ABSTRACT
BACKGROUND: To investigate the association between white matter changes and ventricular expansion in idiopathic normal pressure hydrocephalus (iNPH) based on diffusion spectrum imaging (DSI). METHODS: We included 32 patients with iNPH who underwent DSI using a 3T MRI scanner. The lateral ventricles were manually segmented, and ventricular volumes were measured. Two methods were utilised in the study: manual region-of-interest (ROI) delineation and tract diffusion profile analysis. General fractional anisotropy (GFA) and fractional anisotropy (FA) were extracted in different white matter regions, including the bilateral internal capsule (anterior and posterior limbs) and corpus callosum (body, genu, and splenium) with manual ROI delineation. The 18 main tracts in the brain of each patient were extracted; the diffusion metrics of 100 equidistant nodes on each fibre were calculated, and Spearman's correlation coefficient was used to determine the correlation between diffusion measures and ventricular volume of iNPH patients. RESULTS: The GFA and FA of all ROI showed no significant correlation with lateral ventricular volume. However, in the tract diffusion profile analysis, lateral ventricular volume was positively correlated with part of the cingulum bundle, left corticospinal tract, and bilateral thalamic radiation posterior, whereas it was negatively correlated with the bilateral cingulum parahippocampal (all p < 0.05). CONCLUSIONS: The effect of ventricular enlargement in iNPH on some white matter fibre tracts around the ventricles was limited and polarizing, and most white matter fibre tract integrity changes were not associated with ventricular enlargement; this reflects that multiple pathological mechanisms may have been combined to cause white matter alterations in iNPH.
Subject(s)
Hydrocephalus, Normal Pressure , White Matter , Humans , Hydrocephalus, Normal Pressure/diagnostic imaging , Hydrocephalus, Normal Pressure/pathology , Male , Female , Aged , White Matter/diagnostic imaging , White Matter/pathology , Aged, 80 and over , Diffusion Tensor Imaging/methods , Middle Aged , Diffusion Magnetic Resonance Imaging/methods , Cerebral Ventricles/diagnostic imaging , Cerebral Ventricles/pathology , AnisotropyABSTRACT
AIM: The aim of this study was to identify preoperative magnetic resonance imaging (MRI) findings that can predict the shunt responsiveness in idiopathic normal-pressure hydrocephalus (iNPH) patients and to investigate postoperative outcome and complications. MATERIALS AND METHODS: A total of 192 patients with iNPH who underwent shunt at our hospital between 2000 and 2021 were included to investigate complications. Of these, after exclusion, 127 (1-month postoperative follow-up) and 77 (1-year postoperative follow-up) patients were evaluated. The preoperative MRI features (the presence of tightness of the high-convexity subarachnoid space, Sylvian fissure enlargement, Evans' index, and callosal angle) of the shunt-response and nonresponse groups were compared, and a systematic review was conducted to evaluate whether preoperative MRI findings could predict shunt response. RESULTS: Postoperative complications within one month after surgery were observed in 6.8% (13/192), and the most common complication was hemorrhage. Changes in corpus callosum were observed in 4.2% (8/192). The shunt-response rates were 83.5% (106/127) in the 1-month follow-up group and 70.1% (54/77) in 1-year follow-up group. In the logistic regression analysis, only Evans' index measuring >0.4 had a significant negative relationship with shunt response at 1-month follow-up; however, no significant relationship was observed at 1-year follow-up. According to our systematic review, it is still controversial whether preoperative MRI findings could predict shunt response. CONCLUSION: Evans' index measure of >0.4 had a significant relationship with the shunt response in the 1-month follow-up group. In systematic reviews, there is ongoing debate about whether preoperative MRI findings can accurately predict responses to shunt surgery. Postoperative corpus callosal change was observed in 4.2% of iNPH patients.
