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Acta Bio-medica : Atenei Parmensis Jul 2021Myelomeningocele is a congenital malformation caused by a developmental defect of the spinal cord structures. The exactcause is unknown, but different factors have...
Myelomeningocele is a congenital malformation caused by a developmental defect of the spinal cord structures. The exactcause is unknown, but different factors have been involved includingradiation, malnutrition, drugs. Myelomeningocele can develop at any point in the spine, but the lumbosacral region is affected in over 75% of cases. Chest X-raysand computed tomography study are mandatory to reveal tracheal malformations or associatedanomaliesof the ribs. Treatment of myelomeningocele must be multidisciplinary and involve at the same time neurologists, radiologists, neurosurgeons, thoracic surgeons, bioethical experts and take care of the childand also of the family. Some experiences concern the possibility of a in-utero correction of myelomeningocele, in order to avoiding serious and progressive damages to the nervoussystem. Given the improvement of myelomeningocele management, the quality of life is nowadays more acceptable than in the past; however, some severe forms of myelomeningocele cannot still be corrected: in this cases, a "non-interventional" approach may require a form of passive euthanasia that should be discussed and approved with and by parents and Any dissent of the parents must be respected and considered reasonable. The choice of a "non-intervention", which should be guaranteed to all the people capable of self-determination, is not however so immediate and direct in the case of the minor: the dissent expressed on his behalf by the parents or legal representative may be ethically difficult to be accepted.In this case, the best interest of the child must prevail as the goal of any therapeutic choice.
Topics: Child; Humans; Lumbosacral Region; Meningocele; Meningomyelocele; Quality of Life; Spine
PubMed: 34212901
DOI: 10.23750/abm.v92i3.11600 -
Medicine Nov 2022Neurofibromatosis type 1 (NF-1) can manifest with various neurological symptoms. However, sensory ataxia has not been reported.
RATIONALE
Neurofibromatosis type 1 (NF-1) can manifest with various neurological symptoms. However, sensory ataxia has not been reported.
PATIENT CONCERNS
A 44-year-old man with NF-1 presented with several weeks of unsteady gait. He was diagnosed with gastric neuroendocrine tumor with multiple hepatic metastases 6 years ago and received palliative chemotherapy. Neurological examination revealed ataxia veering to the right side with no motor weakness.
DIAGNOSES
Clinical manifestations and electrodiagnostic studies suggested the dysfunction of the thoracic dorsal column (DC). Initial magnetic resonance imaging showed a lateral thoracic meningocele (LTM) located in the right paravertebral area at the T3-T4 vertebral level, but the spinal cord was unremarkable. Gait disturbance worsened after 9 months, and follow-up magnetic resonance imaging showed high signal intensity involving the right DC at the level adjacent to the LTM and spinal cord atrophy distal to the DC lesion. Tests for well-characterized paraneoplastic antibodies were negative. Ultimately, the patient was assumed to have sensory neuronopathy due to compressive damage to the dorsal root ganglia within the intervertebral foramina by LTM.
INTERVENTIONS
Empirical treatment with vitamin B12 supplementation and corticosteroids failed to improve his condition. The patient underwent decompressive laminectomy and excision of the meningocele with dura repair.
OUTCOMES
The patient temporarily improved to walk with assistance postoperatively. However, he developed dyspnea and hypotension 5 weeks later. Carcinoid heart disease confined the patient to the bed. The patient died of pneumonia 3 months after the operation.
LESSONS
This case with NF-1 shows asymmetric sensory ataxia of subacute progression. LTM may contribute to the development of sensory neuronopathy by damaging sensory neurons of the dorsal root ganglia. The comorbidities of the patient, including gastric neuroendocrine tumor and LTM, made it challenging to investigate the pathomechanism.
