A Rare Case of Dyke-Davidoff-Masson Syndrome in an Adolescent Female.

The Dyke-Davidoff-Masson syndrome (DDMS) is an uncommon neurological disorder whose prevalence is not yet known. There have only been 21 adult manifestations of this rare brain disorder, out of around 100 cases previously documented. Diagnosis is challenging because of the complexity of radiological findings and clinical symptoms, which include ventricle dilation, hypertrophy of the cranial bones, increased pneumatization of the sinuses, and cerebral hemisphere atrophy. It can be inherited or acquired from infections, brain hemorrhage, and hypoxia during pregnancy. Usually, neuroimaging is used to diagnose it. This case study reports the case of a 17-year-old girl who had complex partial seizures at the age of 17 and right-side paralysis since she was three years old.

Dyke-Davidoff-Masson Syndrome: A Case Report.

Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological entity that is predominantly seen in childhood. Here, we present the case of a 13-year-old girl who was brought to the pediatric ward for general examination with a previous history of seizures, speech difficulty, facial deviation, and progressive left-sided hemiparesis that started at the age of two, followed by delayed developmental milestones. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain showed right cerebral hemiatrophy, ventriculomegaly, hyperpneumatization of the sinus, the decreased caliber of cortical veins, and skull thickening on the right were all characteristic findings of DDMS. Based on the history, clinical presentation, and imaging findings from CT and MRI, DDMS was confirmed. Identifying DDMS in a clinical setting can be challenging because of low awareness of the condition and varied clinical presentations. Although CT and MRI imaging are the gold standards in diagnosing DDMS, the early manifestations of the disease cannot be well-appreciated on a CT and would likely require an MRI. Since there is no standardized protocol for managing DDMS, the treatment is primarily symptomatic. Early identification and diagnosis of the syndrome are essential to aid the child's mental and physical development through a multidisciplinary approach. There is also a need to improve awareness of DDMS so that the condition can be considered a potential differential diagnosis amongst other similar conditions and does not get misdiagnosed. The lack of a proper protocol for the management of DDMS prompts more research for a better understanding and early identification of the condition.

Dyke-Davidoff-Masson Syndrome as a Predecessor of Recurrent Seizures in an Adult Male: A Report of a Rare Case.

Dyke-Davidoff-Masson syndrome (DDMS) is a rare condition that usually presents in early life with recurrent seizures. It can be congenital or can be acquired by perinatal hypoxia, infections, and intracranial hemorrhage. Its frequency remains unknown. It is usually diagnosed by neuroimaging. The classical neuroimaging features are unilateral cerebral hemiatrophy, volume loss, and hyperpneumatization of the sinus. We present the case of a 22-year-old male who presented with complex partial status epilepticus and had a history of recurrent seizures since he was six years old. The diagnosis of DDMS was made on neuroimaging.

[Analysis of correlation between the pneumatization degree of paranasal sinuses and skull base and spontaneous cerebrospinal fluid rhinorrhea].

Objective:To investigate the relationship between the pneumatization degree of paranasal sinuses and skull base and the incidence of spontaneous cerebrospinal fluid rhinorrhea （SCSFR）. Methods:Date of 107 patients with SCSFR were reviewed retrospectively. Using CT scans, investigator evaluated the pneumatization degree of paranasal sinuses and skull base, and compared classifications of various imaging characteristics between SCSFR group（case group） and nasal septum deviation group（control group）, including frontal cells, Onodi cell, lateral recess of sphenoid sinus （LRSS）, pneumatization of anterior clinoid process and pneumatization of posterior clinoid process. Results:One hundred and eight fistulas are found among 107 cases. The most common site of the fistulas is found in ethmoid sinus （38.89%）, followed by olfactory cleft（37.04%）, sphenoid sinus （21.30%） and frontal sinus （2.77%）.The incidence of Onodi cell was significantly different between case and control group（χ²=4.755, P<0.05）, and the classification of Onodi cell has a linear effect on the occurrence of SCSFR （Z=3.345, P<0.05）, which suggests the increasing level of Onodi cell can increase the probability in occurrence of SCSFR. The incidence of type Ⅲ LRSS in the case group is significantly higher than in the control group（χ²=18.725, P<0.05）,a linear correlation is found between the classification of LRSS and the incidence of SCSFR （Z=3.578, P<0.05）.There was no significant difference in the incidence of frontal cells between the two groups（χ²=0.672, P>0.05）, nor was there a linear relationship between the classification of frontal cells and the incidence of SCSFR （Z=0.503, P>0.05）. Pneumatization of anterior clinoid process and posterior clinoid process were no significant difference between case and control （P>0.05）,nor were there linear relationships between their classifications and SCSFR （P>0.05）. Conclusion:The most common site of SCSFR is ethmoid sinus, followed by olfactory cleft,the hyperpneumatization of the LRSS and Onodi cell are closely related to its pathogenesis.

