Penetrating Osseous Spicules Causing High-Flow Ventral CSF Leaks in the Setting of Relatively Low BMI : A Preliminary Study.

OBJECTIVE/BACKGROUND: We have anecdotally observed patients with high-flow ventral cerebrospinal fluid (CSF) leaks resulting from penetrating osseous spicules or calcified discs to be relatively thin. The purpose of this study was to explore the validity of this observation and determine if a potential association exists between low body mass index (BMI) and high-flow spinal ventral CSF leaks resulting from such dura-penetrating lesions. METHODS: Sixteen consecutive patients with precisely localized high-flow ventral spinal CSF leaks on dynamic myelography were identified. The cause of the CSF leak was determined. The BMI on the date nearest to and within 2 weeks of myelography was recorded. Utilizing exact sign test, the body mass index was compared to the average BMI from the National Health and Nutrition Examination Survey (Centers for Disease Control), matched to sex and age-range. RESULTS: The cohort consisted of 10 males (63%) and 6 females with a mean age of 54 years (range 37-72 years). In all patients, a spiculated osteophyte/calcified disc was identified at the site of the leak. Fourteen patients (88%) had a BMI below the matched national average, while only two patients (13%) had values above the national average (p = 0.004). CONCLUSIONS: Patients with high-flow ventral CSF leaks resulting from spiculated osteophyte or calcified disc as identified by dynamic myelography are more likely to have a BMI below the U.S. national average, matched for gender and age-range. This exploratory analysis requires confirmation as well as further characterization of potential pathophysiologic mechanisms and impact on radiographic and clinical assessments.

Re-evaluating the Incidence of Idiopathic Intracranial Hypertension in an Era of Increasing Obesity.

PURPOSE: To re-evaluate the population-based incidence of idiopathic intracranial hypertension (IIH) and to determine if it mirrors the rise in obesity. DESIGN: Retrospective, population-based cohort. PARTICIPANTS: All residents of Olmsted County, Minnesota, diagnosed with IIH between January 1, 1990, and December 31, 2014. METHODS: All cases of IIH were identified using the Rochester Epidemiology Project, which is a record-linkage system of medical records for all patient-physician encounters among Olmsted County, Minnesota, residents. All medical records were reviewed to confirm a diagnosis of IIH. The incidence rates of IIH were compared against the incidence of obesity in Minnesota over the same period. MAIN OUTCOME MEASURES: Incidence of IIH, lumbar puncture opening pressures, and body mass index. RESULTS: There were 63 new cases of IIH, yielding an overall age- and gender-adjusted annual incidence of 1.8 per 100 000 (95% confidence interval, 1.3-2.2) between 1990 and 2014. It increased from 1.0 per 100 000 (1990-2001) to 2.4 per 100 000 (2002-2014; P = 0.007). The incidence of IIH was 3.3 per 100 000 in women and 0.3 per 100 000 in men (P ≤ 0.001). In obese women 15 to 44 years of age, the incidence was 22.0 per 100 000 compared with 6.8 per 100 000 among all women in the same age group. A strong correlation was observed between IIH incidence rates and obesity rates in Minnesota (R2 = 0.70, P = 0.008). CONCLUSIONS: The incidence of IIH has increased since 1990, which is highly correlated with the rise in obesity during the same period.

Movement disorders associated with spontaneous CSF leaks: a case series.

IMPORTANCE AND OBJECTIVE: Headache is the most common symptom in spontaneous CSF leaks, frequently associated with additional manifestations. Herein, attention is drawn to movement disorder as a notable manifestation of spontaneous CSF leaks. DESIGN: Four women and one man (ages 51-78 years) with spontaneous CSF leaks and movement disorders were evaluated clinically and by pertinent neuroimaging studies with follow-up of one to seven years (mean 3.2 years). RESULTS: The movement disorder consisted of choreiform movements in two patients, torticollis in one, mixed tremor in one, and parkinsonism in one. All except the last patient had headaches (orthostatic in one, Valsalva maneuver-induced in one, both orthostatic and Valsalva-induced in two, lingering low-grade headache in one). Diffuse pachymeningeal enhancement and sinking of the brain was noted in all. CT-myelography showed definite CSF leak in three and equivocal leak in one, while no leak could be located in the fifth patient. Two patients improved over time with complete resolution of the movement disorder. One responded to epidural blood patch with complete resolution of his choreiform movements. Two patients required surgery and epidural blood patches. Results were drastic but nondurable in one, while complete recovery was achieved in the other. CONCLUSION: Movement disorders are uncommon in spontaneous CSF leaks but occasionally can be one of the major components of the clinical presentation.

