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Parkinsonism & Related Disorders Jan 2021Fragile X Tremor and Ataxia Syndrome is a progressive neurodegenerative disorder that develops in some FMR1 premutation carriers. The objective of this study is to...
INTRODUCTION
Fragile X Tremor and Ataxia Syndrome is a progressive neurodegenerative disorder that develops in some FMR1 premutation carriers. The objective of this study is to characterize how cytokine levels are altered in the FXTAS brain.
METHODS
Fresh frozen cerebellar tissue from FXTAS cases and controls was homogenized and analyzed for 12 different cytokines using a commercially available ELISA panel.
RESULTS
Relative to controls, FXTAS cases showed large and significant increases in the cytokines IL-12 and TNFα. There were large but non-significant increases in the levels of IL-2, IL-8, and IL-10 in FXTAS cases. The cytokines IL-1A, IL-1B, IL-4 IL-6, IL-17A, IFNγ, and GM-CSF were not different between FXTAS and control subjects.
CONCLUSIONS
For the first time, we demonstrate an increase in the pro-inflammatory cytokines TNFα and IL-12 in the FXTAS brain, both of which are implicated in the pathogenesis of Multiple Sclerosis, another neurodegenerative disorder that predominantly consists of white matter disease.
Topics: Aged; Aged, 80 and over; Arachnoiditis; Ataxia; Autopsy; Cerebellum; Female; Fragile X Syndrome; Humans; Interleukin-12 Subunit p35; Male; Middle Aged; Tremor; Tumor Necrosis Factor-alpha; Up-Regulation
PubMed: 33285358
DOI: 10.1016/j.parkreldis.2020.11.026 -
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 -
Journal of Neurosurgery. Case Lessons Apr 2022Hemifacial spasm (HFS) due to an arachnoid cyst at the cerebellopontine angle is rare. Here, the authors reported such a case and analyzed the mechanism of facial nerve...
BACKGROUND
Hemifacial spasm (HFS) due to an arachnoid cyst at the cerebellopontine angle is rare. Here, the authors reported such a case and analyzed the mechanism of facial nerve hyperactivity by reviewing the literature.
OBSERVATIONS
A 40-year-old man presented with right HFS for the past 3 years. Preoperative magnetic resonance imaging revealed a right cerebellopontine angle cystic mass with high intensity on T2-weighted images, low intensity on T1-weighted and diffusion-weighted images, and no contrast effects. Cyst excision and decompression of the facial nerve using a lateral suboccipital approach to monitor abnormal muscle response (AMR) resulted in permanent relief. The cyst was histologically compatible with an arachnoid cyst.
LESSONS
In the present case, when the cyst was dissected, the AMR disappeared and no offending arteries were detected around the root exit zone. Therefore, the cyst itself was responsible for HFS, for which AMR was useful. Limited cases of HFS due to arachnoid cysts without neurovascular compression have been previously reported. The authors suggested that pulsatile compression by the cyst results in facial nerve hyperactivity and secondary HFS.
PubMed: 36303502
DOI: 10.3171/CASE2275 -
Neurologia Medico-chirurgica Apr 2023Cerebrospinal fluid (CSF) production and absorption concept significantly changed in the early 2010s from "third circulation theory" and "classical bulk flow theory" to...
Cerebrospinal fluid (CSF) production and absorption concept significantly changed in the early 2010s from "third circulation theory" and "classical bulk flow theory" to a whole new one as follows: First, CSF is mainly produced from interstitial fluid excreted from the brain parenchyma, and CSF produced from the choroid plexus plays an important role in maintaining brain homeostasis. Second, CSF is not absorbed in the venous sinus via the arachnoid granules, but mainly in the dural lymphatic vessels. Finally, the ventricles and subarachnoid spaces have several compensatory direct CSF pathways at the borders attached to the choroid plexus, e.g., the inferior choroidal point of the choroidal fissure, other than the foramina of Luschka and Magendie. In idiopathic normal pressure hydrocephalus (iNPH), the lateral ventricles and basal cistern are enlarged simultaneously due to the compensatory direct CSF pathways. The average total intracranial CSF volume increased from about 150 mL at 20 years to about 350 mL at 70 years due to the decrease in brain volume with aging and further increased above 400 mL in patients with iNPH. CSF movements are composed of a steady microflow produced by the rhythmic wavy movement of motile cilia on the ventricular surface and dynamic pulsatile flow produced by the brain and cerebral artery pulsation, respiration, and head movement. Pulsatile CSF movements might totally decrease with aging, but it in the ventricles might increase at the foramina of Magendie and Luschka dilation. Aging CSF dynamics are strongly associated with ventricular dilatation in iNPH.
