scholarly journals Frontotemporal brain sagging syndrome: Craniospinal hypovolemia secondary to a T6-T7 cerebrospinal fluid-venous fistula

2020 ◽  
Vol 11 ◽  
pp. 250
Author(s):  
Luis Alberto Ortega-Porcayo ◽  
Eduardo Perusquia Ortega ◽  
Oscar Quiroz-Castro ◽  
Roger Antonio Carrillo-Meza ◽  
Juan Antonio Ponce-Gomez ◽  
...  

Background: The frontotemporal brain sagging syndrome (FTBSS) is defined as an insidious/progressive decline in behavior and executive functions, hypersomnolence, and orthostatic headaches attributed to cerebrospinal fluid (CSF) hypovolemia. Here, a T6 CSF-venous fistula (e.g., between the subarachnoid CSF and a paraspinal vein) resulted in a CSF leak responsible for craniospinal hypovolemia. Case Description: A 56-year-old male started with orthostatic headaches and fatigue after scuba diving. His symptoms included progressive, vertigo, tinnitus, nausea, lack of judgment, inappropriate behavior, memory dysfunction, apathy, tremor, orofacial dyskinesia, dysarthria, dysphagia, and hypersomnolence. The lumbar puncture revealed an opening pressure of 0 cm H2O. Magnetic resonance imaging (MRI) findings included brain sagging, bilateral temporal lobe herniation, and pachymeningeal enhancement. The computed tomography (CT) myelogram showed a thoracic diverticulum and a CSF-venous leak at the T6-T7 level. Surgery, which comprised a T6-T7 laminotomy, allowed for dissecting, clipping, and ligating the diverticulum/fistula. The patient improved postoperatively (e.g., cognitive, behavioral, and brainstem symptoms). The follow-up MRI’s showed the reversion of the sagging index/uncal herniation. Conclusion: The FTBSS should be considered in the differential diagnosis of an early onset frontotemporal dementia. Establishing the diagnosis and localizing the site of a spinal CSF/venous leak warrant both MRI and myelogram CT studies, to pinpoint the CSF leak site for proper surgical clipping/ligation of these thoracic diverticulum/CSF-venous leaks.

2001 ◽  
Vol 15 (5) ◽  
pp. 333-342 ◽  
Author(s):  
Paolo Castelnuovo ◽  
Silvia Mauri ◽  
Davide Locatelli ◽  
Enzo Emanuelli ◽  
Giovanni Delù ◽  
...  

Endoscopic repair of cerebrospinal fluid (CSF) rhinorrhea is becoming a common procedure. The purpose of this study was to perform a literature analysis centering cases of treatment failure and to review our 31 cases with a 1-year minimum follow-up. An extensive search of the literature was conducted, which focused on success rate, follow-up, diagnostic techniques, graft material used, failure rate, and comments on failures. A retrospective analysis of our 31 patients was carried out, and all cases were treated with the endoscopic approach with a 1-year minimum follow-up. From the literature analysis, the median success rate at the first endoscopic attempt is 90%. Our success rate was 87.1%. Failures were analyzed. A unique protocol for CSF leak diagnosis does not exist; we suggest our diagnostic algorithm. Graft material used depends on the authors’ experience, and based on this review of cases to date, did not significantly influence the success rate. The analysis of cases of failure shows that the majority of authors omit details. More research is needed to improve prevention of failures.


1998 ◽  
Vol 88 (2) ◽  
pp. 243-246 ◽  
Author(s):  
Wouter I. Schievink ◽  
Vittorio M. Morreale ◽  
John L. D. Atkinson ◽  
Fredric B. Meyer ◽  
David G. Piepgras ◽  
...  

Object. Spontaneous spinal cerebrospinal fluid (CSF) leaks are an increasingly recognized cause of intracranial hypotension and may require neurosurgical intervention. In the present report the authors review their experience with the surgical management of spontaneous spinal CSF leaks. Methods. Between 1992 and 1997, 10 patients with spontaneous spinal CSF leaks and intracranial hypotension were treated surgically. The mean age of the seven women and three men was 42.3 years (range 22–61 years). Preoperative imaging showed a single meningeal diverticulum in two patients, a complex of diverticula in one patient, and a focal CSF leak alone in seven patients. Surgical exploration in these seven patients demonstrated meningeal diverticula in one patient; no clear source of CSF leakage could be identified in the remaining six patients. Treatment consisted of ligation of the diverticula or packing of the epidural space with muscle or Gelfoam. Multiple simultaneous spinal CSF leaks were identified in three patients. Conclusions. All patients experienced complete relief of their headaches postoperatively. There has been no recurrence of symptoms in any of the patients during a mean follow-up period of 19 months (range 3–58 months; 16 person-years of cumulative follow up). Complications consisted of transient intracranial hypertension in one patient and leg numbness in another patient. Although the disease is often self-limiting, surgical treatment has an important role in the management of spontaneous spinal CSF leaks. Surgery is effective in eliminating the headaches and the morbidity is generally low. Surgical exploration for a focal CSF leak, as demonstrated on radiographic studies, usually does not reveal a clear source of the leak. Some patients may have multiple simultaneous CSF leaks.