[Multiple pneumocele associated with temporal bone hyperpneumatization]. / Mnozhestvennoe pnevmotsele pri giperpnevmatizatsii visochnoi kosti.

The authors consider in brief epidemiology, diagnostics, and variants of the surgical treatment of the rare clinical condition pneumocele and pneumoencephalocele of the temporal bone. Diagnostic and surgical modalities for the management of multiple pneumocele associated with temporal bone hyperpneumatization are discussed.

Skull base and craniocervical bone pneumatisation: two case reports of differing presentations and a review of the literature.

We report two patients with increased central skull base and craniocervical junction bone pneumatisation complicated by extra-osseous gas. One patient presented with symptoms of increasing nasal blockage and 'sinus pressure' on a background of extensive nasal polyposis. He was subsequently found to have a history of repeated Valsalva's manoeuvre, the cessation of which resulted in a rapid decrease in the amount of extra-osseous gas on imaging. The second patient presented following a minor head trauma with dysarthria from a hypoglossal nerve palsy and neck pain, with extensive intra- and extra-cranial gas including within the spinal canal (pneumorrhachis). These radiological findings have been reported previously in patients with Eustachian tube dysfunction and/or activities leading to frequently raised middle ear pressures. We review the possible aetiologies, reported risk factors, and the range of associated imaging abnormalities that may be encountered with this rare appearance.

A Spontaneous Pneumatocele Presenting with External Auditory Canal Obstruction / 대한이비인후과학회지

A pneumatocele is an air-filled cavity in the body. In the head and neck areas, the extensive hyperpneumatization of the temporal and occipital bones is typically associated with pneumatocele formation, which results in the spontaneous collection of gas, usually air, beneath the pericranium or within the skull. We herein report a case of stenosis of the external auditory canal caused by a left mastoid pneumatocele in a 12-year-old boy who was successfully treated with left simple mastoidectomy and canaloplasty.

Giant Spontaneous Epidural Pneumatocele: Case Report and Review of the Literature.

BACKGROUND: Pneumocephalus is a commonly encountered finding in neurosurgery in which air displaces intracranial cerebrospinal fluid after cranial surgery or a cerebrospinal fluid leak into paranasal or mastoid sinuses. When an intracranial air collection becomes chronically established in a fixed loculation causing mass effect, pneumatocele is a more appropriate term. We present an unusual case of a spontaneous giant frontotemporal epidural pneumatocele that persisted for more than 1 year before the patient presented for neurological treatment. CASE DESCRIPTION: A 40-year-old man with a remote history of minor head trauma presented with symptoms of headaches, dizziness, and vertigo. Imaging revealed a right giant epidural pneumocephalus secondary to bony dehiscence of the intracranial wall of the mastoid bone. A subtemporal middle fossa approach was performed to repair the mastoid defect with hydroxyapatite, fat graft, and temporalis fascia. The patient was discharged on postoperative day 2 with improvement in his headaches. One month after discharge operative imaging demonstrated complete resolution of his epidural pneumatocele and improvement in his headaches. CONCLUSIONS: This unique case represents the first reported case in the neurosurgical literature of a giant spontaneous epidural pneumatocele occurring in an adult treated with a middle fossa approach with resolution on follow-up imaging.

Mucosal contact points and paranasal sinus pneumatization: Does radiology predict headache causality?

OBJECTIVES/HYPOTHESIS: The goal of this study was to evaluate the prevalence of mucosal contact points (MCP), concha bullosa (CB), and variable paranasal sinus (PNS) volumes among patients sent for rhinogenic headache workup as compared with controls. STUDY DESIGN: Retrospective study with case and control groups. METHODS: Fifty-three adults with clear PNS computed tomography (CT) scans were included: 28 patients who have originally undergone PNS CT scan as part of sinus (rhinogenic) headache workup, and 25 controls in whom PNS CT scans were obtained for other purposes. All subjects were asked to report their symptoms using a headache scoring system. CT scans of all subjects were analyzed for presence of MCP, CB, as well as for volumes of maxillary, frontal, and sphenoid sinuses. RESULTS: MCP was found in 40% of controls, and in 50%, 50%, and 40% of mild, moderate, and severe headache groups, respectively. CB was found in 24% of controls, and in 33.3%, 58.3%, and 20%, of mild, moderate, and severe headache groups, respectively. Total volume of the measured PNS ranged from 23.9 to 81.4 cm(3) (mean ± standard deviation [SD], 48.3 ± 15.8) in the control group and from 5.31 to 87.4 cm(3) (mean ± SD, 43.6 ± 16) in the patient group. No statistically significant difference was found between groups regarding all studied variables. CONCLUSIONS: Radiological identification of MCP, CB, or hyperpneumatized sinuses does not seem to be a predictor of headache causality. Further studies are required to identify clinical scenarios in which these variations may contribute to pain symptoms. LEVEL OF EVIDENCE: 3b.