Topics: Humans; Hydrocephalus; Brain; Choroid Plexus; Lateral Ventricles; Subarachnoid Space; Cerebrospinal Fluid; Cerebral Ventricles
PubMed: 36858632
DOI: 10.2176/jns-nmc.2022-0331 -
RoFo : Fortschritte Auf Dem Gebiete Der... Oct 2021Normal pressure hydrocephalus is a disease in elderly patients and one of the most common causes of treatable dementia. It occurs frequently with microangiopathy and...
BACKGROUND
Normal pressure hydrocephalus is a disease in elderly patients and one of the most common causes of treatable dementia. It occurs frequently with microangiopathy and Alzheimer's disease, so that differential diagnosis plays an important role. This is crucially determined by imaging findings. Therapy consists of cerebrospinal fluid drainage through a shunt, which should be performed as early as possible to improve the chances of success.
METHOD
This report is based on a summary of the relevant literature that has been reviewed in PubMed with reference to epidemiology, symptoms, pathophysiology, diagnostics, and therapy. The results were supplemented by the joint guidelines of the German Society of Neurology and the German Society of Neurosurgery.
RESULTS AND CONCLUSION
The understanding of the pathophysiologic changes leading to normal pressure hydrocephalus has expanded significantly in recent years to include concepts explaining relevant comorbidities. Diagnosis is based on radiological and clinical indicators, although accurate differentiation with respect to comorbidities is not always possible. A high response rate to treatment can be achieved by good patient selection. Positive prognostic markers for therapeutic success include Disproportionately Enlarged Subarachnoid Space Hydrocephalus (DESH), short disease duration, predominant gait disturbance, and few comorbidities.
KEY POINTS
· Normal pressure hydrocephalus mainly affects patients older than 65 years of age with high comorbidity rate for microangiopathy and Alzheimer's disease. · Radiologic findings play an important role in the diagnosis and follow-up after shunting. · The earlier a shunt is placed, the better the outcome.
CITATION FORMAT
· Illies T, Eckert B, Kehler U. What Radiologists Should Know About Normal Pressure Hydrocephalus. Fortschr Röntgenstr 2021; 193: 1197 - 1206.
Topics: Aged; Alzheimer Disease; Cerebrospinal Fluid Shunts; Diagnostic Imaging; Humans; Hydrocephalus; Hydrocephalus, Normal Pressure; Radiologists; Subarachnoid Space
PubMed: 34530457
DOI: 10.1055/a-1425-8065 -
Fluids and Barriers of the CNS Mar 2021Studies of mammalian CSF dynamics have been focused on three things: paravascular flow, pressure and pulsatility, and "bulk" flow; and three (respective) potential...
BACKGROUND
Studies of mammalian CSF dynamics have been focused on three things: paravascular flow, pressure and pulsatility, and "bulk" flow; and three (respective) potential motive forces have been identified: vasomotor, cardiac, and ventilatory. There are unresolved questions in each area, and few links between the different areas. The American alligator (Alligator mississippiensis) has pronounced plasticity in its ventilatory and cardiovascular systems. This study was designed to test the hypothesis that the greater cardiovascular and ventilatory plasticity of A. mississippiensis would result in more variation within the CSF dynamics of this species.