Klippel­Trenaunay­Weber syndrome (KTWS) and spontaneous spinal CSF leak: coincidence or link.

OBJECTIVE: To highlight the occurrence of spontaneous cerebrospinal fluid (CSF) leak in the setting of Klippel­Trenaunay­Weber syndrome (KTWS). BACKGROUND: KTWS is a congenital multicomponent disorder of angiogenesis plus limb asymmetry. The cause of spontaneous CSF leaks often remains unknown, but the notion of a pre-existing dural weakness related to a disorder of connective tissue matrix is gaining momentum. REPORT OF CASES AND METHODS: Two women with KTWS developed spontaneous CSF leaks. Each underwent extensive head and spine imaging studies. One patient underwent surgery to treat the CSF leak and later an epidural blood patch upon partial recurrence of her symptoms. The other patient, who had intermittent CSF leak, developed cerebral venous thrombosis requiring several months of anticoagulation therapy. Both patients have histories of visceral bleeding: gastrointestinal in 1 patient and genitourinary in the other. RESULTS: The predominant site of vascular anomaly was the left lower limb in 1 patient and the right upper limb in the other, while the involved limb was larger in 1 patient and smaller in the other. Each patient presented with orthostatic headaches. One had additional choreiform movements and cognitive difficulties that responded to the treatment of the leak. Head magnetic resonance imaging in both patients showed diffuse pachy meningeal enhancement and evidence of sinking of the brain. Computed tomography myelography in 1 patient disclosed the site of the leak; and she underwent surgery to treat the leak, and later an epidural blood patch upon partial recurrence of her symptoms to which she responded well. The other patient had intermittent leak with history of long remission and was reluctant to go through invasive diagnostic or therapeutic measures. CONCLUSION: The occurrence of an uncommon disorder (spontaneous CSF leak) in the setting of a rare congenital disorder in 2 unrelated patients is intriguing. Whether this represents coincidence or a link is not clear but deserves further observations and investigation.

Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks.

Spontaneous intracranial hypotension typically results from spontaneous cerebrospinal fluid (CSF) leak, often at spine level and only rarely from skull base. Once considered rare, it is now diagnosed far more commonly than before and is recognized as an important cause of headaches. CSF leak leads to loss of CSF volume. Considering that the skull is a rigid noncollapsible container, loss of CSF volume is typically compensated by subdural fluid collections and by increase in intracranial venous blood which, in turn, causes pachymeningeal thickening, enlarged pituitary, and engorgement of cerebral venous sinuses on magnetic resonance imaging (MRI). Another consequence of CSF hypovolemia is sinking of the brain, with descent of the cerebellar tonsils and brainstem as well as crowding of the posterior fossa noted on head MRI. The clinical consequences of these changes include headaches that are often but not always orthostatic, nausea, occasional emesis, neck and interscapular pain, cochleovestibular manifestations, cranial nerve palsies, and several other manifestations attributed to pressure upon or stretching of the cranial nerves or brain or brainstem structures. CSF lymphocytic pleocytosis or increase in CSF protein concentration is not uncommon. CSF opening pressure is often low but can be within normal limits. Stigmata of disorders of connective tissue matrix are seen in some of the patients. An epidural blood patch, once or more, targeted or distant, at one site or bilevel, has emerged as the treatment of choice for those who have failed the conservative measures. Epidural injection of fibrin glue of both blood and fibrin glue can be considered in selected cases. Surgery to stop the leak is considered when the exact site of the leak has been determined by neurodiagnostic studies and when less invasive measures have failed. Subdural hematomas sometimes complicate the CSF leaks; a rebound intracranial hypertension after successful treatment of a leak is not rare. Cerebral venous sinus thrombosis as a complication is fortunately less common, and superficial siderosis and bibrachial amyotrophy are rare. Short-term recurrences are not uncommon, and long-term recurrences are not rare.

Ventral "spinal epidural meningeal cysts"--not epidural and not cysts? Case series and review of the literature.

