic headache in CVT might be caused by decreased intracranial CSF volume that leads to the inferior displacement of the brain and traction on pain-sensitive intracranial vessels, despite increased CSF pressure on measurement. CVT should be considered in the differential diagnosis when a patient complains of orthostatic headache.

Treatment of orthostatic headache without intracranial hypotension: A case report

Authors: Gil-Gouveia R.

INTRODUCTION:: Orthostatic headache is very suggestive of intracranial hypotension. It has a good prognosis as it usually responds to conservative treatment or epidural blood patches. CASE REPORT:: A 36-year-old female presented with severe and prolonged orthostatic headache starting after a seizure. No stigma of intracranial hypotension was detected on brain MRI, and intracranial pressure was within normal range. No imaging evidence of a fistula was found. She was refractory to symptomatic treatment including five epidural blood patches. Progressive improvement occurred simultaneously to the introduction of vitamin A supplementation. DISCUSSION:: A series of six similar patients is discussed, in which five patients remained severely symptomatic and workdisabled at an average follow-up of four years. It is proposed that the pathophysiological mechanism producing orthostatic headache might not be dependent on intracranial hypotension and could respond to vitamin A.

Idiopathic intracranial hypertension is a rare cause of headache in children

Authors: Viuff AC, Hansen JK, Møller HU.

Idiopathic intracranial hypertension is often believed to be an illness exclusively occurring in obese women in their twenties and thirties. This case describes a four-year-old boy presenting with headache, vomiting photophobia and double vision for six days. He did not have a fever; and all exams and tests, including a magnetic resonance imaging of the brain showed normal values. During the eye examination, he was found to have bilateral papilloedema and when undergoing lumbar puncture an elevated pressure of 230 mm water was discovered. The patient was diagnosed with idiopathic intracranial hypertension and treated with azetazolamide. Within few days, his symptoms disappeared.

High-pressure headaches: idiopathic intracranial hypertension and its mimics

Authors: Peng KP, Fuh JL, Wang SJ.

Idiopathic intracranial hypertension (IIH) is a rare disorder that typically affects obese women of childbearing age, but can also occur in paediatric populations. Patients usually present with diffuse, daily headache and visual disturbances, but either symptom can occur in isolation. Patients with IIH often have papilloedema; however, IIH without papilloedema is fairly common in patients with chronic daily headache. The pathogenesis of IIH is unknown; the high incidence of comorbid bilateral transverse sinus stenosis (BTSS) in patients with IIH suggests that the two conditions are linked, although no direct causal relationship has been established. Cerebrospinal fluid (CSF) pressure monitoring or lumbar puncture-which provides immediate symptomatic relief-are important in making a diagnosis of IIH. Current treatments for IIH include weight reduction, medical treatment, CSF diversion surgery, optic nerve sheath fenestration and, potentially, endovascular stenting (in patients with BTSS). Prevention of visual loss (which can be substantial) is the main goal of treatment. Residual headache and IIH recurrence are not uncommon after treatment, and regular follow-up is, therefore, warranted even in patients who achieve remission. This Review provides an update of current knowledge of the aetiology, pathophysiology and treatment of IIH.

Headache Due to Spontaneous Intracranial Hypotension and Subsequent Cerebral Vein Thrombosis

Authors: Costa P, Del Zotto E, Giossi A, Volonghi I, Poli L, Frigerio M, Padovani A, Pezzini A.

Cerebral vein thrombosis (CVT) is a rare complication of spontaneous intracranial hypotension (SIH). When to suspect a thrombotic disorder during the course of intracranial hypotension is not fully elucidated. A 48-year-old woman was admitted because of SIH with no signs of CVT on neuroimaging. The occurrence of diplopia and blurred vision 12 days later led to the performance of further investigations, which revealed thrombosis of the left lateral sinus, in the absence of variations in the headache characteristics. Among the other 4 cases of SIH clearly preceding the occurrence of CVT reported so far, only one had a change in the headache pattern related to CVT development. Although a change in the characteristics of headache is considered a marker of CVT in patients with SIH, this is not invariably part of the clinical scenario. Any new neurologic finding on exam in the disease course should raise a suspicion of venous thrombosis, thus prompting further specific investigations.
© 2012 American Headache Society.

Wind instruments and headaches

Authors: Martínez-Lage JF, Galarza M, Pérez-Espejo MA, López-Guerrero AL, Felipe-Murcia M.

The authors illustrate the cases of two children with headaches, one diagnosed with Chiari type 1 malformation and the other with hydrocephalus, who played wind instruments. Both patients manifested that their headaches worsened with the efforts made during playing their musical instruments. We briefly comment on the probable role played by this activity on the patients' intracranial pressure and hypothesize that the headaches might be influenced by increases in their intracranial pressure related to Valsalva maneuvers. We had serious doubts on if we should advise our young patients about giving up playing their music instruments.


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