Mild Hydrocephalus Research Articles

Distinct roles of Kif6 and Kif9 in mammalian ciliary trafficking and motility.

Ciliary beat and intraflagellar transport depend on dynein and kinesin motors. The kinesin-9 family members Kif6 and Kif9 are implicated in motile cilia motilities across protists and mammals. How they function and whether they act redundantly, however, remain unclear. Here, we show that Kif6 and Kif9 play distinct roles in mammals. Kif6 forms puncta that move bidirectionally along axonemes, whereas Kif9 appears to oscillate regionally on the ciliary central apparatus. Consistently, only Kif6 displays microtubule-based motor activity in vitro, and its ciliary localization requires its ATPase activity. Kif6 deficiency in mice disrupts coordinated ciliary beat across ependymal tissues and impairs cerebrospinal fluid flow, resulting in severe hydrocephalus and high mortality. Kif9 deficiency causes mild hydrocephalus without obviously affecting the ciliary beat or the lifespan. Kif6-/- and Kif9-/- males are infertile but exhibit oligozoospermia with poor sperm motility and defective forward motion of sperms, respectively. These results suggest Kif6 as a motor for cargo transport and Kif9 as a central apparatus regulator.

Atypical epidermoid cyst of the fourth ventricle with minimal diffusion-restriction.

Epidermoid cysts are benign, slow growing extra-axial lesions most commonly found in the cerebellopontine angle that have a characteristic imaging pattern of restricted diffusion on diffusion-weighted imaging (DWI). A 10-year-old male with a history of asthma and diabetes was found to have a lesion within the fourth ventricle on a magnetic resonance imaging (MRI) brain study. MRI showed a well-circumscribed vermian lesion without contrast enhancement or restricted diffusion with mild hydrocephalus. He was referred to the neurosurgical service once he acutely developed symptoms of dizziness. He underwent a gross-total resection of the lesion on which histopathology confirmed an epidermoid cyst. Here, we report a case of an atypical epidermoid cysts found in the midline of the fourth ventricle without restricted diffusion on MRI. Avid restricted-diffusion on DWI is usually pathognomonic for an epidermoid cyst when evaluating an extra-axial lesion, yet we report the second case in the literature of an epidermoid cyst without this classical imaging characteristic.

Recovery of Learning and Memory Deficits Despite Persistent Pyknosis of the Hippocampal Pyramidal Neurons of Adult Hydrocephalic Mice.

The hippocampal alterations resulting from hydrocephalus are associated with various cognitive dysfunctions. Reduced learning and memory are early functional deficits that recover with time in experimental hydrocephalus. This study investigated the recovery processes of learning and memory loss in relation to the morphology of hippocampal pyramidal neurons and the degree of expansion of the ventricles. Hydrocephalus was induced in adult mice by intracisternal injection of sterile kaolin while controls received sham operation. Neurobehavioral tests for memory and learning were conducted, after which the animals were sacrificed in batches: 7 (acute) and 28 (subacute) days postinduction. After sacrifice, mice were categorized into mild and moderate hydrocephalus, and their fixed brain samples were processed for hematoxylin, eosin, and Nissl stains. In moderate acute hydrocephalus, the indices of learning and memory were reduced escape latency (67.20 ± 12.83 s), number of platform crossing (4.000 ± 1.658), duration in platform quadrant (4.000 ± 1.658), and percent of total investigation (44.857% ± 3.981%) but not in the subacute stage. Pyknotic indices (PI) were significantly higher in the cornu ammonis (CA)1 and 3 regions in all hydrocephalic groups than in controls. However, within groups, PI was significantly higher only in the CA1 of moderate acute (28.149% ± 1.875%) compared to moderate subacute hydrocephalic group (12.903% ± 3.23%). Hydrocephalus caused cellular injury to the hippocampus associated with spatial learning and memory deficits. However, these functional deficits were partially reversed in moderate subacute hydrocephalus despite the persistence of the structural alterations in the CA1 and CA3 subregions.