BACKGROUND: Ventral spinal epidural meningeal cysts are rare entities for which the pathogenesis is poorly understood. OBJECTIVE: We present the clinical, radiographic, surgical, and pathologic findings of 4 patients with extensive ventral spinal epidural meningeal cysts and review the relevant literature. In addition, we discuss a suspected mechanism for pathogenesis. METHODS: Four patients with anterior spinal epidural meningeal cysts are retrospectively reviewed. RESULTS: Ventral spinal epidural meningeal cysts are often large, extending on average from C2 to L1 in our series. Patients typically present with a prolonged course of symptoms and signs, including segmental muscle weakness and atrophy, subtle myelopathy, mild to moderate spinal pain, and headache. Histopathologic analysis of the cyst wall demonstrates collagenous tissue consistent with dura but without arachnoid features. Dynamic computed tomographic myelography is the study of choice for localization of the primary dural defect. Patient symptoms and neurological deficits routinely improve after appropriate surgical intervention. CONCLUSION: Diverse signs and symptoms herald the presentation of ventral spinal meningoceles. Intraoperative, radiographic, and pathological findings are all suggestive of an intradural dissection as the etiology. Hence, they may be more appropriately named "ventral spinal intradural dissecting meningoceles." Definitive treatment involves identification and obliteration of the dural defect.

Orthostatic headaches in the syndrome of the trephined: resolution following cranioplasty.

OBJECTIVE: To draw attention to the syndrome of the trephined as a potential cause for orthostatic headaches without cerebrospinal fluid (CSF) leak. BACKGROUND: Orthostatic headaches typically result from CSF leaks but sometimes may occur in conditions without any evidence of CSF leakage. METHODS: A 37-year-old right-handed woman became comatose after a motor vehicle accident with cerebral contusions and massive left cerebral edema. A large frontoparietal craniectomy was carried out. In 5 months, she made good neurologic recovery. Freeze-preserved bone flap was placed back. In several weeks she was functionally near normal. Two years later, she began to complain of orthostatic headache and gradually additional manifestations appeared including progressive gait unsteadiness, imprecise speech, cognitive difficulties, and an increasing left hemiparesis along with progressive sinking of the skull defect and shift of the midline and ventricular distortion. She underwent removal of resorptive sinking bone flap and construction of an acrylic cranioplasty. RESULTS: At 6-month follow-up, there was complete resolution of the orthostatic headaches, remarkable neurologic improvement along with resolution of midline shift and ventricular distortion. CONCLUSION: The syndrome of the trephined is yet another cause of orthostatic headaches without CSF leak.

Positron emission tomography-computed tomography in paraneoplastic neurologic disorders: systematic analysis and review.

OBJECTIVE: To evaluate the cancer detection rate of whole-body positron emission tomography-computed tomography (PET-CT) in a paraneoplastic neurologic context. DESIGN: Retrospective medical record review. SETTING: Mayo Clinic, Rochester, Minnesota. PATIENTS: Fifty-six consecutive patients with clinically suspected paraneoplastic neurologic disorders who underwent PET-CT after negative standard evaluations, including CT. MAIN OUTCOME MEASURE: Rate of cancer detection. RESULTS: Abnormalities suggestive of cancer were detected using PET-CT in 22 patients (39%); 10 patients (18%) had cancer confirmed histologically. Cancers detected (limited stage in 9 of 10 patients and extratruncal in 4) were as follows: 2 thyroid papillary cell carcinomas, 3 solitary lymph nodes with unknown primary (2 adenocarcinomas and 1 small cell carcinoma), 1 tonsil squamous cell carcinoma, 3 lung carcinomas (1 adenocarcinoma, 1 small cell, and 1 squamous cell), and 1 colon adenocarcinoma. Detection of a well-characterized neuronal nuclear or cytoplasmic paraneoplastic autoantibody was associated with a successful PET-CT-directed cancer search (P < .001). Detection of limited-stage cancer facilitated early initiation of oncologic treatments and immunotherapy; cancer remission was reported in 7 patients, and sustained improvements in neurologic symptoms were reported in 5 (median follow-up, 11 months; range, 2-48 months). Combined data from 2 previous studies using conventional PET alone (123 patients) revealed that 28% of patients had a PET abnormality suggestive of cancer and that 12% had a cancer diagnosis. CONCLUSION: In a paraneoplastic neurologic context, PET-CT improves the detection of cancers when other screening test results are negative, particularly in the setting of seropositivity for a neuronal nuclear or cytoplasmic autoantibody marker of cancer.

