-
Nature and Science of Sleep 2023The pathogenesis of different narcolepsy phenotypes remains unclear. In rare cases, narcolepsy can be attributable to secondary brain pathologies affecting the midbrain....
OBJECTIVE
The pathogenesis of different narcolepsy phenotypes remains unclear. In rare cases, narcolepsy can be attributable to secondary brain pathologies affecting the midbrain. These cases may elucidate the pathological background and the treatment of narcolepsy, but are often limited by poor objective symptom characterization and effects of therapeutic intervention, especially by modern diagnostic standards.
METHODS
A young adult presented with excessive daytime sleepiness (EDS) that was refractory to classic narcolepsy medication. Diagnosis of narcolepsy was made based on the pathologically shortened sleep latencies in polysomnography and Multiple Sleep Latency Test (MSLT), together with confirmed sleep-onset REM-sleep (SOREM). Preserved hypocretin levels in cerebrospinal fluid, together with the absence of cataplectic events confirmed the diagnosis of narcolepsy type II. MRI revealed a large arachnoid cyst with compression of the midbrain.
RESULTS
Six months after fenestration of the cyst, the patient's EDS had vastly improved. No further SOREM was observed, and polysomnographic and MSLT sleep latencies normalized. No further drug treatment was required.
CONCLUSION
Symptomatic narcolepsy due to space-occupying lesions in the mesencephalon comprises a unique curative treatment option. Here, surgical intervention offers an effective curative therapeutic approach. However, differential diagnosis of symptomatic narcolepsy requires special consideration.
PubMed: 37964993
DOI: 10.2147/NSS.S407808 -
Journal of Neurosurgery. Case Lessons Nov 2023Selective dorsal rhizotomy (SDR) can improve the spastic gait of carefully selected patients with cerebral palsy. Spinal arachnoid cysts are a rare pathology that can...
BACKGROUND
Selective dorsal rhizotomy (SDR) can improve the spastic gait of carefully selected patients with cerebral palsy. Spinal arachnoid cysts are a rare pathology that can also cause spastic gait secondary to spinal cord compression.
OBSERVATIONS
The authors present an interesting case of a child with cerebral palsy and spastic diplegia. He was evaluated by a multidisciplinary team and determined to be a good candidate for SDR. Preoperative evaluation included magnetic resonance imaging (MRI) of the spine, which identified an arachnoid cyst causing spinal cord compression. The cyst was surgically fenestrated, which provided some gait improvement. After recovering from cyst fenestration surgery, the patient underwent SDR providing further gait improvement.
LESSONS
SDR can be beneficial for some patients with spastic diplegia. Most guidelines do not include spinal MRI in the preoperative evaluation for SDR. However, spinal MRI can be beneficial for surgical planning by localizing the level of the conus. It may also identify additional spinal pathology that is contributing to the patient's spasticity. In rare cases, such as this one, patients may benefit from staged surgery to address structural causes of spastic gait prior to proceeding with SDR.
PubMed: 37956422
DOI: 10.3171/CASE23478 -
BMC Pediatrics Nov 2023To assess the cognitive function changes and brain network neuroplasticity in school-age children having large (diameter > 5 cm) left middle fossa arachnoid cyst (MFACs).
BACKGROUND
To assess the cognitive function changes and brain network neuroplasticity in school-age children having large (diameter > 5 cm) left middle fossa arachnoid cyst (MFACs).
METHODS
Eleven patients and 22 normal controls (NC) between 6 and 14 years of age were included. The CNS Vital Signs (CNS VS) were administered for cognitive assessment. The differences of cognitive data and functional connectivity (FC) in resting-state functional magnetic resonance imaging (rs-fMRI) were compared between the patient group and the NC group. The correlations between the altered FC and cognitive data in the patient group were assessed.