Pediatric Case of Autoimmune Cerebellitis with Positive Anti-GAD Antibodies and Accidental Finding of Roth Spots

Abstract We present a pediatric case of autoimmune cerebellitis, which was distinguished by the presence of positive anti-glutamic acid decarboxylase (anti-GAD) antibodies. The patient, a young child, presented with a four-day history of progressively worsening headaches, coupled with persistent vomiting. These symptoms prompted immediate medical evaluation. Initial computed tomography (CT) imaging revealed a hypodense area in the left cerebellum and mild hydrocephalus, indicating fluid accumulation in the brain. Subsequent magnetic resonance imaging (MRI) provided more detailed visualization, confirming the diagnosis of acute cerebellitis, which was characterized by bilateral enlargement of the cerebellum and an associated mass effect that exerted pressure on the adjacent brain structures. The medical team initiated a comprehensive treatment regimen that included antibiotics to address any potential bacterial infection, antivirals to counter possible viral etiologies, immunoglobulin therapy to modulate the immune response, and corticosteroids to reduce inflammation. The patient responded well to this treatment protocol, with follow-up imaging showing a significant reduction in cerebellar swelling and overall improvement in clinical symptoms. The detection of elevated anti-GAD antibodies, along with anti-thyroglobulin antibodies, indicated an autoimmune origin of the cerebellitis. This autoimmune response can lead to the body’s immune system mistakenly attacking healthy brain tissue. Moreover, the incidental finding of a Roth spot, a retinal hemorrhage with a white center, during a routine eye examination suggested potential systemic involvement, possibly indicating a broader immune-mediated condition.

Fast Quantitative Magnetic Resonance Imaging Evaluation of Hydrocephalus Using 3-Dimensional Fluid-Attenuated Inversion Recovery: Initial Experience.

This study aimed to demonstrate the initial experience of using fast quantitative magnetic resonance imaging (MRI) to evaluate hydrocephalus. A total of 109 brain MRI volumetry examinations (acquisition time, 7 minutes 30 seconds) were performed in 72 patients with hydrocephalus. From the measured ventricular system and brain volumes, ventricle-brain volume percentage was calculated to standardize hydrocephalus severity (processing time, <5 minutes). The obtained values were categorized into no, mild, and severe based on the fronto-occipital horn ratio (FOHR) and the ventricle-brain volume percentages reported in the literature. The measured volumes and percentages were compared between patients with mild hydrocephalus and those with severe hydrocephalus. The diagnostic performance of brain hydrocephalus MRI volumetry was evaluated using receiver operating characteristic curve analysis. Ventricular volumes and ventricle-brain volume percentages were significantly higher in in patients with severe hydrocephalus than in those with mild hydrocephalus (FOHR-based severity: 352.6 ± 165.6 cm 3 vs 149.1 ± 78.5 cm 3 , P < 0.001, and 26.8% [20.8%-33.1%] vs 12.1% ± 6.0%, P < 0.001; percentage-based severity: 359.5 ± 143.3 cm 3 vs 137.0 ± 62.9 cm 3 , P < 0.001, and 26.8% [21.8%-33.1%] vs 11.3% ± 4.2%, P < 0.001, respectively), whereas brain volumes were significantly lower in patients with severe hydrocephalus than in those with mild hydrocephalus (FOHR-based severity: 878.1 ± 363.5 cm 3 vs 1130.1 cm 3 [912.1-1244.2 cm 3 ], P = 0.006; percentage-based severity: 896.2 ± 324.6 cm 3 vs 1142.3 cm 3 [944.2-1246.6 cm 3 ], P = 0.005, respectively). The ventricle-brain volume percentage was a good diagnostic parameter for evaluating the degree of hydrocephalus (area under the curve, 0.855; 95% confidence interval, 0.719-0.990; P < 0.001). Brain MRI volumetry can be used to evaluate hydrocephalus severity and may provide guide interpretation because of its rapid acquisition and postprocessing times.