Cephalalgia ◽  
2008 ◽  
Vol 28 (12) ◽  
pp. 1345-1356 ◽  
Author(s):  
WI Schievink

Spontaneous intracranial hypotension is an uncommon but not rare cause of new onset daily persistent headaches. A delay in diagnosis is the norm. Women are affected more commonly than men and most are in the fifth or sixth decade of life. The underlying cause is a spontaneous spinal cerebrospinal fluid (CSF) leak. Typically the headache is orthostatic in nature but other headache patterns occur as well. Associated symptoms are common and include neck pain, a change in hearing, diplopia, facial numbness, cognitive abnormalities and even coma. Typical imaging findings consist of subdural fluid collections, pachymeningeal enhancement, pituitary hyperaemia and brain sagging, but magnetic resonance imaging may be normal. Myelography is the study of choice to identify the CSF leak but is not always necessary to make the diagnosis. Treatment consists of bedrest, abdominal binder, epidural blood patching, percutaneous fibrin glue injection or surgical CSF leak repair. Outcomes have been poorly studied.


2014 ◽  
Vol 6 (4) ◽  
Author(s):  
Vivek Verma ◽  
Matthew Roman ◽  
Disha Shah ◽  
Marina Zaretskaya ◽  
Mohamed H. Yassin

A 54-year-old female living in Europe presented with gait ataxia, dizziness, and bilateral hearing loss. Magnetic resonance imaging (MRI) revealed non-specific white matter changes. The patient’s condition gradually deteriorated over two years without diagnosis. The patient continued to decline cognitively and neurologically with worsening ataxia and upper motor neuron signs. Repeat MRI showed worsening white matter changes. Lumbar puncture, not previously done, showed positive Lyme testing. Treatment with intravenous ceftriaxone resulted in marked neurological improvement. Four years after symptom, the patient has short-term memory deficits and chronic fatigue, but is otherwise neurologically, cognitively, and functionally intact. Follow up MRI findings remain largely unchanged. Because cases of intraparenchymal or encephalopathic neuroborreliosis in America are lacking, so are treatment options. We present a rare case and discuss our experience with antibiotic treatment. This case lends evidence to define optimal treatment of this disease, imperative for hastening neurological recovery.


Cephalalgia ◽  
1999 ◽  
Vol 19 (2) ◽  
pp. 80-87 ◽  
Author(s):  
CP O'Carroll ◽  
M Brant-Zawadzki

The authors report four cases of headache and other symptomatology related to the syndrome of intracranial hypotension. They were seen in a routine clinical practice over the past 3 years. The clinical features, magnetic resonance imaging (MRI) findings, and follow-up of these patients are described. Review of the prior literature on the topic is also included. All four patients presented with orthostatic headache syndrome. Three of the four demonstrated diffuse leptomeningeal thickening and enhancement on MRI studies. One subsequently developed a subdural effusion. One patient demonstrated downward displacement of the posterior fossa initially, which resolved on follow-up MRI scanning. Possible pathophysiologies of the syndrome are discussed.


2017 ◽  
Vol 25 (3) ◽  
pp. 338-343 ◽  
Author(s):  
Julia Kroth ◽  
Dumitru Ciolac ◽  
Vinzenz Fleischer ◽  
Nabin Koirala ◽  
Julia Krämer ◽  
...  

Background: Currently, no unequivocal predictors of disease evolution exist in patients with multiple sclerosis (MS). Cortical atrophy measurements are, however, closely associated with cumulative disability. Objective: Here, we aim to forecast longitudinal magnetic resonance imaging (MRI)-driven cortical atrophy and clinical disability from cerebrospinal fluid (CSF) markers. Methods: We analyzed CSF fractions of albumin and immunoglobulins (Ig) A, G, and M and their CSF to serum quotients. Results: Widespread atrophy was highly associated with increased baseline CSF concentrations and quotients of albumin and IgA. Patients with increased CSFIgA and CSFIgM showed higher functional disability at follow-up. Conclusion: CSF markers of blood–brain barrier integrity and specific immune response forecast emerging gray matter pathology and disease progression in MS.


1999 ◽  
Vol 37 (2) ◽  
pp. 467-470 ◽  
Author(s):  
K. K. Singh ◽  
M. D. Nair ◽  
K. Radhakrishnan ◽  
J. S. Tyagi

This is the first report of a case in which diagnosis of en-plaque tuberculoma on the basis of magnetic resonance imaging (MRI) findings was confirmed by a Mycobacterium tuberculosiscomplex-specific PCR assay of cerebrospinal fluid. The accuracy of the diagnosis was supported by good response to antitubercular drugs, which was shown by repeat MRI studies performed after treatment.