RESULTS
Patient group had significantly poorer attention (including Complex Attention, Sustained Attention, Simple Attention, Cognitive Flexibility, and Executive Function) and memory function (Visual Memory and Working Memory) than the NC group (uncorrected p-value, p-unc < 0.05). Whole-brain local correlation (LCOR) analysis showed an extensively lower LCOR in the patient group (voxel threshold p-unc < 0.001, cluster-size threshold of false discovery rate adjusted p (p-FDR) < 0.001). Functional connectivity (FC) analysis showed that bilateral frontal and temporal lobes connectivity in the patient group was significantly lower than the NC group (p-FDR < 0.05). Seed-based FC analysis indicated that there was altered FC between the right temporal lobe and the left temporal-parietal/temporal-occipital area (p-FDR < 0.05). In the patient group, most of the altered FC had a negative correlation to the cognitive score, while the FC in the right temporal lobe-left temporal-occipital area positively correlated to Verbal/Visual Memory (r = 0.41-0.60, p-FDR < 0.05). In correlation analysis between clinical data and cognitive score, the only significant result was a low correlation between cyst size and Reaction Time (-0.30--0.36, P-FDR < 0.05).
CONCLUSIONS
School-aged children with large left MFAC showed significantly lower cognitive performance primarily in attention and memory domains. Distinct from neuroplasticity in a unilateral brain lesion, compensation in the healthy hemisphere in MFAC patients was sparse.
Topics: Humans; Child; Arachnoid Cysts; Magnetic Resonance Imaging; Brain; Memory, Short-Term; Cognition
PubMed: 37919687
DOI: 10.1186/s12887-023-04148-1 -
Annals of Medicine and Surgery (2012) Nov 2023Arachnoid cyst (AC) is the most frequently founded lesion reported in adolescent patients suffering from chronic subdural haematoma (CSDH). Association between these two...
INTRODUCTION AND IMPORTANCE
Arachnoid cyst (AC) is the most frequently founded lesion reported in adolescent patients suffering from chronic subdural haematoma (CSDH). Association between these two distinct clinical entities is known for a long time. However, in the literature there are numerous clinical cases that reflect this relationship and few large series that analyze them in detail. Paediatric population is more rarely affected with this association.
CASE PRESENTATION
The authors report the case of AC of incidental discovery complicated with CSDH in 15-year-old male recreational boxer presented with progressive onset of holocranial drug-resistant throbbing headache with favourable clinical course after conservative treatment.
CLINICAL DISCUSSION
ACs are a well-known predisposing cause for CSDH after head trauma. In all cases of CSDH in children, the diagnosis of ruptured AC should be considered. Rupture may be spontaneous or following even mild head trauma with rupture of bridging veins causing subdural bleeding as it was seen in our patient who was practicing a full-contact free-sparring sport like boxing sustaining repeated and direct mild head traumas. MRI is recommended to detect small cysts in adolescents with CSDH. The management of these patients remains controversial.
CONCLUSION
This is a rare reported case of CSDH complicating an AC in a juvenile recreational male boxer. This association remains extremely rare in children and adolescents, as evidenced by the rare cases reported in the literature.
PubMed: 37915627
DOI: 10.1097/MS9.0000000000001338 -
Cureus Sep 2023Spinal extradural arachnoid cysts are an uncommon condition and their exact causative triggers are still unclear. They appear to be extradural arachnoid outpouchings...
Spinal extradural arachnoid cysts are an uncommon condition and their exact causative triggers are still unclear. They appear to be extradural arachnoid outpouchings that connect to the intraspinal subarachnoid region via a little dura defect. These cysts are most commonly seen in the thoracic spine, followed by the lumbosacral junction. Compression of the spinal cord or nerve roots leads to the development of the symptoms. The most morbid symptom associated with these compressing extradural cysts is paresthesia. Numerous theories have been proposed about their origins, and the related conditions include spinal trauma, spina bifida, and the lymphedema-distichiasis syndrome. Their position in the spine influences the symptoms manifested. The diagnosis is made via MRI. Surgery is only performed on individuals with neurological impairment, and treatment is based on the clinical presentation. The preferred course of therapy is total surgical excision. We present a case that involves the successful surgical removal of an extradural spinal arachnoid cyst in a 10-year-old girl. Given the rarity of this pathology, its wide array of presenting symptoms, and the successful therapeutic protocol that was followed in this particular case, we believe this article shall prove beneficial to the medical fraternity.