L1cam alternative shorter transcripts encoding the extracellular domains were overexpressed in the intestine of L1cam knockdown mice

Hirschsprung disease (HSCR) is a congenital disorder of functional bowel obstruction due to the absence of enteric ganglia in distal bowel. Different L1cam variants were reportedly associated with L1cam syndrome and HSCR, whose phenotypes lacked predictable relevance to their genotypes. Using next-generation sequencing (NGS), we found an L1CAM de novo frameshift mutation in a female with mild hydrocephalus and skip-type HSCR. A nearly identical L1cam variant was introduced into FVB/NJ mice via the CRISPR-EZ method. A silent mutation was created via ssODN to gain an artificial Ncol restriction enzyme site for easier genotyping. Six L1cam protein-coding alternative transcripts were quantitatively measured. Immunofluorescence staining with polyclonal and monoclonal L1cam antibodies was used to characterize L1cam isoform proteins in enteric ganglia. Fifteen mice, seven males and eight females, generated via CRISPR-EZ, were confirmed to carry the L1cam frameshift variant, resulting in a premature stop codon. There was no prominent hydrocephalus nor HSCR-like presentation in these mice, but male infertility was noticed after observation for three generations in a total of 176 mice. Full-length L1cam transcripts were detected at a very low level in the intestinal tissues and almost none in the brain of these mice. Alternative shorter transcripts encoding the extracellular domains were overexpressed in the intestine of L1cam knockdown mice. Immunofluorescence confirmed no fulllength L1cam protein in enteric ganglia. These shorter L1cam isoform proteins might play a role in protecting L1cam knockdown mice from HSCR.

Cavernous Malformations: What They Have Taught Us.

Cavernous Malformations: What They Have Taught Us.

Lhermitte-Duclos Disease.

HomeRadiologyVol. 307, No. 1 PreviousNext Reviews and CommentaryFree AccessImages in RadiologyLhermitte-Duclos DiseaseDhairya A. Lakhani , SoHyun BooDhairya A. Lakhani , SoHyun BooAuthor AffiliationsFrom the Department of Radiology, West Virginia University, Ruby Memorial Hospital, 1 Medical Center Dr, Morgantown, WV 26506 (D.A.L., S.H.B.); and Department of Neuroradiology, Rockefeller Neuroscience Institute, West Virginia University, Morgantown, WV (S.H.B.).Address correspondence to D.A.L. (email: [email protected]).Dhairya A. Lakhani SoHyun BooPublished Online:Dec 13 2022https://doi.org/10.1148/radiol.221979MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In An 18-year-old woman with no past medical history presented with chronic headache and new onset cerebellar ataxia. MRI of the brain (Figure, A–D) demonstrated a mass in the left cerebellar hemisphere. The mass was predominantly hyperintense with T2-weighted images with intrinsic hyper- to isointense striations relative to gray matter (Figure, A–B). The mass was predominantly hypointense with T1-weighted images with striations hypo- to isointense relative to gray matter (Figure, C). The mass showed no enhancement with contrast-enhanced images (Figure, D) and did not restrict diffusion with diffusion-weighted images (not shown). Mild obstructive-pattern hydrocephalus was present from compression of the fourth ventricle.Images in an 18-year-old woman who presented with chronic headache and cerebellar ataxia. (A, B) Axial T2-weighted, (C) precontrast T1-weighted, and (D) postcontrast T1-weighted MRI scans of the brain demonstrate a mass in the left cerebellar hemisphere. The mass was predominantly hyperintense with (A, B) T2-weighted images with intrinsic hyper- to isointense striations relative to gray matter and predominantly hypointense with (C) T1-weighted images with hypo- to isointense striations relative to gray matter. No intrinsic contrast enhancement was seen with (D) postcontrast images.Download as PowerPointOpen in Image Viewer The typical striated, corduroy, or tiger-striped folial pattern consisting of alternating bands with T2-weighted and T1-weighted images is characteristic of Lhermitte-Duclos disease or dysplastic cerebellar gangliocytoma, a rare hamartomatous disorder (1,2). It is an Aunt Minnie diagnosis and has close association with Cowden syndrome. Clinically, patients may be asymptomatic or present from symptoms related to mass effect (1).Disclosures of conflicts of interest: D.A.L. RSNA William W. Olmsted Trainee Editorial Fellow (2022). S.H.B. No relevant relationships.

An Unusual Case of Malignant Melanoma with Metastasis to the Placenta During Pregnancy.