Subject(s)
Hydrocephalus, Normal Pressure , Magnetic Resonance Imaging , Postoperative Complications , Humans , Hydrocephalus, Normal Pressure/surgery , Hydrocephalus, Normal Pressure/diagnostic imaging , Female , Male , Magnetic Resonance Imaging/methods , Aged , Postoperative Complications/diagnostic imaging , Treatment Outcome , Cerebrospinal Fluid Shunts , Retrospective Studies , Preoperative Care/methods , Aged, 80 and over , Middle AgedABSTRACT
PURPOSE: The potential relationship between mastication ability and cognitive function in idiopathic normal pressure hydrocephalus (iNPH) patients is unclear. This report investigated the association between mastication and cognitive function in iNPH patients using the gray level of the co-occurrence matrix on the lateral pterygoid muscle. METHODS: We analyzed data from 96 unoperated iNPH patients who underwent magnetic resonance imaging (MRI) between December 2016 and February 2023. Radiomic features were extracted from T2 MRI scans of the lateral pterygoid muscle, and muscle texture parameters were correlated with the iNPH grading scale. Subgroup analysis compared the texture parameters of patients with normal cognitive function with those of patients with cognitive impairment. RESULTS: The mini-mental state examination score correlated positively with the angular second moment (P < 0.05) and negatively with entropy (P < 0.05). The dementia scale (Eide's classification) correlated negatively with gray values (P < 0.05). Gray values were higher in the cognitive impairment group (64.7 ± 16.6) when compared with the non-cognitive impairment group (57.4 ± 13.3) (P = 0.005). Entropy was higher in the cognitive impairment group (8.2 ± 0.3) than in the non-cognitive impairment group (8.0 ± 0.3) (P < 0.001). The area under the receiver operating characteristic curve was 0.681 (P = 0.003) and 0.701 (P < 0.001) for gray value and entropy, respectively. CONCLUSION: Our findings suggest an association between heterogeneity of mastication and impaired cognitive function in iNPH patients and highlight muscle texture analysis as a potential tool for predicting cognitive impairment in these patients.
Subject(s)
Cognition , Cognitive Dysfunction , Hydrocephalus, Normal Pressure , Magnetic Resonance Imaging , Pterygoid Muscles , Humans , Hydrocephalus, Normal Pressure/diagnostic imaging , Hydrocephalus, Normal Pressure/surgery , Hydrocephalus, Normal Pressure/psychology , Hydrocephalus, Normal Pressure/physiopathology , Male , Female , Aged , Aged, 80 and over , Cognition/physiology , Cognitive Dysfunction/psychology , Cognitive Dysfunction/physiopathology , Cognitive Dysfunction/diagnostic imaging , Pterygoid Muscles/diagnostic imaging , Pterygoid Muscles/pathology , Mastication/physiologyABSTRACT
While the cause of altered mentation in the elderly may be multifactorial, infectious etiologies may be missed. This case report aims to detail an account of a patient with dementia, found to have Lyme meningitis in the setting of a normal pressure hydrocephalus (NPH). The patient smelled of urine and presented with ambulatory dysfunction, fitting the "wet, wacky, and wobbly" triad of NPH while also having subjective chills and leukocytosis. Non-contrast brain CT scan showed dilated ventricles. Cerebrospinal fluid (CSF) studies suggested aseptic meningitis. Serum studies using a modified two-tiered algorithm confirmed the diagnosis of Lyme disease. Treatment of the underlying condition with a prolonged course of doxycycline improved symptoms and clinical course. Review of the literature on the association between Lyme meningitis and NPH reveals that few cases of Lyme-related NPH have been reported worldwide and further research into the pathophysiology, diagnostic approach, treatment modalities, and management of NPH secondary to Lyme meningitis may be warranted.
Subject(s)
Dementia , Hydrocephalus, Normal Pressure , Lyme Disease , Meningitis , Humans , Aged , Hydrocephalus, Normal Pressure/diagnosis , Hydrocephalus, Normal Pressure/diagnostic imaging , Brain , Lyme Disease/complications , Lyme Disease/diagnosis , Meningitis/complications , Meningitis/diagnosis , Dementia/complications , Dementia/diagnosisABSTRACT
Idiopathic normal pressure hydrocephalus (iNPH), a syndrome characterized by cognitive impairment, gait abnormalities, and urinary incontinence attributable to cerebrospinal fluid circulatory insufficiency, is associated with an obscure etiology. Several methodologies and imaging analysis techniques, including visualization of cerebral hemodynamics and diffusion tensor imaging have emerged in recent years to determine functional alterations in cases of iNPH. These advancements have led to more comprehensive understanding of the pathophysiological mechanisms underlying iNPH. In this article, we discuss the historical evolution of imaging modalities for iNPH and contemporaneous imaging techniques designed to acquire deeper insight into this disease.
