Orthostatic Headaches Associated With Spontaneous Intracranial Hypotension and Autonomic Dysfunction—A Case Series in Young Patients

Background: Orthostatic headaches can be noted in spontaneous intracranial hypotension and orthostatic intolerance. We present a case series of young patients diagnosed with spontaneous intracranial hypotension and were treated for the same but subsequently developed orthostatic intolerance. Methods: We retrospectively reviewed charts for seven young patients with orthostatic headaches related to spontaneous intracranial hypotension and orthostatic intolerance. Results: Patients were diagnosed with spontaneous intracranial hypotension. Diagnosis was confirmed by identifying epidural contrast leakage and three of seven patients were noted to have early renal contrast excretion on computerized tomography myelography. Patients were treated with epidural blood patches. All patients showed persistent symptoms of autonomic dysfunction after treatment of spontaneous intracranial hypotension and orthostatic intolerance was confirmed with head-up tilt table test. Conclusions: Patients with spontaneous intracranial hypotension failing to improve following epidural blood patching should be evaluated for orthostatic intolerance.

Dural tear from diagnostic lumbar puncture followed by long-term morbidity: a case report

Background Lumbar punctures are performed in different medical settings and are a key procedure in the diagnosis of several neurological conditions. Complications are rare and generally self-limiting. There are no reports of symptomatic accumulation of fluid in the epidural space after lumbar puncture in adults and there are no studies on long-term outcome after post dural puncture headache (PDPH). Case A lumbar puncture was performed in a 29 y.o. slender woman with unspecific symptoms to rule out neuro-infection. Next day MRI showed substantial accumulation of CSF in the epidural space from C2 to the sacrum dislocating the spinal chord in the spinal canal. The condition was ameliorated by epidural blood-patching. At 5 months she was still impaired by severe orthostatic headache. Conclusions The only plausible explanation for the massive CSF leak was a dural tear occurring during multiple attempts of lumbar puncture. Anterior dislocation of the spinal chord due to CSF leak is not a recognised complication to lumbar puncture. This complication was followed by long-term disability in our case. The diagnosis can be made by MRI. A difficult procedure with several attempts and use of traumatic technique may increase risk of this complication.

Behavioral Variant Frontotemporal Dementia as a Serious Complication of Spontaneous Intracranial Hypotension

BACKGROUND Behavioral variant frontotemporal dementia (bvFTD) is a devastating early onset dementia. Symptoms of bvFTD may be caused by spontaneous intracranial hypotension (SIH), a treatable disorder, but no comprehensive study of such patients has been reported. OBJECTIVE To describe detailed characteristics of a large cohort of patients with SIH and symptoms of bvFTD. METHODS We identified patients with SIH who met clinical criteria for bvFTD. Patients were compared to a cohort of SIH patients without bvFTD. RESULTS The mean age for the 21 men and 8 women was 52.9 yr (range, 37–65 yr). All 29 patients with bvFTD symptoms had hypersomnolence. Magnetic resonance imaging showed brain sagging in all patients, cerebrospinal fluid (CSF) opening pressure low in about half of patients, but a spinal CSF leak could not be detected in any patient. All patients underwent epidural blood patching, but 26 patients eventually underwent 1 or more surgical procedures. Overall, a good outcome was obtained in 21 patients (72%); 20 (91%) of 22 patients who had not undergone prior Chiari surgery compared to 1 (14%) of 7 patients who did undergo Chiari surgery (P < .003). Compared to SIH patients without symptoms of bvFTD (n = 547), those with bvFTD symptoms were older, more often male, less often demonstrated CSF leak on spinal imaging, and more often underwent surgery (P < .02). CONCLUSION bvFTD in SIH is rare and associated with brain sagging and hypersomnolence. Spinal CSF leaks are rarely detected. bvFTD symptoms are often refractory to the usual percutaneous procedures but most patients can be cured.

Ehlers Danlos, POTS, and Occult Cerebrospinal Fluid Leak: A Case Report

Ehlers Danlos syndromes (EDS) are associated with spontaneous intracranial hypotension (SIH) and postural orthostatic tachycardia syndrome (POTS). We hypothesized that some POTS patients might in fact have occult SIH due to unappreciated EDS. We describe a 26-year-old female with a history of POTS and headache who presented to us with negative imaging for cerebrospinal fluid leak (CSF). Upon examination with clinical evidence of EDS and supportive history, we performed an epidural blood patch despite repeat imaging evidence negative for CSF leak resulting in resolution of the patient’s POTS, headache, and cognitive symptoms. Patients with POTS and connective tissue disorders associated with dural weakness may be suffering from occult chronic CSF leaks. MRI imaging may be falsely reassuring and dissuade clinicians from performing epidural blood patching among patients who can be cured.

Intraspinal hemorrhage in spontaneous intracranial hypotension: link to superficial siderosis? Report of 2 cases

Spontaneous intracranial hypotension due to a spinal CSF leak has become a well-recognized cause of headaches, but such spinal CSF leaks also are found in approximately half of patients with superficial siderosis of the CNS. It has been hypothesized that friable vessels at the site of the spinal CSF leak are the likely source of chronic bleeding in these patients, but such an intraspinal hemorrhage has never been visualized. The authors report on 2 patients with spontaneous intracranial hypotension and intraspinal hemorrhage, offering support for this hypothesis. A 33-year-old man and a 62-year-old woman with spontaneous intracranial hypotension were found to have a hemorrhage within the ventral spinal CSF collection and within the thecal sac, respectively. Treatment consisted of microsurgical repair of a ventral dural tear in the first patient and epidural blood patching in the second patient. The authors suggest that spontaneous intracranial hypotension should be included in the differential diagnosis of spontaneous intraspinal hemorrhage, and that the intraspinal hemorrhage can account for the finding of superficial siderosis when the CSF leak remains untreated.