PubMed: 37900454
DOI: 10.7759/cureus.46047 -
The Kaohsiung Journal of Medical... Jan 2024
Review
Topics: Adult; Humans; Cerebellopontine Angle; Arachnoid Cysts; Magnetic Resonance Imaging
PubMed: 37873877
DOI: 10.1002/kjm2.12772 -
Cureus Sep 2023Spinal subarachnoid hemorrhage (SSAH) is a rare condition that can cause spinal cord or nerve root compression and permanent neurologic damage. The reported etiologies...
Spinal subarachnoid hemorrhage (SSAH) is a rare condition that can cause spinal cord or nerve root compression and permanent neurologic damage. The reported etiologies include trauma, vascular malformations or aneurysms, coagulopathies, neoplasms, autoimmune disease, and spontaneous hemorrhage. If there is evidence of neurologic deterioration, it is commonly managed as a surgical emergency, but cases of conservative management have also been reported. In this case series, we present three patients who suffered from SSAH. The first was a spontaneous cervical SSAH that occurred following cardiac catheterization, the second was a spontaneous thoracolumbar SSAH in a patient with a known history of coagulopathy, and the third was a thoracolumbar SSAH that was caused by a dural arteriovenous fistula (dAVF). All three patients exhibited neurologic deficits and thus underwent emergent decompression and hematoma evacuation. The patient with the dAVF also required open ligation of the fistula. Following surgical intervention, all three patients regained at least partial neurologic function, but one patient developed symptomatic arachnoid cysts that required further intervention. The presented case series highlights the importance and time-sensitivity of surgical decompression in patients experiencing neurologic deficits from SSAH. These cases underscore the urgency of timely neurosurgical intervention to mitigate neurologic impairment and add insights to the existing literature on this rare condition.
PubMed: 37868412
DOI: 10.7759/cureus.45627 -
BMC Pregnancy and Childbirth Oct 2023Fetal cerebral aneurysm other than aneurysm of vein of Galen aneurysmal malformation (VGAM) is extremely rare. This report describes prenatal features of aneurysm of the...
BACKGROUND
Fetal cerebral aneurysm other than aneurysm of vein of Galen aneurysmal malformation (VGAM) is extremely rare. This report describes prenatal features of aneurysm of the posterior cerebral artery (APCA) with rapid progression and its natural intrauterine course of the disease, which has never been reported.
CASE PRESENTATION
This is the first report of prenatal features of APCA, detected at 34-36 weeks of gestation, simulating choroid plexus cyst or arachnoid cyst. The diagnosis was based on color flow ultrasound with tracing along the course of cerebral arteries. Also, rendered 3D color flow ultrasound was helpful in demonstrating course of the vessels feeding the aneurysm and supporting the diagnosis. The aneurysm showed nature of rapidly progressive changes, leading to leakage resulting in intracerebral and intraventricular hemorrhage as well as high output state associated with anemia. Prenatal diagnosis and management are very challenging. This case ended up with planned delivery at 37 weeks, giving birth to a surviving male newborn, weighing 2600 g. The neonatal CT brain scans and CTA confirmed the prenatal findings. The prognosis was relatively poor because of extensive intracerebral hemorrhage with severe hydrocephalus and brain midline shift. The couple opted for neonatal palliative care without neurosurgical correction.
CONCLUSION
This study demonstrate that the most important tool for prenatal diagnosis is color Doppler ultrasound, which will demonstrate turbulent blood flow. Three-dimension color Doppler ultrasound is helpful in supporting the diagnosis. The case presented here suggests that the disease has a natural course of rapid progression and massive brain destruction or high output congestive heart failure can be expected.