Subject(s)
Hydrocephalus, Normal Pressure , Humans , Hydrocephalus, Normal Pressure/diagnostic imaging , Hydrocephalus, Normal Pressure/complications , Diffusion Tensor Imaging/methodsABSTRACT
INTRODUCTION: Enlarged perivascular spaces (ePVS) may be an indicator of glymphatic dysfunction. Limited studies have evaluated the role of ePVS in idiopathic normal pressure hydrocephalus (iNPH). We aimed to characterize the distribution and number of ePVS in iNPH compared to controls. METHODS: Thirty-eight patients with iNPH and a pre-shunt MRI were identified through clinical practice. Age- and sex-matched controls who had negative MRIs screening for intracranial metastases were identified through a medical record linkage system. The number of ePVS were counted in the basal nuclei (BN) and centrum semiovale (CS) using the Wardlaw method blinded to clinical diagnosis. Imaging features of disproportionately enlarged subarachnoid space hydrocephalus (DESH), callosal angle, Fazekas white matter hyperintensity (WMH) grade, and the presence of microbleeds and lacunes were also evaluated. RESULTS: Both iNPH patients and controls had a mean age of 74 ± 7 years and were 34% female with equal distributions of hypertension, dyslipidemia, diabetes, stroke, and history of smoking. There were fewer ePVS in the CS of patients with iNPH compared to controls (12.66 vs. 20.39, p < 0.001) but the same in the BN (8.95 vs. 11.11, p = 0.08). This remained significant in models accounting for vascular risk factors (p = 0.002) and MRI features of DESH and WMH grade (p = 0.03). CONCLUSIONS: Fewer centrum semiovale ePVS may be a biomarker for iNPH. This pattern may be caused by mechanical obstruction due to upward displacement of the brain leading to reduced glymphatic clearance.
Subject(s)
Hydrocephalus, Normal Pressure , Nervous System Malformations , Humans , Female , Aged , Aged, 80 and over , Male , Hydrocephalus, Normal Pressure/diagnostic imaging , Magnetic Resonance Imaging , Brain , Corpus Callosum , Basal GangliaABSTRACT
BACKGROUND AND OBJECTIVES: Very divergent prevalence rates for idiopathic normal pressure hydrocephalus (iNPH) are reported, probably due to differences in study sample selection and diagnostic criteria. This MRI-based study aimed to determine the prevalence of iNPH and iNPH-specific radiologic changes and their association with clinical symptoms in a large, 70-year-old population-based cohort (Gothenburg H70). METHODS: In this cross-sectional study, disturbances in gait and balance, cognition, and urinary continence were assessed using clinical examination and self-report. MRI was evaluated for iNPH-specific imaging markers. iNPH was diagnosed according to International Guidelines (I.G.). Based on radiologic findings, participants were allocated to 1 of 4 groups: (A) Evans index (EI) ≤0.3 (reference), (B) EI >0.3 without other iNPH-typical radiologic findings, (C) radiologically probable iNPH according to I.G., and (D) radiologically holistically probable (h-probable) iNPH fulfilling radiologic criteria according to I.G. plus highly iNPH-specific changes according to an experienced neuroradiologist. RESULTS: The Gothenburg H70 Studies include 791 individuals (377 men, 414 women) born in 1944 who underwent brain MRI. The prevalence of iNPH was 1.5% (2.1% for men, 0.96% for women) according to I.G. Ninety participants (11%) had EI >0.3 without other iNPH-typical radiologic findings, 29 (3.7%) fulfilled the I.G. radiologic probable iNPH criteria alone, and 11 (1.4%) were classified as radiologically h-probable iNPH. Forty participants (5.1%) had I.G. radiologic features of iNPH (70% men vs 30% women, p = 0.005). Gait disturbances were more common in participants with EI >0.3 without other radiologic iNPH features (B) (33%) compared with the reference group (A) (19%) (p = 0.006). All clinical symptoms were more common in participants with I.G. radiologic features of iNPH (C + D) than they were in the reference group (A) (p < 0.03). DISCUSSION: The iNPH prevalence of 1.5% among 70-year-olds, which is considerably higher than earlier reported in this age group, suggests that iNPH may be more common than previously assumed. This is supported by the 5.1% total prevalence of imaging signs of iNPH. Ventriculomegaly without other iNPH-typical radiologic findings may be an early sign of developing iNPH in some patients.