2003 ◽  
Vol 99 (5) ◽  
pp. 840-842 ◽  
Author(s):  
Wouter I. Schievink ◽  
M. Marcel Maya ◽  
Mary Riedinger

Object. Intracranial hypotension due to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural headaches, but reliable follow-up data are lacking. The authors undertook a study to determine the risk of a recurrent spontaneous spinal CSF leak. Methods. The patient population consisted of a consecutive group of 18 patients who had been evaluated for consideration of surgical repair of a spontaneous spinal CSF leak. The mean age of the 15 women and three men was 38 years (range 22–55 years). The mean duration of follow up was 36 months (range 6–132 months). The total follow-up time was 654 months. A recurrent spinal CSF leak was defined on the basis of computerized tomography myelography evidence of a CSF leak in a previously visualized but unaffected spinal location. Five patients (28%) developed a recurrent spinal CSF leak; the mean age of these four women and one man was 36 years. A recurrent CSF leak developed in five (38%) of 13 patients who had undergone surgical CSF leak repair, compared with none (0%) of five patients who had been treated non-surgically (p = 0.249). The recurrent leak occurred between 10 and 77 months after the initial CSF leak, but within 2 or 3 months of successful surgical repair of the leak in all patients. Conclusions. Recurrent spontaneous spinal CSF leaks are not rare, and the recent successful repair of such a leak at another site may be an important risk factor.


2017 ◽  
Vol 35 (6_suppl) ◽  
pp. e539-e539
Author(s):  
Igor Nunes-Silva ◽  
Eric Barret ◽  
Mohammed Baghdadi ◽  
Victor Srougi ◽  
Silvia Garcia Barreras ◽  
...  

e539 Background: Magnetic resonance imaging (MRI) plays an important role as a treatment-monitoring tool along focal therapy (FT) follow-up. This is the first study to assess MRI ability to correctly predict prostate cancer (PCa) local T-stage in the FT postoperative scenario. We aimed to describe MRI effectiveness in predicting upstaging in two groups of men: 1) men who failure after primary FT and then underwent salvage robotic-assisted radical prostatectomy (S-RARP) and 2) men who underwent RARP as primary treatment (P-RARP). Methods: Prospective data of 2775 men underwent RARP for localized PCa from 2000 to 2016 were reviewed. Twenty-two men underwent S-RARP after FT failure (S-RARP group). Total 2750 underwent RARP as first treatment. Matched-pair 1:2 selection of 44 out of 2750 patients by age defined primary RARP group (P-RARP). All patients underwent MRI immediately before RARP. MRI findings were confronted with final surgical pathology. Primary endpoint: sensitivity, specificity, positive and negative predictive values; positive (+LR) and negative (-LR) likelihood ratio regarding upstaging analysis on S-RARP. Secondary endpoint: same effectiveness analysis on P-RARP. Results: Preoperative MRI failed in predicting upstaging in 80% versus 91.7% of patients (p = 0.515) that presented final pathological status ≥ pT3a on S-RARP and P-RARP groups, respectively. On the other hand, when final pathology described a localized disease T2a-T2c, MRI correctly predicted the final pathological status in 81.8% versus 95.2% of patients (p = 0.27) on S-RARP and P-RARP groups, respectively. Between-group analysis, showed sensitivity and specificity rates of 20% versus 8.33% and 81.8% versus 95.23%, respectively; Positive and negative predictive values of 33.33% versus 50% and 69.23% versus 64.51%, respectively; Positive (+LR) and negative (-LR) likelihood ratio of 1.1 versus 1.74 and 0.98 versus 0.96, respectively. Conclusions: MRI has shown to be a weak diagnostic tool for predicting extra-prostatic disease along FT follow-up. Urologists may be warned about the risk of underdiagnosis and undertreatment in patients presenting failure after FT.


2016 ◽  
Vol 32 (1) ◽  
pp. 120-126 ◽  
Author(s):  
Alexander J. P. W. Hartmann ◽  
Bruno P. Soares ◽  
Beau B. Bruce ◽  
Amit M. Saindane ◽  
Nancy J. Newman ◽  
...  

Magnetic resonance imaging (MRI) signs of elevated intracranial pressure and idiopathic intracranial hypertension have been well characterized in adults but not in children. The MRIs of 50 children with idiopathic intracranial hypertension and 46 adults with idiopathic intracranial hypertension were reviewed for optic nerve head protrusion, optic nerve head enhancement, posterior scleral flattening, increased perioptic cerebrospinal fluid, optic nerve tortuosity, empty or partially empty sella, tonsillar herniation, enlargement of Meckel’s cave meningoceles, and transverse venous sinus stenosis(TSS). Compared to adolescents (11-17 years, n = 40) and adults (>17 years, n = 46), prepubescent children (<11 years, n = 10) had lower frequencies of scleral flattening (50% vs 89% and 85%, P = .02), increased perioptic cerebrospinal fluid (60% vs 84% and 89%, P = .08), optic nerve tortuosity (20% vs 46% and 59%, P = .07), empty or partially empty sella (56% vs 78% and 93%, P = .007), and TSS (67% vs 93% and 96%, P = .04). Children with idiopathic intracranial hypertension have similar MRI findings as adults, but they are less frequent in prepubescent children.


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