Chronic daily headache in the adults: differential diagnosis between symptomatic Chiari I malformation and spontaneous intracranial hypotension

Authors: Eliana Mea, Luisa Chiapparini, Massimo Leone, Angelo Franzini, Giuseppe Messina and Gennaro Bussone.

This article briefly reviews the spectrum of headaches associated with Chiari type I malformation (CMI) and specifically analyzes the current data on the possibility of this malformation as an etiology for some cases of chronic daily headache (CDH). CMI is definitely associated with cough headache and not with primary episodic headaches, with the rare exception of basilar migraine-like cases. With regard to CDH, there is no clear evidence supporting an association with CMI. A magnetic resonance imaging (MRI) study would be justified only in patients showing either a Valsalva-aggravating component or cervicogenic features. Hydrocephalus and low-intracranial pressure syndrome should be ruled out in patients showing tonsillar herniation in an MRI study and consulting due to daily headache.

Diagnostic Criteria for Headache Due to Spontaneous Intracranial Hypotension: A Perspective.

Authors: Schievink WI, Dodick DW, Mokri B, Silberstein S, Bousser MG, Goadsby PJ.

The clinical and radiographic manifestations of spontaneous intracranial hypotension are highly variable and many patients do not satisfy the 2004 International Classification of Headache Disorders criteria. We developed new diagnostic criteria for spontaneous intracranial hypotension based on cases we have seen reflecting the variable manifestations of the disorder. These criteria provide a basis for change when the classification criteria are next revised. The diagnostic criteria consist of A, orthostatic headache; B, the presence of at least one of the following: low opening pressure (≤60 mm H(2) O), sustained improvement of symptoms after epidural blood patching, demonstration of an active spinal cerebrospinal fluid leak, cranial magnetic resonance imaging changes of intracranial hypotension (eg, brain sagging or pachymeningeal enhancement); C, no recent history of dural puncture; and D, not attributable to another disorder.

Headache caused by raised intracranial pressure and intracranial hypotension

Author: N M Ramadan.

Extremes of intracranial pressure commonly cause headache. Benign intracranial hypertension is a rare syndrome of increased intracranial pressure manifesting as headache, intracranial noises, transient visual obscuration, and palsy of the sixth cranial nerve. Endocrine disorders such as obesity and hypoparathyroidism, hypervitaminosis A, tetracycline use and thyroid replacement are probable causes of benign intracranial hypertension. In the majority of cases, however, it is idiopathic. Benign intracranial hypertension is though to be caused by cerebral edema, high cerebrospinal fluid outflow resistance and high cerebral venous pressure, or a combination of the three. The management of benign intracranial hypertension includes, symptomatic headache relief, removal of offending risk factor(s), and medical or surgical reduction of intracranial pressure. Spontaneous intracranial hypotension is more rare than benign intracranial hypertension. Postural headache (worse in the upright position) is the hallmark of spontaneous intracranial hypotension. Typically, the cerebrospinal fluid pressure is less than 60 mm H2O. Diminished cerebrospinal fluid production, hyperabsorption, and leak are postulated mechanisms of spontaneous intracranial hypotension. Empirical treatment includes bed rest, administration of caffeine, corticosteroids or mineralocorticoids, epidural blood patch, and epidural saline infusion.

Headaches caused by decreased intracranial pressure: diagnosis and management

Authors: Mokri, Bahram 

Current Opinion in Neurology: June 2003 - Volume 16 - Issue 3 - pp 319-326 Headache

Purpose of review: More patients with spontaneous intracranial hypotension are now being diagnosed, and it is realized that most cases result from spontaneous cerebrospinal fluid leaks. A broader clinical and imaging spectrum of the disorder is recognized. This paper reviews new insights into the variability of clinical manifestations, imaging features, etiological factors, anatomy of leaks, and implications of these in patient management.

Recent findings: Spontaneous intracranial hypotension should not be equated with post-lumbar puncture headaches. In a substantial minority of patients, headaches are not orthostatic and may mimic other types of headache. Additional diverse neurological manifestations may dominate the clinical picture and patients may occasionally have no headache at all. Reports on unusual presentations of the disorder continue to appear in the literature. Furthermore, additional imaging features of cerebrospinal fluid leaks are recognized. High-flow and slow-flow leaks may present diagnostic challenges, and require modification of diagnostic studies aimed at locating the site of the leak. Stigmata of connective tissue abnormality, especially abnormalities of fibrillin and elastin, are seen in a notable minority of patients, pointing to weakness of the dural sac as one of the etiological factors. After treatment of spontaneous intracranial hypotension, surgically or by epidural blood patch, a rebound and self-limiting intracranial hypertension may sometimes develop.

Summary: In the past decade, interest in spontaneous intracranial hypotension has been rekindled, with a substantial growth of knowledge on various aspects of the disorder. We are in the learning phase, and new information will probably appear in the future, with notable diagnostic and therapeutic implications.


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