Subject(s)
Hydrocephalus, Normal Pressure , Male , Humans , Female , Aged, 80 and over , Aged , Sweden/epidemiology , Cross-Sectional Studies , Hydrocephalus, Normal Pressure/diagnostic imaging , Hydrocephalus, Normal Pressure/epidemiology , Prevalence , GaitABSTRACT
OBJECTIVE: The aim of this study is to investigate long-term prognostic factors and clinical outcomes in patients with idiopathic normal pressure hydrocephalus (iNPH) treated with ventriculo-peritoneal shunt (VPS). METHODS: This single-center retrospective observational study of prospectively collected data included patients with probable iNPH treated with VPS surgery. All patients underwent complete preoperative assessment, including past medical history and neurological examination, dynamic cerebrospinal fluid (CSF) flow brain magnetic resonance imaging (MRI), and preoperative CSF samplings. NPH-consistent brain MRI findings and favorable responses to CSF subtraction tests were the main factors considered for VPS surgery eligibility. All patients were subsequently followed up every six to twelve months (mean follow-up time 40 months, minimum to maximum interval 6-150 months). RESULTS: A total of 238 patients with a diagnosis of probable iNPH treated with VPS were enrolled. Age, comorbidities, and response to CSF samplings were not significantly associated with a better long-term outcome after VPS surgery. The results of our retrospective analysis demonstrated a statistically significant association between the presence of preoperative aqueductal CSF flow acceleration on dynamic brain MRI and neurological outcomes at 12 and 18 months after VPS surgery. CONCLUSIONS: Long-term outcomes of iNPH patients treated with VPS surgery do not appear to be directly influenced by preoperative comorbidities, age, or responses to CSF samplings. Alterations in preoperative brain MRI CSF dynamics were found to be related to long-term outcomes following VPS surgery, highlighting the role of radiological assessment as a prognostic factor in patients deemed suitable candidates for VPS surgery.
Subject(s)
Hydrocephalus, Normal Pressure , Ventriculoperitoneal Shunt , Humans , Hydrocephalus, Normal Pressure/surgery , Hydrocephalus, Normal Pressure/diagnostic imaging , Male , Female , Aged , Prognosis , Retrospective Studies , Follow-Up Studies , Middle Aged , Aged, 80 and over , Treatment Outcome , Cohort Studies , Magnetic Resonance ImagingABSTRACT
This study aimed to retrospectively review the frequency and clinical features of 13 patients with progressive supranuclear palsy (PSP) and idiopathic normal pressure hydrocephalus (iNPH). All patients were found to have PSP-Richardson's syndrome (PSP-RS). Shunt surgery was effective in 5 of 11 patients (45.5%). A comparison of these 5 patients who responded to shunt surgery versus the remaining 6 patients revealed a significant difference in the reduction of frontal lobe blood flow on cerebral perfusion single-photon emission computed tomography (SPECT) (P = 0.018). These results suggest that PSP-RS is common in patients with PSP and iNPH and indicate the usefulness of cerebral perfusion SPECT in estimating the effect of shunt surgery.
Subject(s)
Hydrocephalus, Normal Pressure , Supranuclear Palsy, Progressive , Humans , Supranuclear Palsy, Progressive/diagnostic imaging , Supranuclear Palsy, Progressive/surgery , Hydrocephalus, Normal Pressure/diagnostic imaging , Hydrocephalus, Normal Pressure/surgery , Retrospective Studies , Frontal LobeABSTRACT
Idiopathic normotensive hydrocephalus (iNH) is a widespread disease in elderly patients. The effectiveness of iNG treatment and the subsequent quality of patients' lives directly depends on timely and early diagnosis. The criteria for diagnosing iNG that are used in neuroimaging can also be found in patients without clinical manifestations of this disease, and the widely used tap-test is an invasive technique with a rather low sensitivity. The need for early diagnosis and initiation of treatment before the development of irreversible damage to brain structures determines the relevance of the search for an accessible, minimally invasive, accurate and safe diagnostic method. The article presents a clinical observation of the use of phase-contrast MRI of cerebrospinal fluid (CSF) in a female patient with a positive response to the tap test with a quantitative analysis of changes in CSF flow parameters and ALVI and Evans indices depending on the time after CSF evacuation. Phase-contrast MRI of CSF with a quantitative assessment of CSF flow parameters in combination with an assessment of the ALVI index has the potential to increase the accuracy of diagnosing iNH and is of scientific interest for further research.