Craniocervical hyperpneumatization with concurrent pneumorrhachis, pneumomediastinum, and subcutaneous emphysema in a weightlifter.

BACKGROUND CONTEXT: Acquired hyperpneumatization of the skull base and upper cervical vertebrae is extremely rare and is thought to occur in patients who habitually perform the Valsalva maneuver or engage in repetitive positive pressure activities such as scuba diving or free diving. Craniocervical hyperpneumatization has been reported to cause intracranial and extracranial pneumatoceles but is not generally considered as a cause of pneumorrhachis (air in the spinal canal). Pneumorrhachis is relatively rare, and usually occurs in a localized form, either in the cervical spine secondary to skull base fractures or in the thoracic spine secondary to pneumomediastinum or pneumothorax. Here, we report a case of extensive pneumorrhachis extending from the skull base to the thoracolumbar junction in association with marked axio-atlanto-occipital hyperpneumatization and pneumomediastinum. This unique constellation of findings likely resulted from complications of the Valsalva maneuver during strenuous exercise. PURPOSE: To present a unique case of axio-atlanto-occipital hyperpneumatization with concurrent marked cervicothoracic pneumorrhachis, subcutaneous emphysema, and pneumomediastinum and to provide a review of the relevant literature, pathophysiology, and treatment strategies related to hyperpneumatization and pneumorrhachis. STUDY DESIGN/SETTING: A unique case report from an urban medical center. PATIENT SAMPLE: A single case. OUTCOME MEASURES: Imaging findings and clinical history. METHODS: Imaging data from a picture archiving and communication system and clinical data from an electronic medical record system were analyzed. RESULTS: A 58-year-old previously healthy man presented with 3 to 4 weeks of neck pain, shoulder pain, and intermittent hand and finger numbness that developed after weightlifting. On physical examination, he had mild hyperreflexia and decreased pinprick sensation within the T5-T8 dermatomes. Initial radiographic and computed tomography (CT) studies demonstrated extensive craniocervical hyperpneumatization involving the occipital bone, clivus, and C1 and C2 vertebral bodies. There was also pneumorrhachis extending throughout the entire cervical and thoracic spine, which caused moderate dural compression. Pneumomediastinum and subcutaneous emphysema were present. Maxillofacial CT showed dehiscent bone involving the dens, atlas, and occipital bone, with adjacent soft-tissue gas and pneumorrhachis. He was managed conservatively and advised to stop performing the Valsalva maneuver during weightlifting. His symptoms resolved, and follow-up imaging showed complete resolution of pneumorrhachis and partial reversal of hyperpneumatization. CONCLUSIONS: Craniocervical hyperpneumatization is a rare complication of the Valsalva maneuver. Most reported cases have involved only the skull base, or the skull base and C1, and many have been further complicated by microfractures leading to pneumocephalus or extracranial pneumatoceles. We present a unique case of extensive craniocervical hyperpneumatization that extended to the level of C2 and was complicated by microfractures causing severe pneumorrhachis. Concurrent pneumomediastinum in this case may have been an independent complication of the Valsalva maneuver, which could have contributed to pneumorrhachis. Alternatively, pneumomediastinum may have been caused by migration of gas through the neural foramen from the epidural space, driven by positive pressure generated by the one-way valve effect of the Eustachian tube during periods of exertion.

Spontaneous pneumocephalus associated with pneumosinus dilatans.

The majority of cases of pneumocephalus are secondary to trauma or medical intervention. Spontaneous, non-traumatic pneumocephalus is an uncommon condition. Most cases of spontaneous pneumocephalus require surgery. However, if there is no evidence of infection or cerebrospinal fluid leak, bed rest and follow-up imaging is an alternative treatment. Herein, we report a 31-year-old man with spontaneous pneumocephalus associated with pneumosinus dilatans.

Spontaneous Pneumocephalus Associated with Pneumosinus Dilatans

The majority of cases of pneumocephalus are secondary to trauma or medical intervention. Spontaneous, non-traumatic pneumocephalus is an uncommon condition. Most cases of spontaneous pneumocephalus require surgery. However, if there is no evidence of infection or cerebrospinal fluid leak, bed rest and follow-up imaging is an alternative treatment. Herein, we report a 31-year-old man with spontaneous pneumocephalus associated with pneumosinus dilatans.