Topics: Male; Humans; Adult; Neurofibromatosis 1; Meningocele; Spinal Cord; Ataxia; Neuroendocrine Tumors
PubMed: 36397418
DOI: 10.1097/MD.0000000000031718 -
Radiographics : a Review Publication of... 2013
Topics: Cerebrospinal Fluid Rhinorrhea; Encephalocele; Female; Fistula; Humans; Male; Meningocele; Radiography; Skull Base
PubMed: 23607102
DOI: 10.1148/Radiographics.33.2.135002 -
The Pan African Medical Journal 2022
Topics: Humans; Meningocele; Meningomyelocele; Spinal Dysraphism
PubMed: 36405669
DOI: 10.11604/pamj.2022.42.288.36209 -
Neurology India 2022The culprit of trigeminal neuralgia (TGN) may occur at any point between the nerve's root entry zone (REZ) and Meckel's cave. Meckel's cave meningoencephaloceles are... (Review)
Review
BACKGROUND
The culprit of trigeminal neuralgia (TGN) may occur at any point between the nerve's root entry zone (REZ) and Meckel's cave. Meckel's cave meningoencephaloceles are rare middle cranial fossa defects that usually remain asymptomatic but may contain prolapsed trigeminal nerve rootlets and result in TGN. Their management and surgical outcomes remain poorly understood.
OBJECTIVES
To perform a systematic review of clinical presentation and surgical outcomes of middle fossa defects presenting with trigeminal nerve-related symptoms.
MATERIALS AND METHODS
A systematic review was conducted in accordance with the PRISMA guidelines for all reports of middle cranial fossa defects causing trigeminal nerve-related symptoms. The pathophysiology, presentation, surgical management, and outcomes are discussed and illustrated with a case.
RESULTS
Initial search from inception to March 2021 identified 33 articles for screening. After applying inclusion and exclusion criteria, 6 articles were included representing a total of 8 cases in addition to our case (n = 9). All 9 patients were females and 33.3% (n = 3) presented with classic trigeminal neuralgia. "Empty sella" syndrome and radiologic signs of intracranial hypertension were present in 40%-62%. No patient presented with cerebrospinal fluid leak. The preferred treatment modality was surgical with subtemporal extradural repairs using combinations of autologous fat and muscle grafts and synthetic dura. Postoperative outcomes were only available in 55.5% (n = 5) of the cases, and nearly all reported complete symptom resolution, except for one case in which the meningoencephalocele wall was incised, along with trigeminal rootlets adhered to it. Our patient had immediate and durable symptom relief after a 4-year follow-up.
CONCLUSIONS
MEC containing prolapsed trigeminal nerve rootlets can cause typical trigeminal neuralgia from chronic pulsatile stress. This supports the hypothesis that the compressive or demyelinating culprit can locate more ventrally on the course of the trigeminal nerve. Subtemporal extradural surgical repairs can be safe, effective, and durable. Incising the MEC wall should be avoided as it may have trigeminal rootlets adhered to it.
Topics: Cranial Fossa, Middle; Dura Mater; Encephalocele; Female; Humans; Male; Meningocele; Trigeminal Nerve; Trigeminal Neuralgia
PubMed: 35864609
DOI: 10.4103/0028-3886.349629 -
Journal of Radiology Case Reports Aug 2020The sphenoid sinus is an uncommon location for protrusion of a meningocele. When this does occur, it nearly always presents with leakage of cerebrospinal fluid through...
The sphenoid sinus is an uncommon location for protrusion of a meningocele. When this does occur, it nearly always presents with leakage of cerebrospinal fluid through the nasal cavity. We present a case of a 38-year-old female found to have a meningocele protruding into the left sphenoid sinus, who presented with intractable headache but no CSF rhinorrhea. The lesion was discovered on computed tomography angiography, which was performed in order to rule out intracranial pathology as the etiology of her headache. Prior imaging, including pre- and post-contrast MRI, demonstrated the fluid within the sphenoid sinus, but did not reveal the communication through a defect in the base of the skull. Thus, it was assumed to be strictly related to sinus disease in the past. Our case represents a phenomenon whereby meningoceles protruding through the basilar skull into the sphenoid sinus or any other location are potentially misdiagnosed due to poor visualization of the osseous defect and lack of awareness of this entity.