Subject(s)
Hydrocephalus, Normal Pressure , Hydrocephalus , Humans , Female , Aged , Hydrocephalus, Normal Pressure/diagnostic imaging , Hydrocephalus/diagnostic imaging , Magnetic Resonance Imaging/methods , Brain , Spinal Puncture , Neuroimaging , Cerebrospinal FluidABSTRACT
BACKGROUND: Reports of freezing of gait (FoG) in idiopathic normal pressure hydrocephalus (iNPH) are few and results are variable. This study's objective was to evaluate the frequency of FoG in a large cohort of iNPH patients, identify FoG-associated factors, and assess FoG's responsiveness to shunt surgery. METHODS: Videotaped standardized gait protocols with iNPH patients pre- and post-shunt surgery (n = 139; median age 75 (71-79) years; 48 women) were evaluated for FoG episodes by two observers (Cohens kappa = 0.9, p < 0.001). FoG episodes were categorized. Mini-mental state examination (MMSE) and MRI white matter hyperintensities (WMH) assessment using the Fazekas scale were performed. CSF was analyzed for Beta-amyloid, Tau, and Phospho-tau. Patients with and without FoG were compared. RESULTS: Twenty-two patients (16%) displayed FoG at baseline, decreasing to seven (8%) after CSF shunt surgery (p = 0.039). The symptom was most frequently exhibited during turning (n = 16, 73%). Patients displaying FoG were older (77.5 vs. 74.6 years; p = 0.029), had a slower walking speed (0.59 vs. 0.89 m/s; p < 0.001), a lower Tinetti POMA score (6.8 vs. 10.8; p < 0.001), lower MMSE score (21.3 vs. 24.0; p = 0.031), and longer disease duration (4.2 vs. 2.3 years; p < 0.001) compared to patients not displaying FoG. WMH or CSF biomarkers did not differ between the groups. CONCLUSIONS: FoG is occurring frequently in iNPH patients and may be considered a typical feature of iNPH. FoG in iNPH was associated with higher age, longer disease duration, worse cognitive function, and a more unstable gait. Shunt surgery seems to improve the symptom.
Subject(s)
Gait Disorders, Neurologic , Hydrocephalus, Normal Pressure , Parkinson Disease , Humans , Female , Aged , Hydrocephalus, Normal Pressure/diagnostic imaging , Hydrocephalus, Normal Pressure/surgery , Hydrocephalus, Normal Pressure/complications , Gait Disorders, Neurologic/etiology , Gait , Cerebrospinal Fluid Shunts/methodsABSTRACT
OBJECTIVE: Several radiological markers have been linked to clinical improvement after shunt surgery for idiopathic normal pressure hydrocephalus (iNPH). However, iNPH has no pathognomonic feature, and patients are still diagnosed as probable, possible, or unlikely cases based on clinical symptoms, imaging findings, and invasive supplementary tests. The predictive value of the disproportionately enlarged subarachnoid space hydrocephalus (DESH) score is not yet conclusively determined, but it might offer a more accurate diagnostic method. The aim of the present retrospective cohort study was to validate the predictive power of the DESH score for clinical improvement after shunt surgery in iNPH patients. METHODS: We retrospectively obtained presurgical MRI and/or CT scans from 71 patients with iNPH who underwent ventriculoperitoneal shunt surgery. Radiological images were evaluated for Evans index (EI), corpus callosal angle (CA), tight high convexity (THC), Sylvian fissure dilation, and focal sulci dilation. These markers were aggregated to determine the DESH score. Patient journal entries were used to subjectively determine the extent of improvement in gait function, urinary incontinence, and/or cognition as a measure of shunt surgery response. RESULTS: Multiple logistic regression analysis, controlling for age and sex (α = 0.05), showed that DESH score was significantly correlated (OR 1.77) with subjective shunt-surgery response at a minimum of 1-month follow-up. Patients with higher DESH scores were more likely to have a favorable response to shunt surgery. CONCLUSION: Aggregating radiological markers into the DESH score is useful for predicting shunt responders among iNPH patients and can aid the selection of patients for surgery. These findings provide further support for the DESH score as a diagnostic tool for iNPH.
