Spontaneous low cerebrospinal pressure/volume headaches

Abstract

The majority, if not all, of the cases of spontaneous intracranial hypotension result from spontaneous cerebrospinal fluid (CSF) leaks. The disorder has a broad clinical and imaging spectrum with substantial variability in clinical and imaging features, in CSF findings, and in response to treatment. Headache is the most common symptom and is typically orthostatic, but with chronicity the orthostatic features may blur into a chronic, lingering headache. Other clinical features include neck pain, nausea, emesis, interscapular pain, diplopia, dizziness, change in hearing, visual blurring, radicular upper extremity symptoms, and a variety of other, but much less common, manifestations. The most common imaging feature is diffuse pachymeningeal gadolinium enhancement. Other manifestations include imaging evidence of sinking of the brain, subdural fluid collections, enlargement of the pituitary, engorgement of venous sinuses, and engorgement of epidural venous plexus. CSF opening pressure is typically low and CSF analysis may be normal or show increased protein concentration and a primarily lymphocytic pleocytosis. No longer can the entity be simply equated with post-spinal puncture headaches. The pathogenetic core and the independent variable is decrease in CSF volume, whereas clinical imaging and CSF findings, including CSF opening pressures, are all variables dependent on the loss of CSF volume. Many patients respond well to treatment, but some present stubborn therapeutic challenges. A subgroup of patients with orthostatic headaches is gradually recognized who have disorders other than CSF leaks.

When Should I Do Dynamic CT Myelography? Predicting Fast Spinal CSF Leaks in Patients with Spontaneous Intracranial Hypotension

“…13 In addition, the variability of CSF opening pressure has also been observed. 4 Our study supports these observations and demonstrates the lack of association between findings on head MR imaging or opening pressure and the rate of CSF leak.…”

“…1,4,11,12 However, to our knowledge, the ability of spinal MR imaging to predict fast CSF leaks has not been previously described.…”

“…1,[4][5][6][7] The combination of postural headaches and typical changes on head MR imaging-which include diffuse pachymeningeal enhancement, subdural fluid collections, and "sagging" or descent of the brain associated with descent of the cerebellar tonsils, partial effacement of basal cisterns, and inferior displacement of the optic chiasm-suggests the diagnosis of SIH. 4 In our experience, the greatest diagnostic challenge is no longer the recognition of SIH but the confirmation and localization of the site of CSF leak.…”

Signals for nausea and emesis: Implications for models of upper gastrointestinal diseases

“…Hamster Cytotoxic drugs: Cyclophosphamide (Hobbs et al, 1976) Intragastric irritants: LiCl (Fox, 1977) Apomorphine (Nowlis et al, 1980) Nicotine (Etscorn et al, 1986) Other: 2-deoxy-D-glucose (Dibattista, 1988) Human Cytotoxic drugs: Chemotherapy (Rudd and Andrews, 2005) Chemotherapy (Schwartz et al, 1996) Intragastric irritants: Ipecac (Jackson and Smith, 1978), Copper sulfate (Liu et al, 2001) Ipecac (Jackson and Smith, 1978) Motion (Yates et al, 1998) (Arwas et al, 1989) Apomorphine (Schofferman, 1976) Morphine (Bailey et al, 1993) Radiation (Cordts et al, 1987) (Carrell et al, 1986) Hormones and neurotransmitters: CCK (Miaskiewicz et al, 1989) Other: Pregnancy (Weigel and Weigel, 1989), Reduced intracranial pressure (Mokri, 2004) Pregnancy (Bayley et al, 2002) Pregnancy (Corbett et al, 2003;Lopez et al, 2004), Gastric bypass surgery (Kushner et al, 2004) Monkey Cytotoxic drugs: Cisplatin (Fukui et al, 1993) Cyclophosphamide (Hikami et al, 1990) Intragastric irritants: Copper sulfate (Fukui et al, 1993) LiCl (Bergman and Glowa, 1986) Motion (Wilpizeski et al, 1987) (Wilpizeski et al, 1987) Radiation (Brizzee, 1956) Nicotine (Spealman, 1983) Hormones and neurotransmitters: CCK (Perera et al, 1993) Mouse Cytotoxic drugs: Cisplatin …”

Factors affecting cerebrospinal fluid opening pressure in patients with spontaneous intracranial hypotension

“…The acronym SEEPS can be used to summarize the typical MRI features: subdural fluid collections, enhancement of pachymeninges, engorgement of venous structures, pituitary enlargement, and sagging of the brain (Schievink, 2006;Spears, 2014;Limaye et al, 2016). The mechanisms of these abnormalities can be well explained by the Monro-Kellie hypothesis (Mokri, 2001;2004;2013;Limaye et al, 2016). In the intact rigid skull, the total volume including that of the brain, CSF, and intracranial blood is constant.…”