Abstracts 2011

Schievink WI, Dodick DW, Mokri B, Silberstein S, Bousser MG, Goadsby PJ.
Headache. 2011 Oct;51(9):1442-4.
doi: 10.1111/j.1526-4610.2011.01911.x.
Abstract
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.
PMID: 21658029

Schievink WI, Maya MM, Nuño M.
J Neurosurg Spine. 2011 Oct;15(4):433-40.
doi: 10.3171/2011.5.SPINE10890.
Abstract
OBJECT:
Spontaneous intracranial hypotension is an important cause of new-onset daily persistent headache. Cerebellar hemorrhage has been identified as a possible feature of spontaneous intracranial hypotension. The authors reviewed the MR imaging studies from a group of patients with spontaneous intracranial hypotension to assess the presence of cerebellar hemorrhage.
METHODS:
Medical records and radiological images were reviewed in 262 cases involving patients with spontaneous intracranial hypotension who had undergone MR imaging of the brain as well as spinal imaging.
RESULTS:
Chronic cerebellar hemorrhages were found in 7 (2.7%) of the 262 patients with spontaneous intracranial hypotension. These hemorrhages were found in 7 (19.4%) of the 36 patients with a ventral spinal CSF leak and in none of the 226 patients who did not have such a CSF leak (p < 0.0001). The degree of hemosiderin deposits was variable, ranging from mild involvement of the cerebellar folia to widespread superficial siderosis. Only the 1 patient with superficial siderosis had symptoms due to the hemorrhages. The time period between the onset of symptoms due to spontaneous intracranial hypotension and MR imaging examination was significantly longer in those patients with cerebellar hemorrhage than in those with a ventral spinal CSF leak and no evidence for cerebellar hemorrhage (mean 19.6 years vs 2.3 months, p < 0.0001).
CONCLUSIONS:
Chronic cerebellar hemorrhage should be included among the manifestations of spontaneous intracranial hypotension. The severity is variable, but the hemorrhage generally is asymptomatic. The underlying spinal CSF leak is ventral and mostly of long duration.
PMID: 21740128
Full text

Schievink WI, Maya MM.
J Neurosurg. 2011 Jul;115(1):113-5.
doi: 10.3171/2011.2.JNS101805.
Abstract
OBJECT:
Spontaneous intracranial hypotension (SIH) is a significant cause of new-onset daily persistent headache. A generalized connective tissue disorder also involving the intracranial arteries has been suspected in the population with SIH. Therefore, the authors reviewed angiographic studies for the presence of intracranial aneurysms in a group of patients with SIH.
METHODS:
Magnetic resonance angiography studies of the brain were performed in 93 patients with SIH (mean age 43 years, range 14-86 years) and in 291 controls (mean age 56 years, range 28-78 years).
RESULTS:
Intracranial aneurysms were detected in 8 (8.6%) of the 93 patients with SIH (95% CI 2.9%-14.3%). This incidence was higher than in the control population (3 (1.0%) of 291 (95% CI 0%-2.2%; p = 0.0007). In 7 patients the aneurysms were incidental, and in 1 patient SIH developed 5 weeks after an aneurysmal subarachnoid hemorrhage.
CONCLUSIONS:
In this retrospective case-control study, the frequency of intracranial aneurysms among patients with SIH was significantly higher than in the control population.
PMID: 21395391
full text: thejns.org/doi/full/10.3171/2011.2.JNS101805

Liu FC, Fuh JL, Wang YF, Wang SJ.
Cephalalgia. 2011 Apr;31(6):691-5.
doi: 10.1177/0333102410394676.
Abstract
OBJECTIVE:
Spontaneous intracranial hypotension (SIH) is caused by spinal cerebrospinal fluid (CSF) leakage. An underlying connective tissue disorder has been hypothesized to cause dural weakness and predisposition to CSF leak. We conducted a case-controlled study to investigate the role of connective tissue disorders in SIH patients.
METHODS:
We recruited 55 consecutive SIH patients (38 F, 17 M; mean age, 40.8 ± 9.8 years) and 55 age- and sex-matched control individuals (mean age, 38.0 ± 8.9 years) for this study. The connective tissue disorders were evaluated by: (i) Beighton hypermobility scores and revised diagnostic criteria for benign joint hypermobility syndrome; (ii) skin and skeletal manifestations of Ehlers-Danlos syndrome (EDS); and (iii) skeletal features of Marfan syndrome.
RESULTS:
The frequencies of joint hypermobility according to Beighton scores >4/9 (SIH 23.6% vs controls 16.4%, P = 0.48) and revised benign joint hypermobility syndrome criteria (SIH 23.6% vs controls 34.5%, P = 0.29) did not differ between SIH patients and controls. Sixteen patients and 16 controls had one or more skin features of EDS (P = 1.0). Nine SIH patients (16.4%) demonstrated the skeletal features of Marfan syndrome; this frequency did not differ from that of the control group (9.1%; P = 0.262). Only dolichostenomelia (disproportionately long limbs) was more prominent in SIH patients than in controls (34.5% vs 9.1%; P = 0.002).
CONCLUSION:
Compared with Western studies, the frequencies of connective tissue disorders were higher in our SIH patients. However, these frequencies did not differ between SIH patients and control individuals, except for dolichostenomelia.
PMID: 21220378

Wicklund MR, Mokri B, Drubach DA, Boeve BF, Parisi JE, Josephs KA.
Neurology. 2011 Apr 19;76(16):1377-82.
doi: 10.1212/WNL.0b013e3182166e42.
Abstract
BACKGROUND:
Behavioral variant frontotemporal dementia (bvFTD) is a relatively well-defined clinical syndrome. It is associated with frontal and temporal lobe structural/metabolic changes and pathologic findings of a neurodegenerative disease. We have been evaluating patients with clinical and imaging features partially consistent with bvFTD but with evidence also suggestive of brain sagging, which we refer to as frontotemporal brain sagging syndrome (FBSS).
METHODS:
Retrospective medical chart review to identify all patients seen at our institution between 1996 and 2010, who had a clinical diagnosis of FTD and imaging evidence of brain sag.
RESULTS:
Eight patients, 7 male and 1 female, were diagnosed with FBSS. The median age at symptom onset was 53 years. All patients had insidious onset and slow progression of behavioral and cognitive dysfunction accompanied by daytime somnolence and headache. Of the 5 patients with functional imaging, all showed evidence of hypometabolism of the frontotemporal regions. On brain MRI, all patients had evidence of brain sagging with distortion of the brainstem; 3 patients had diffuse pachymeningeal enhancement. CSF opening pressure was varied and CSF protein was mildly elevated. A definite site of CSF leak was not identified by myelogram or cisternography, except in one patient with a site highly suggestive of leak who subsequently underwent surgery confirming a CSF leak. In 2 patients with a neuropathologic examination, there was no evidence of a neurodegenerative disease.
CONCLUSIONS:
This case series demonstrates that FBSS may mimic typical bvFTD but should be recognized as an unusual presentation that is potentially treatable.
PMID: 21502595
Full text: PMC3087405