METHODS
Pressure transducers were surgically implanted into the cranial subarachnoid space of 12 sub-adult alligators; CSF pressure and pulsatility were monitored along with EKG and the exhalatory gases. In four of the alligators a second pressure transducer was implanted into the spinal subarachnoid space. In five of the alligators the CSF was labeled with artificial microspheres and Doppler ultrasonography used to quantify aspects of the spinal CSF flow.
RESULTS
Both temporal and frequency analyses of the CSF pulsations showed highly variable contributions of both the cardiac and ventilatory cycles. Unlike the mammalian condition, the CSF pressure pulsations in the alligator are often of long (~ 3 s) duration, and similar duration CSF unidirectional flow pulses were recorded along the spinal cord. Reduction of the duration of the CSF pulsations, as during tachycardia, can lead to a "summation" of the pulsations. There appears to be a minimum duration (~ 1 s) of isolated CSF pulsations. Simultaneous recordings of cranial and spinal CSF pressures reveal a 200 ms delay in the propagation of the pressure pulse from the cranium to the vertebral canal.
CONCLUSIONS
Most of the CSF flow dynamics recorded from the alligators, are similar to what has been reported from studies of the human CSF. It is hypothesized that the link between ventilatory mechanics and CSF pulsations in the alligator is mediated by displacement of the spinal dura. The results of the study suggest that understanding the CSF dynamics of Alligator may provide unique insights into the evolutionary origins and functional regulation of the human CSF dynamics.
Topics: Alligators and Crocodiles; Animals; Cardiovascular Physiological Phenomena; Cerebrospinal Fluid; Hydrodynamics; Respiratory Physiological Phenomena; Subarachnoid Space; Time Factors
PubMed: 33712028
DOI: 10.1186/s12987-021-00248-1 -
Asian Journal of Surgery Jun 2023
Review
Topics: Humans; Spinal Cord Diseases; Arachnoid Cysts; Magnetic Resonance Imaging
PubMed: 36641278
DOI: 10.1016/j.asjsur.2022.12.110 -
Surgical Neurology International 2021Intracranial arachnoid cysts (AC) are benign, cerebrospinal fluid filled spaces within the arachnoid layer of the meninges. Neurosurgical intervention in children and... (Review)
Review
BACKGROUND
Intracranial arachnoid cysts (AC) are benign, cerebrospinal fluid filled spaces within the arachnoid layer of the meninges. Neurosurgical intervention in children and young adults has been extensively studied, but the optimal strategy in the elderly remains unclear. Therefore, we performed a single center retrospective study combined with a systematic review of the literature to compare cystoperitoneal (CP) shunting with other surgical approaches in the elderly cohort.
METHODS
Retrospective neurosurgical database search between January 2005 and December 2018, and systematic review of the literature using PRISMA guidelines were performed. Inclusion criteria: Age 60 years or older, radiological diagnosis of intracranial AC, neurosurgical intervention, and neuroradiological (NOG score)/clinical outcome (COG score). Data from both sources were pooled and statistically analyzed.
RESULTS
Our literature search yielded 12 studies (34 patients), which were pooled with our institutional data (13 patients). CP shunts (7 patients; 15%), cyst fenestration (28 patients; 60%) and cyst marsupialisation/resection (10 patients; 21%) were the commonest approaches. Average duration of follow-up was 23.6, 26.9, and 9.5 months for each approach, respectively. There was no statistically significant association between choice of surgical intervention and NOG score ( = 0.417), COG score ( = 0.601), or complication rate ( = 0.955). However, CP shunting had the lowest complication rate, with only one patient developing chronic subdural haematoma.
CONCLUSION
CP shunting is a safe and effective surgical treatment strategy for ACs in the elderly. It has similar clinical and radiological outcomes but superior risk profile when compared with other approaches. We advocate CP shunting as first line neurosurgical intervention for the management of intracranial ACs in the elderly.
PubMed: 34992940
DOI: 10.25259/SNI_463_2021 -
Journal of Stroke and Cerebrovascular... Sep 2021the prevalence of intracranial aneurysms and arachnoid cysts is higher in patients with autosomal dominant polycystic kidney disease (ADPKD) than in the general...