A unifying hypothesis for a patient with superficial siderosis, low-pressure headache, intraspinal cyst, back pain, and prominent vascularity.

A source of bleeding is often not evident during the evaluation of patients with superficial siderosis of the CNS despite extensive imaging. An intraspinal fluid-filled collection of variable dimensions is frequently observed on spine MR imaging in patients with idiopathic superficial siderosis. A similar finding has also been reported in patients with idiopathic intracranial hypotension. The authors report on a patient with superficial siderosis and a longitudinally extensive intraspinal fluid-filled collection secondary to a dural tear. The patient had a history of low-pressure headaches. His spine MR imaging and spine CT suggested the possibility of an underlying vascular malformation, but none was found on angiography. Repair of the dural tear resulted in resolution of the intraspinal fluid collection and CSF abnormalities. The significance of the association between superficial siderosis and idiopathic intracranial hypotension, and potential therapeutic and pathophysiological implications, are the subject of this report.

Surgical anatomy of dural defects in spontaneous spinal cerebrospinal fluid leaks.

OBJECTIVE: Spontaneous intracranial hypotension is typically caused by a spontaneous spinal cerebrospinal fluid (CSF) leak. The configuration of the related dural defects can be complex. We describe our experience with the surgical anatomy of these defects. METHODS: Thirteen consecutive patients with spontaneous spinal CSF leaks who underwent surgical exploration at Mayo Clinic between 1994 and 2003 were studied. All patients' records, imaging studies, detailed intraoperative findings, and outcomes were reviewed. RESULTS: There were four men and nine women with an average age of 40 years (range, 12-62 yr). Preoperative imaging studies revealed a single site of CSF leak in eight patients, two sites in three patients, and multiple sites in two patients. Intraoperatively, the exact site of leakage could not be found in four patients. Among the other nine patients, primary closure of a meningeal diverticulum was achieved in one patient. Significant regional attenuation of the dura prevented primary repair of the leak site in eight patients. Muscle, fibrin glue, and Gelfoam (Upjohn Co., Kalamazoo, MI) soaked in patient's own blood were commonly used to pack the epidural space in an attempt to seal the site of the leak. Ligation of two nonappendicular nerve roots allowed closure of the leak in one of these patients. Postoperatively, resolution of symptoms occurred in eight patients, significant improvement was noted in three patients, and only transient resolution in two. The mean duration of follow-up was 20.5 months. CONCLUSION: Surgery for closure of spontaneous spinal CSF leaks may not be straightforward. Even when extradural CSF leakage is discovered preoperatively by imaging studies, it may not always be possible to identify the exact site of the leakage intraoperatively. Furthermore, the anatomy of the dural defects may be complex and not amenable to primary closure. In such cases, the use of adjuvant techniques during surgical exploration may be effective.

Amphiphysin autoimmunity: paraneoplastic accompaniments.

Amphiphysin-IgG was identified in 71 patients among 120,000 evaluated serologically for paraneoplastic autoantibodies. Clinical information was available for 63 patients. Cancer was detected in 50 (mostly limited), proven histologically in 46, and was imaged intrathoracically in 4 patients (lung, small-cell [27] and non-small cell [1]), breast [16] and melanoma [2]). Neurological accompaniments included (decreasing frequency): neuropathy, encephalopathy, myelopathy, stiff-man phenomena, and cerebellar syndrome. In a case examined neuropathologically, parenchymal T-lymphocyte infiltration (predominantly CD8(+)) was prominent in lower brainstem, spinal cord, and dorsal root ganglion. Coexisting paraneoplastic autoantibodies, identified in 74% of patients, predicted a common neoplasm and indicated other neuronal autoantigen targets that plausibly explained several neurological manifestations; for example, P/Q-type Ca(2+)-channel antibody with Lambert-Eaton syndrome (n = 5), anti-neuronal nuclear antibody type 1 with sensory neuronopathy (n = 7), K(+)-channel antibody with limbic encephalitis (n = 1) or neuromyotonia (n = 1), and collapsin response-mediator protein-5-IgG with optic neuritis (n = 3). Patients with isolated amphiphysin-IgG (n = 19) were more likely to be women (with breast cancer, p < 0.05) and to have myelopathy or stiff-man phenomena (p < 0.01). Overall, a minority of women (39%) and men (12%) had stiff-man phenomena. Only 10% of women (some with lung carcinoma) and 4% of men fulfilled diagnostic criteria for stiff-man syndrome.