Topics: Infant, Newborn; Female; Pregnancy; Male; Humans; Posterior Cerebral Artery; Ultrasonography, Prenatal; Aneurysm; Prenatal Diagnosis; Cysts
PubMed: 37848838
DOI: 10.1186/s12884-023-06056-9 -
Frontiers in Neuroscience 2023Xanthogranulomas are considered rare tumors, with their sellar and non-sellar frequency ranging from 1.6 to 7% among intracranial lesions, and described as a separate...
Xanthogranulomas are considered rare tumors, with their sellar and non-sellar frequency ranging from 1.6 to 7% among intracranial lesions, and described as a separate entity by the World Health Organization in 2000. The diagnosis of sellar xanthogranulomas is challenging, given their uncertain origin and clinical course. In addition, the limited reporting of sellar xanthogranuloma cases and the absence of characteristic images make these entities difficult to distinguish from other cystic lesions of the sellar region, such as adamantinomatous craniopharyngiomas, Rathke's cleft cysts, pituitary tumors, arachnoid cysts, epidermoid cysts, and dermoid cysts. Here, we describe the clinical presentation, radiological findings, immunohistochemical/histopathological analysis, and the ultrastructural examination by transmission electron microscopy of five sellar xanthogranulomas cases reported in two care centers in Cordoba, Argentina. Two males and three females between 37 and 73 years of age (average 51.8 years) presented with persistent headaches, generalized endocrine defects, and visual problems. MRI revealed cystic formations in the sellar region, which usually projected into adjacent tissues such as the suprasellar region or cavernous sinuses, and compressed other structures such as the optic chiasm, pituitary gland, and cranial nerves. All patients underwent surgical intervention to remove the tumor tissue. The histopathological analysis of the samples showed cellular tissue with a xanthogranulomatous appearance, inflammatory cellular infiltrate (mainly lymphocytes and macrophages), fibroblasts, abundant collagen fibers, and hemorrhages. An ultrastructural analysis helped to identify cellular infiltrates and granules resulting from tumor cell activity. The data support the hypothesis that sellar xanthogranulomas could occur as an inflammatory reaction secondary to the rupture and hemorrhage of a previous cystic process, thereby generating an expansion of the tumor body toward adjacent tissues. The information obtained from these cases contributes to the current knowledge about this disease's origin and clinical and histological evolution. However, the scarcity of patients and the observed phenotypic heterogeneity make its diagnosis still challenging. Undoubtedly, more investigations are needed to provide additional information in order to be able to achieve a more accurate diagnosis and effective treatment of this rare disease.
PubMed: 37811322
DOI: 10.3389/fnins.2023.1227144 -
Surgical Neurology International 2023Neuroglial cysts (NCs) are uncommon benign cysts covered by an epithelial layer, accounting for <1% of all intracranial cysts. The optimal management approach for these...
BACKGROUND
Neuroglial cysts (NCs) are uncommon benign cysts covered by an epithelial layer, accounting for <1% of all intracranial cysts. The optimal management approach for these cysts remains a subject of debate. Given their rarity, management principles used for arachnoid cysts can be applied to NCs.
CASE DESCRIPTION
We present a case of a 35-year-old male without prior medical history, who presented to the neurosurgery clinic with complaints of absence seizures. A neurological examination revealed subtle weakness in the left upper limb. Brain magnetic resonance imaging demonstrated a large cystic lesion in the posterior frontal and anterior parietal lobes of the right hemisphere. The patient underwent an awake craniotomy, during which a cystoventricular fenestration was performed on the motor cortex cyst. Histopathological examination confirmed the diagnosis of NC. At the 4-month follow-up, the patient experienced complete recovery, with normal strength (5/5) in all limbs and absence of seizure remission.
CONCLUSION
This case highlights the successful use of awake craniotomy for the fenestration of an NC in the motor cortex. Given the rarity of NCs, there is no consensus on the optimal treatment strategy. However, in this particular case, the patient achieved complete recovery without any new neurological deficits following the procedure.
PubMed: 37810319
DOI: 10.25259/SNI_477_2023