Subject(s)
Hydrocephalus, Normal Pressure , Ventriculoperitoneal Shunt , Humans , Hydrocephalus, Normal Pressure/surgery , Hydrocephalus, Normal Pressure/diagnostic imaging , Male , Female , Aged , Prognosis , Retrospective Studies , Aged, 80 and over , Middle Aged , Treatment Outcome , Magnetic Resonance Imaging , Subarachnoid Space/surgery , Subarachnoid Space/diagnostic imaging , Cohort StudiesABSTRACT
BACKGROUND: There are currently many imaging indicators for idiopathic normal pressure hydrocephalus (iNPH). However, their diagnostic performance has not been well compared, especially in differentiating iNPH from Alzheimer's disease (AD). This study aimed to evaluate the diagnostic performance of these imaging indicators in differentiating iNPH from AD. METHODS: We retrospectively collected patients with iNPH from the West China Hospital between June 2016 and December 2023. Age-sex-matched patients with AD and healthy controls (HCs) are included as controls (ChiCTR2300070078, March 2023). Twelve imaging indicators were evaluated on MRI, including disproportionately enlarged subarachnoid space hydrocephalus (DESH), Evans' index (EI), callosal angle, z-EI, temporal horn, dilated Sylvian fissure, focal sulcal dilation, tight high convexity, deep white matter hyperintensities, periventricular hyperintensities, DESH scale, and Simplified Radscale. We analyzed the receiver operating characteristic curves and calculated the sensitivity, specificity, negative predictive value (NPV), positive predictive value (PPV), and accuracy. RESULTS: A total of 46 patients with iNPH (mean age: 73.1 ± 6.5; 35 males), 46 patients with AD (mean age: 73.0 ± 6.6; 35 males), and 46 HCs (mean age: 73.0 ± 5.9; 35 males) were included. The largest area under the receiver operating characteristic curve (AUC) was found in EI (0.93; 95â¯% CI: 0.89-0.98) and z-EI (0.93; 95â¯% CI: 0.87-0.98). DESH scale ≥ 6 had the highest specificity (93â¯%, 43/46). CONCLUSION: EI and z-EI had the best diagnostic performance in differentiating iNPH from AD. The DESH scale could assist in diagnosing iNPH due to its high specificity.
Subject(s)
Alzheimer Disease , Hydrocephalus, Normal Pressure , Magnetic Resonance Imaging , Humans , Hydrocephalus, Normal Pressure/diagnostic imaging , Alzheimer Disease/diagnostic imaging , Male , Aged , Female , Diagnosis, Differential , Retrospective Studies , Aged, 80 and over , Magnetic Resonance Imaging/methods , Middle Aged , Sensitivity and SpecificityABSTRACT
OBJECTIVE: To use third ventricle morphometric variables as a tool for the selection of patients with idiopathic normal pressure hydrocephalus (iNPH) who are candidates for ventriculoperitoneal shunts (VPS). MATERIAL AND METHODS: Retrospective study enrolling patients with iNPH. Katzman infusion test was performed and a Routâ¯>â¯12â¯mmHg/mL/min was considered a positive result. The transverse diameter and the volume of the third ventricle were measured in the preoperative MRI. Postoperative improvement was assessed with the NPH score. The results were analysed with SPSS software. RESULTS: 52 patients with a mean age of 76 years were analysed. There was no difference in the diameter of the third ventricle among patients with a positive result and those with a negative result in the infusion test (12.28 vs 11.68â¯mm; pâ¯=â¯0.14). Neither were difference detected in the ventricle volume of both groups (3.6 vs 3.5cc; pâ¯=â¯0.66). Those patients who improved after VPS had a smaller third ventricle compared to those who did not respond after surgery (11.85â¯mm vs. 12.96â¯mm; pâ¯=â¯0.009). Diameter and volume of third ventricle present a significant strong correlation (Pearson correlation coefficientâ¯=â¯0.72; pâ¯<â¯0.0001). CONCLUSION: Morphometric variables of third ventricle may be useful in predicting a good response to VPS in patients with idiopathic normal pressure hydrocephalus.