Syndrome resembling PSP after surgical repair of ascending aorta dissection or aneurysm.

Seven patients, after seemingly uncomplicated surgery for ascending aorta aneurysm or dissection, with or without aortic valve replacement, developed an unusual and fairly stereotyped biphasic neurologic disorder without imaging evidence of related cerebral ischemia or infarct. The initial phase was mild, nonprogressive, or receding. The latent and progressive phase closely resembled a progressive supranuclear palsy phenotype. The disorder may prove to be self-limiting but leaves the patients with considerable neurologic deficits.

Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks.

Four patients, aged 10 to 44 years, with spontaneous cerebrospinal fluid (CSF) leaks and intracranial hypotension developed intracranial hypertension after treatment of their CSF leaks. The leak was at the spinal level in all patients (thoracic level, 2; lumbar level, 1; and undetermined, 1). One patient responded to an epidural blood patch. Three patients responded to surgery, of whom 2 had not responded to prior epidural blood patches. Treatment resulted in complete resolution of symptoms, including orthostatic headaches and disappearance of magnetic resonance imaging abnormalities. However, all patients later developed steady headaches different from their previous headaches. None had recurrence of magnetic resonance imaging abnormalities or any evidence of occlusion of cerebral venous sinuses. All had increased CSF opening pressures. One had bilateral papilledema, and another had no venous pulsations on examination of fundi. Follow-up was possible in 2 patients. One responded well to treatment with acetazolamide, and the other improved gradually and was asymptomatic within several months.

Cervical artery dissections: evidence for redissection in previously dissected arteries: report of three cases.

OBJECTIVE AND IMPORTANCE: Among patients with spontaneous internal carotid artery (ICA) or vertebral artery dissections, there is a small but significant risk of development of dissection in an uninvolved ICA or vertebral artery at some time in the future. Recurrence of dissection in a previously dissected and healed ICA or vertebral artery is very rare. We retrospectively reviewed more than 400 cases of cervical artery dissections in our cerebrovascular records for 1970 through 2000. CLINICAL PRESENTATION: Three patients with recurrence of dissection in previously dissected and presumably healed ICAs were identified. All three patients were women. Their ages at the time of the initial dissection were 42, 38, and 31 years. Initial ICA dissections were bilateral in all cases, occurring simultaneously in one case and 1 year apart in the other two cases. All dissections were spontaneous. Redissection of a previously dissected and healed ICA in these three patents occurred 11, 6, and 12 years later, respectively. Two patients had fibromuscular dysplasia. CONCLUSION: Our experience with these three cases, from among more than 400 cases of cervical artery dissections treated at our institution, and the very limited number of reported cases in the literature indicate the rarity of recurrence of dissection in previously dissected and healed cervical arteries. An underlying arteriopathy may be suspected in many of these cases. Management requires medical and sometimes surgical approaches.

Rarity of encephalopathy associated with autoimmune thyroiditis: a case series from Mayo Clinic from 1950 to 1996.

Corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis (also called Hashimoto's encephalopathy) is a rare, life-threatening, treatable, and possibly autoimmune condition. We identified nine patients (with the diagnosis made after 1979) who had relapsing encephalopathy compatible with previous reports of Hashimoto's encephalopathy and no other identifiable cause of encephalopathy at Mayo Clinic Rochester. Of these nine patients, three were clinically hypothyroid, four were subclinically hypothyroid, and two were euthyroid. Thyroid antibodies were positive in eight of eight patients in whom these measurements were made. Electroencephalographic abnormalities were identified in eight of the nine patients (89%). Magnetic resonance imaging (MRI) abnormalities considered etiologically related to encephalopathy were present in three patients (33%). An increased protein concentration was noted on cerebrospinal fluid examination in seven patients (78%). Of the six patients who received high-dose glucocorticoid therapy, 5 (83%) had improvement of neurologic symptoms. In conclusion, encephalopathy associated with autoimmune thyroiditis is rare but important to recognize because it may be responsive to high-dose glucocorticoid therapy. We believe that this condition is not caused by thyroid dysfunction or antithyroid antibodies but represents an association of an uncommon autoimmune encephalopathy with a common autoimmune thyroid disease. The term Hashimoto's encephalopathy is a misnomer and should not be used.