Dural Reduction Surgery: A Treatment Option for Frontotemporal Brain Sagging Syndrome

Abstract

Frontotemporal brain sagging syndrome is a dementia associated with hypersomnolence, personality changes, and features of intracranial hypotension on magnetic resonance imaging. The literature is sparse with respect to treatment options; many patients simply worsen. We present a case in which this syndrome responded to lumbar dural reduction surgery. Postoperative magnetic resonance imaging indicated normalization of brain sagging and lumbar intrathecal pressure. Although no evidence of cerebrospinal leak was found, extremely thin dura was noted intraoperatively, suggesting that a thin and incompetent dura could result in this low-pressure syndrome. Clinicians who encounter this syndrome should consider dural reduction surgery as a treatment strategy.RÉSUMÉ: Chirurgie réductrice du sac dural : Une option thérapeutique dans le cas de l'affaissement de la région fronto-temporale du cerveau. Le syndrome d'affaissement (brain sagging) fronto-temporal constitue une démence associée à l'hyper-somnolence, à des changements de personnalité ainsi qu'à des traits d'hypotension intracrânienne mis en évidence par l'imagerie par résonnance magnétique (IRM). Peu d'écrits ont abordé les options de traitement. Qui plus est, nombreux sont les patients atteints dont la condition ira en se dégradant. Nous voulons présenter ici un cas à propos duquel ce type d'affaissement a bien répondu à une chirurgie réductrice du sac dural. Des examens postopératoires par IRM ont en effet indiqué une normalisation de l'affaissement fronto-temporal et de la pression lombaire intrathécale. Bien qu'aucune preuve de fuite de liquide cérébro-spinal n'ait été observée, une duremère extrêmement mince a été notée peropératoirement, ce qui suggère qu'une dure-mère amincie et inefficace pourrait résulter de ce syndrome d'hypotension. Les médecins cliniciens confrontés à un tel syndrome devraient du coup envisager la chirurgie réductrice du sac dural à titre de stratégie thérapeutique. CASE REPORTA 46-year-old right-handed man was referred for 2 years of progressive personality changes. He first developed hypersomnolence, mild frontal headaches not affected by postural change, and mild dysphagia for liquids. Eight months after onset, he developed memory loss and personality changes. He was charged with sexual assault, reckless driving, he started abusing cocaine, went on spending sprees, and developed hyperphagia.Fourteen months after onset, a brain magnetic resonance imaging (MRI) scan revealed a sagging brain with herniation of the cerebral tonsils below the foramen magnum and flattening of the pons ( Figure 1A). This was initially considered to be a Chiari I malformation and prompted referral to neurosurgery. Before surgical intervention, a psychiatric referral was made to address the unclear etiology of personality changes. After psychiatric assessment, the patient was referred to neurology with the suspicion of frontotemporal dementia. At this time, the patient's issues were hypersomnolence and apathy. He denied headache, depression, hallu...

Brain Sagging Dementia—Diagnosis, Treatment, and Outcome

“…Upon further scrutiny, one may reveal that the symptoms started with severe headaches several months to years before cognitive decline. 22,27,29,30,35 Like patients with SIH who may feel worsening of headaches towards the end of the day, patients with BSD may feel behavioral and cognitive decline towards the evening. 16 However, we are not sure whether an acute (within minutes) positional worsening and improvement of behavioral and cognitive symptoms are related to BSD.…”

“…Although their minor complaints of headache may mask the seriousness of the disease, patients with BSD have behavioral changes and lack of insight that can lead to poor decision-making with serious actions such as sexual assault and felony. 5-7,16,32,34,35,37…”

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

“…Treatment options for FTBSS depend on the CSF leak type [ Table 1 ]. [ 1 , 3 - 8 , 10 , 13 , 15 , 16 , 18 - 21 , 23 ] CSF-venous fistulas (Type 3 leaks) do not respond in the long-term to targeted blood patches and the main options include sacrificing a nonappendicular nerve root and/or direct venous fistula obliteration. [ 10 ] In our case, we decided to clipped and ligated a T6 CSF-venous fistula and a T7 diverticulum, following which symptoms and brain sagging improved [ Figure 3 ].…”

Spontaneous Intracranial Hypotension: Atypical Radiologic Appearances, Imaging Mimickers, and Clinical Look-Alikes

“…Patients with SIH can also have behavioral changes, and there have been several reports of SIH presenting with frontotemporal dementia-like symptoms. [75][76][77][78] The etiology of this relationship is not known but may be related to brain sag, obstruction of the venous outflow, and associated swelling of the diencephalon. 78 In most cases, cognitive symptoms promptly improve almost immediately after treating the underlying SIH.…”

“…78 In most cases, cognitive symptoms promptly improve almost immediately after treating the underlying SIH. [75][76][77][78] Pituitary Tumor or Apoplexy. As the volume of intracranial CSF declines in SIH, the compensatory enlargement of the cerebral venous system includes the hypophyseal veins.…”