Subject(s)
Hydrocephalus, Normal Pressure , Magnetic Resonance Imaging , Third Ventricle , Ventriculoperitoneal Shunt , Humans , Hydrocephalus, Normal Pressure/diagnostic imaging , Hydrocephalus, Normal Pressure/surgery , Third Ventricle/diagnostic imaging , Third Ventricle/surgery , Third Ventricle/pathology , Aged , Male , Female , Retrospective Studies , Aged, 80 and over , Organ Size , Middle Aged , Patient SelectionABSTRACT
Neurosarcoidosis (NS) is a rare subtype of sarcoidosis with a poor prognosis and diverse clinical presentations that often poses a diagnostic and therapeutic challenge. We describe the case of a 53-year-old male with an initial diagnosis of lingual sarcoidosis, who subsequently developed ataxia and rapidly progressive cognitive impairment. A lumbar puncture revealed hypoglycorrhachia, hyperproteinorrachia, lymphocytic pleocytosis, and elevated IL-6 levels (600 pg/ml). Cerebrospinal fluid flow cytometry showed an elevated CD4 lymphocyte concentration and a CD4+/CD8+ ratio of 3.91, indicative of NS. Brain MRI showed hyperintense periventricular and subcortical lesions on FLAIR/T2 resembling progressive multifocal leukoencephalopathy (PML), although negative PCR for JC virus ruled out the differential diagnosis. Following a favorable evolutionary course with corticosteroid pulses, the patient relapsed with normotensive hydrocephalus, treated with immunosuppressants and ventriculoperitoneal shunting with a good response to date. This case underscores the importance of maintaining a high index of suspicion for NS in individuals with sarcoidosis and neurologic symptoms. In these cases, cerebrospinal fluid biomarkers such as IL-6 and CD4+/CD8+ ratio are essential to guide the diagnosis. Furthermore, it highlights that hydrocephalus is a rare complication and requires a multidisciplinary approach, including medical and neurosurgical treatment.
La neurosarcoidosis es un subtipo raro de sarcoidosis con mal pronóstico y diversas presentaciones clínicas que a menudo plantea un reto diagnóstico y terapéutico. Describimos el caso de un varón de 53 años con diagnóstico inicial de sarcoidosis lingual, que posteriormente desarrolló ataxia y deterioro cognitivo de rápida evolución. Una punción lumbar reveló hipoglucorraquia, hiperproteinorraquia, pleocitosis linfocítica y niveles elevados de IL-6 (600 pg/ml). La citometría de flujo del líquido cefalorraquídeo mostró una concentración elevada de linfocitos CD4 y un cociente CD4+/CD8+ de 3.91, indicativo de neurosarcoidosis. La RM cerebral evidenció lesiones hiperintensas periventriculares y subcorticales en FLAIR/T2 que se asemejaban a una leucoencefalopatía multifocal progresiva (LMP), aunque la PCR negativa para el virus JC descartó el diagnóstico diferencial. Tras un curso evolutivo favorable con pulsos de corticoides, el paciente recayó con hidrocefalia normotensiva, tratada con inmunosupresores y derivación ventriculoperitoneal con buena respuesta hasta la fecha. Este caso subraya la importancia de mantener un alto índice de sospecha de neurosarcoidosis en individuos con sarcoidosis y síntomas neurológicos. En estos casos, los biomarcadores del líquido cefalorraquídeo tales como la IL-6 y el cociente CD4+/CD8+ son esenciales para orientar el diagnóstico. Además, destaca que la hidrocefalia es una complicación poco frecuente y requiere un abordaje multidisciplinario, que incluya tratamiento médico y neuroquirúrgico.