Topics: Adult; Computed Tomography Angiography; Female; Headache; Humans; Magnetic Resonance Imaging; Meningocele; Nausea; Photophobia; Sphenoid Sinus; Vision Disorders; Vomiting
PubMed: 33088417
DOI: 10.3941/jrcr.v14i8.3761 -
The Pan African Medical Journal 2022
Topics: Humans; Spinal Dysraphism; Meningomyelocele
PubMed: 36338552
DOI: 10.11604/pamj.2022.42.258.35894 -
AJNR. American Journal of Neuroradiology 1981
Topics: Cranial Fossa, Posterior; Humans; Infant; Male; Meningitis; Meningocele; Occipital Bone; Radiography
PubMed: 6784543
DOI: No ID Found -
AJNR. American Journal of Neuroradiology Aug 2019The prevalence of patent facial nerve canals and meningoceles along the facial nerve course is unknown. This study aimed to assess the frequency of such findings in... (Review)
Review
BACKGROUND AND PURPOSE
The prevalence of patent facial nerve canals and meningoceles along the facial nerve course is unknown. This study aimed to assess the frequency of such findings in asymptomatic patients.
MATERIALS AND METHODS
A retrospective review was completed of patients with high-resolution MR imaging of the temporal bone whose clinical presentations were unrelated to facial nerve pathology. Facial nerve canals were assessed for the presence of fluid along each segment and meningoceles within either the labyrinthine segment (fluid-filled distention, ≥1.0-mm diameter) or geniculate ganglion fossa (fluid-filled distention, ≥2.0-mm diameter). If a meningocele was noted, images were assessed for signs of CSF leak.
RESULTS
Of 204 patients, 36 (17.6%) had fluid in the labyrinthine segment of the facial nerve canal and 40 (19.6%) had fluid in the geniculate ganglion fossa. Five (2.5%) had meningoceles of the geniculate ganglion fossa; no meningoceles of the labyrinthine segment of the canal were observed. No significant difference was observed in the ages of patients with fluid in the labyrinthine segment of the canal or geniculate ganglion compared with those without fluid ( = .177 and = .896, respectively). Of the patients with a meningocele, one had a partially empty sella and none had imaging evidence of CSF leak or intracranial hypotension.
CONCLUSIONS
Fluid within the labyrinthine and geniculate segments of the facial nerve canal is relatively common. Geniculate ganglion meningoceles are also observed, though less frequently. Such findings should be considered of little clinical importance without radiologic evidence of CSF otorrhea, meningitis, or facial nerve palsy.
Topics: Facial Nerve Diseases; Humans; Magnetic Resonance Imaging; Meningocele; Prevalence; Retrospective Studies; Temporal Bone
PubMed: 31296524
DOI: 10.3174/ajnr.A6133 -
Cureus Jul 2018Herniations of the brain and/or meninges through an opening of the skull often occur through the foramen magnum, e.g., Chiari malformations and encephaloceles. The... (Review)
Review
Herniations of the brain and/or meninges through an opening of the skull often occur through the foramen magnum, e.g., Chiari malformations and encephaloceles. The herniation of brain matter through the cribriform plate is a rare incident and has not been reported frequently. The presence of such an occurrence still requires attention and anatomical understanding. This review will examine the potential causes of cribriform plate herniation and its distinguishability to nasal encephaloceles. The sloping of brain tissue toward potential space/opening in response to elevated pressures in the cranium to accommodate for the added pressure are features seen in herniation. The presence of a pedicle and stalk seen in an encephalocele define its characteristics, which are not visible in a 'classical' herniation. Cerebrospinal fluid (CSF) fistula commonly occurs at the cribriform plate, and due to the structural weakness, a pathway is formed. This is often seen in conjunction with meningoceles. Delineating between herniation and encephaloceles is important for both clinicians and neurosurgeons.
PubMed: 30214849
DOI: 10.7759/cureus.2961