Intracranial hypotension

Intracranial hypotension is a rare cause of orthostatic headache: a review of the etiology, treatment and prognosis of 13 cases

Authors: Güler S, Cağlı B, Utku U, Unlü E, Celik Y.

Objectives: The aim of this investigation is to examine the causes, clinical picture, treatment, and prognosis of spontaneous intracranial hypotension, a rare cause of orthostatic headache, among the cases presenting in our clinic. Methods: Thirteen cases (5 males and 8 females), diagnosed with spontaneous intracranial hypotension in our clinic between January 1st, 2009 and October 30th, 2011, were included in this study. The presenting symptoms, treatment, findings on cranial magnetic resonance imaging, cerebrospinal fluid pressure measured at lumbar puncture (in available patients), and the healing period of the patients were recorded. Results: Five patients with orthostatic headache and accompanying symptoms were treated with bed rest, increase in oral fluid intake, intravenous hydration and caffeine, and experienced a complete recovery. Complete recovery was observed in two patients (15.3%) within 10 days, in another two (15.3%) within 15 days and in one patient (7.6%) within 21 days. Headache and other clinical symptoms significantly regressed within 30 days in four patients (37.6%) who received similar treatment, but a mild headache persisted intermittently during follow-up in these individuals. As the headache had not resolved after 30 days, an epidural blood patch was applied in these four cases (37.6%) and the clinical picture completely improved within 10 to 15 days. Conclusion: Spontaneous intracranial hypotension should primarily be suspected in cases complaining about postural headache and contrast-enhanced cranial imaging should be performed. The presence of cranial nerve paralysis and pyramidal tract signs should b considered. Conservative treatments should be considered initially, however if conservative treatments fail, epidural blood patches must be applied.

Treatment of spontaneous intracranial hypotension: evolution of the therapeutic and diagnostic modalities

Authors: Franzini A, Messina G, Chiapparini L, Bussone G.

Magnetic resonance imaging in patients affected by orthostatic headache often allows the diagnosis of spontaneous intracranial hypotension (SIH). Nevertheless, in the last 5 years, the diagnostic and therapeutic strategy for spontaneous intracranial hypotension (SIH) has been significantly modified. Specifically, all invasive techniques aimed at localization and demonstration of "spontaneous" spinal fistulas (myelography, isotopic cisternography, and so on) have been progressively abandoned. Also myelo-MR, although not an invasive exam, is no longer considered necessary to establish the most appropriate treatment. This change is due to the development and demonstration of a pathogenetic theory which considers the cerebrospinal fistula not as the primary cause of intracranial hypotension, but as the consequence of an imbalance between epidural and cerebrospinal fluid (CSF) pressures. In a consecutive series of 80 patients, we performed a standardized epidural lumbar injection of a dense compound aimed to raise the epidural pressure irrespective of the presence and site of CSF leaks. The technique used, the long-term results of this treatment and the diagnostic pathways will be discussed.

Spontaneous intracranial hypotension and epidural blood patch: a report involving seven cases

Authors: Ferraro F, Marano E, Petruzzi J, Tedeschi E, Santulli L, Elefante A.

Spontaneous intracranial hypotension is a rare condition caused by spontaneous cerebrospinal fluid leak. It is characterised by orthostatic headache, diffuse pachymeningeal enhancement on brain imaging and low cerebrospinal fluid pressure. Seven patients with spontaneous intracranial hypotension were treated conservatively: of these, four responded to drug treatment and three underwent a lumbar autologous epidural blood patch (EBP). A complete response was obtained in two patients after a single EBP; one patient underwent a second EBP and then became asymptomatic. Clinical improvement coincided with a dramatic reduction of pachymeningeal enhancement. The aetiology and brain imaging findings, and the technique and effectiveness of EBP are discussed.

Update on intracranial hypertension and hypotension

Authors: Hoffmann, Jan; Goadsby, Peter J.

Purpose of review: The aim of this article is to review recent findings on the pathophysiology of idiopathic changes in intracranial pressure. The review will focus on idiopathic intracranial hypertension (IIH) and spontaneous intracranial hypotension (SIH).

Recent findings: Substantial evidence indicates that IIH is associated with delayed absorption of cerebrospinal fluid (CSF). Stenoses of the transverse sinus are common in IIH, but their clinical significance has not been entirely clarified. Despite the observed efficacy of endovascular treatment in some IIH patients, a correlation between the extent of observed stenoses and the clinical course of the disease could not be demonstrated. The underlying cause of SIH is a spontaneous CSF leakage into the epidural space. Conservative treatment and the epidural blood patch remain the treatment of choice for this rare syndrome.

Summary: Recent clinical evidence indicates that IIH is probably a result of CSF outflow abnormality rather than of CSF production. Further research is needed to elucidate the causes of elevated intracranial pressure and the mechanism leading to visual loss. Prospective randomized clinical trials are needed to clarify a possible therapeutic potential of endovascular treatment. Research efforts on SIH should focus further on associated connective tissue disorders predisposing to CSF leaks.

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.

Skull thickening, paranasal sinus expansion, and sella turcica shrinkage from chronic intracranial hypotension

Authors: Yoon MK, Parsa AT, Horton JC.

In children or young adults, the morphology of the skull can be altered by excessive drainage of CSF following placement of a ventriculoperitoneal (VP) shunt. In Sunken Eyes, Sagging Brain Syndrome, gradual enlargement of the orbital cavity occurs from low or negative intracranial pressure (ICP), leading to progressive bilateral enophthalmos. The authors report several heretofore unrecognized manifestations of this syndrome, which developed in a 29-year-old man with a history of VP shunt placement following a traumatic brain injury at the age of 9 years. Magnetic resonance imaging showed typical features of chronic intracranial hypotension, and lumbar puncture yielded an unrecordable subarachnoid opening pressure. The calvaria was twice its normal thickness, owing to contraction of the inner table. The paranasal sinuses were expanded, with aeration of the anterior clinoid processes, greater sphenoid wings, and temporal bones. The sella turcica showed a 50% reduction in cross-sectional area as compared with that in control subjects, resulting in partial extrusion of the pituitary gland. These new features broaden the spectrum of clinical findings associated with low ICP. Secondary installation of a valve to restore normal ICP is recommended to halt progression of these rare complications of VP shunt placement.


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