OBJECTIVES
the prevalence of intracranial aneurysms and arachnoid cysts is higher in patients with autosomal dominant polycystic kidney disease (ADPKD) than in the general population. A genotype correlation was reported for intracranial aneurysms, but it is unclear for arachnoid cysts. Therefore, the genotype correlation with intracranial aneurysms and arachnoid cysts was investigated in ADPKD.
MATERIALS AND METHODS
intracranial aneurysms and arachnoid cysts were screened by magnetic resonance imaging (MRI), and PKD genotypes were examined using next-generation sequencing for 169 patients with ADPKD.
RESULTS
PKD1-, PKD2- and no-mutation were identified in 137, 24 and 8 patients, respectively. Intracranial aneurysms and arachnoid cysts were found in 34 and 25 patients, respectively, with no significant difference in frequency. Genotype, sex, estimated glomerular filtration rate and age at ADPKD diagnosis significantly affected the age at brain MRI. The proportional hazard risk analyzed using the age at brain MRI adjusted by these four variables was 5.0-times higher in the PKD1 group than in the PKD2 group for arachnoid cysts (P = 0.0357), but it was not different for intracranial aneurysms (P = 0.1605). Arachnoid cysts were diagnosed earlier in the PKD1 group than in the PKD2 group (54.8 vs 67.7 years, P = 0.0231), but no difference was found for intracranial aneurysms (P = 0.4738) by Kaplan-Meier analysis.
CONCLUSIONS
this study demonstrated the correlation between arachnoid cysts and PKD1 mutation. The reported association of arachnoid cysts with advanced renal disease may be due to the common correlation of these factors with PKD1 mutation.
Topics: Adult; Aged; Arachnoid Cysts; Cerebral Angiography; DNA Mutational Analysis; Female; Genetic Predisposition to Disease; High-Throughput Nucleotide Sequencing; Humans; Intracranial Aneurysm; Magnetic Resonance Angiography; Male; Middle Aged; Mutation; Phenotype; Polycystic Kidney, Autosomal Dominant; Risk Assessment; Risk Factors; TRPP Cation Channels
PubMed: 34175641
DOI: 10.1016/j.jstrokecerebrovasdis.2021.105943 -
NMC Case Report Journal 2022Arachnoiditis ossificans (AO) is a rare disease, wherein ossified lesions in the subarachnoid space obstruct the flow of spinal fluid or compress the spinal cord,...
Arachnoiditis ossificans (AO) is a rare disease, wherein ossified lesions in the subarachnoid space obstruct the flow of spinal fluid or compress the spinal cord, thereby causing myelopathy. Here we describe a rare case of AO and discuss the diagnosis and treatment strategies for this disease. A 66-year-old man with a history of subarachnoid hemorrhage presented with gait disturbance and dysuria for 7 months. Spinal magnetic resonance imaging and computed tomography (CT) myelography showed syringomyelia at the T5-T8 level and dorsally tethered spinal cord at the T8-T10 level. Preoperative noncontrast CT was not performed. The patient was diagnosed with adhesive arachnoiditis and underwent arachnoidolysis. However, intraoperative findings showed the presence of ossification lesions on the dorsal surface of the spinal cord, and intraoperative ultrasound (IOU) showed a hyperintense lesion with acoustic shadowing on the dorsal surface of the spinal cord, with limited visibility of the spinal cord. After removal of the lesions, IOU showed untethered and well-decompressed spinal cord and restoration of cerebrospinal fluid pulsation. Based on these findings, the patient was finally diagnosed with AO, which is an extremely rare disease, with an unknown frequency of occurrence. Therefore, all patients with adhesive spinal arachnoiditis require a preoperative noncontrast CT scan to evaluate for ossification lesions. In this case, we were fortunate to be able to treat AO with IOU, which demonstrated specific findings.
PubMed: 36263188
DOI: 10.2176/jns-nmc.2022-0036