Isolated cerebrospinal fluid hypertension in chronic headache: diagnostic innovations and clinical implications

Isolated cerebrospinal fluid hypertension (ICH) is a condition of increased cerebrospinal fluid (CSF) pressure in the cranial-spinal compartment without an identifiable cause. Isolated headache is the most common symptom of ICH, while missing may be signs such as papilledema or sixth nerve palsy. This fact makes difficult the clinical diagnosis of headache attributable to ICH in headache sufferers. Another source of confusion stems from the CSF pressure measurement. It has been observed that a single-spot CSF opening pressure measurement may be insufficient to identify elevated CSF pressure in headache sufferers. A new method of CSF pressure measurement has been able to identify pressure-related features of isolated CSF hypertension (ICH). In fact, nocturnal or postural headache and abnormal pressure pulsations are the more common pressure-related features of ICH in patients with chronic headache. The compressive action of these abnormal pressure pulsations causes the periventricular white matter microstructure alterations leading to the focal diffusion tensor imaging findings in patients with ICH. Abnormal pressure pulsations are a marker of ICH in chronic headache. The identification of the CSF pressure-related features may be useful for differentiating headache sufferers with ICH from those with primary headache disorder in clinical practice. The therapeutic strategy in these headache sufferers with ICH includes the CSF removal and a medical treatment.