Subject(s)
Central Nervous System Diseases , Dementia , Hydrocephalus, Normal Pressure , Sarcoidosis , Humans , Male , Sarcoidosis/complications , Sarcoidosis/diagnosis , Middle Aged , Hydrocephalus, Normal Pressure/diagnosis , Hydrocephalus, Normal Pressure/diagnostic imaging , Central Nervous System Diseases/complications , Dementia/etiology , Magnetic Resonance Imaging , Disease Progression , Diagnosis, DifferentialABSTRACT
AIM: To prove the superiority of the electromagnetic (EM) neuronavigation technique to increase the accuracy of intraventicular shunt catheter placement, and to reduce accompanying complications. MATERIAL AND METHODS: A total of 21 patients with hydrocephalus [age range (years): 53-84] were studied. All of them had undergone thin-slice, navigation-compatible, computed tomography (CT) preoperatively. Shunt surgery was performed under the guidance of EM neuronavigation technology. All patients underwent follow-up CT the next day to evaluate catheter tip placement and were followed up at 1, 3, 6, and 12 months. RESULTS: All catheter tips were placed properly in front of the foramen of Monro in the desired position, except in one case in which the tip migrated to the perimesencephalic cistern and underwent reoperation in the early postoperative period. No complications due to infection and obstruction were observed in the medium- and long-term follow-ups. The complication rate due to the incorrect catheter positioning was 4.76% of the total cases. CONCLUSION: The placement of the ventricular catheter under EM-guided navigation technology reduces the proximal-end failure caused by malpositioning, obstruction, and infection.
Subject(s)
Hydrocephalus, Normal Pressure , Hydrocephalus , Adult , Humans , Neuronavigation/methods , Hydrocephalus, Normal Pressure/diagnostic imaging , Hydrocephalus, Normal Pressure/surgery , Hydrocephalus, Normal Pressure/complications , Electromagnetic Phenomena , Neurosurgical Procedures/methods , Hydrocephalus/surgery , Hydrocephalus/etiology , Ventriculoperitoneal Shunt/adverse effects , Catheters/adverse effectsABSTRACT
BACKGROUND AND PURPOSE: Normal pressure hydrocephalus is a treatable cause of dementia associated with distinct mechanical property signatures in the brain as measured by MR elastography. In this study, we tested the hypothesis that specific anatomic features of normal pressure hydrocephalus are associated with unique mechanical property alterations. Then, we tested the hypothesis that summary measures of these mechanical signatures can be used to predict clinical outcomes. MATERIALS AND METHODS: MR elastography and structural imaging were performed in 128 patients with suspected normal pressure hydrocephalus and 44 control participants. Patients were categorized into 4 subgroups based on their anatomic features. Surgery outcome was acquired for 68 patients. Voxelwise modeling was performed to detect regions with significantly different mechanical properties between each group. Mechanical signatures were summarized using pattern analysis and were used as features to train classification models and predict shunt outcomes for 2 sets of feature spaces: a limited 2D feature space that included the most common features found in normal pressure hydrocephalus and an expanded 20-dimensional (20D) feature space that included features from all 4 morphologic subgroups. RESULTS: Both the 2D and 20D classifiers performed significantly better than chance for predicting clinical outcomes with estimated areas under the receiver operating characteristic curve of 0.66 and 0.77, respectively (P < .05, permutation test). The 20D classifier significantly improved the diagnostic OR and positive predictive value compared with the 2D classifier (P < .05, permutation test). CONCLUSIONS: MR elastography provides further insight into mechanical alterations in the normal pressure hydrocephalus brain and is a promising, noninvasive method for predicting surgical outcomes in patients with normal pressure hydrocephalus.
Subject(s)
Elasticity Imaging Techniques , Hydrocephalus, Normal Pressure , Hydrocephalus , Humans , Hydrocephalus, Normal Pressure/diagnostic imaging , Hydrocephalus, Normal Pressure/surgery , Magnetic Resonance Imaging/methods , Brain/diagnostic imaging , Brain/surgery , Treatment OutcomeABSTRACT
A woman in her 70s presented with approximately 2 years of sudden-onset gait and cognitive problems. She had been diagnosed with normal pressure hydrocephalus (NPH) and underwent ventriculoperitoneal shunt (VPS) placement 1 year prior. Before VPS placement, brain imaging showed ventriculomegaly and chronic infarction of the right putamen and claustrum. A lumbar drain trial resulted in modest improvement of gait dysfunction. She underwent VPS placement for suspected NPH, but her symptoms remained unchanged. Examination revealed mild cognitive impairment, left-sided and lower body predominant parkinsonism, as well as disproportionately prominent postural instability. Gait analysis showed increased gait variability, reduced velocity and shortened step length bilaterally. Motor and gait abnormalities did not change after administration of levodopa. Her symptoms have remained stable for up to 52 months since symptom onset. We postulate that the infarction affecting the right putamen and claustrum could have led to a higher-level gait disorder mimicking NPH.