IntroductionWe describe an unusual case of metastatic melanoma of the brain with an unknown primary site during pregnancy.Case DescriptionA 35-year-old woman in the third trimester of pregnancy presented with ataxia, nausea, vomiting, headaches and diplopia. CT of the brain revealed a hyper-attenuating 2.1 cm mass in the fourth ventricle with mild obstructive hydrocephalus. A healthy newborn was delivered by urgent caesarean section. Craniotomy and resection of the brain lesion confirmed melanoma. Pathology of the placenta reported a ‘focal nest of melanocytes identified in intervillous space’.DiscussionBrain and maternal placenta pathology findings were consistent with melanoma, making this case relevant because of the possibility of metastatic melanoma in a fetus.ConclusionEpidemiological data on congenital and infantile melanoma are scarce. Also, there is no database for long-term follow-up of children born to pregnant mothers with metastatic melanoma. Delayed presentation of melanoma in the child cannot be ruled out.LEARNING POINTSMelanoma brain metastasis is an uncommon initial presentation during pregnancy.During pregnancy, vague symptoms such as headaches and nausea can easily be attributed to the pregnancy itself rather than more serious conditions like cancer.Metastatic melanoma diagnosed during pregnancy with disease in the placenta is a rare occurrence and should trigger close follow-up of the neonate secondary to concerns of transplacental metastasis.

Postinfectious coronavirus disease 2019 hemorrhagic cerebellitis: illustrative case.

BACKGROUNDConditions that can mimic posterior fossa tumors are rare. Their identification is crucial to avoid unnecessary surgical intervention, especially when prompt initiation of medical therapy is critical.OBSERVATIONSThe authors presented a case of pseudotumoral hemorrhagic cerebellitis in a 3-year-old boy who presented initially with headache, persistent vomiting, and decreased level of consciousness 9 weeks after severe acute respiratory syndrome coronavirus 2 infection. Magnetic resonance imaging showed a left cerebellar hemorrhagic mass–like lesion with edema and mild hydrocephalus. The patient responded to high-dose steroids and was discharged 2 weeks later with complete recovery.LESSONSWhen evaluating patients with possible tumor syndromes, it is important to also consider rarer inflammatory syndromes that can masquerade as neoplasms. Postinfectious hemorrhagic cerebellitis is one such syndrome.

Suprasellar arachnoid cyst with temporal extension or temporal cyst with suprasellar extension?

A 4-year-old female child presented to an emergency hospital with headache and episodes of seizures. Computed tomography and magnetic resonance imaging of the skull (Figs. 1a, b and c) were performed; they showed large suprasellar cystic lesions with right temporal extension and mild hydrocephalus. Subsequently, an endoscopic ventriculocystocysternotomy was performed (Figs. 1d, e, and f), which showed good results. Suprasellar arachnoid cysts account for 9%–21% of the pediatric arachnoid cysts [1,2]. According to the morphology and characteristics, there are three types of suprasellar arachnoid cysts, as follows: 1. Diencephalic leaflet dilatation of the Liliequist membrane with the formation of purely suprasellar cysts, which presents with hydrocephalus; 2. The defect of the mesencephalic leaflet of the Liliequist membrane with dilatation of the interpeduncular cistern, which presents without hydrocephalus; 3. Asymmetrical form that extends to other subarachnoid spaces, which presents with macrocrania and mild or no hydrocephalus [1], similar to the present case. The expansion could be due to an osmotic gradient, a slit valve mechanism, tissue debris transudation from the choroid plexus, or ectopic glial cells [3]. The first treatment option for suprasellar arachnoid cysts should be endoscopic ventriculocystocysternotomy; ventriculoperitoneal shunt may be considered the second treatment option if endoscopic ventriculocystocysternotomy fails [2]. Figure Caption Magnetic resonance imaging of the brain: (a) T1 axial and (b) T2 axial showing a large suprasellar (black arrows) cyst with expansion to the right temporal pole, having a mass effect and mild ventricular dilatation(white arrows) without ependymal transudation; (c) Sagittal FIESTA-T2 with compressive effect on the brain stem(white arrow) and basilar artery(black arrow) displacement; Endoscopic view: (d) ventriculocystotomy; (e) the third ventricular floor with pituitary stalk(black arrow) and gland(white arrow), dorsum of the sella turcica(black curved arrow), posterior communicating artery(black arrowhead), and oculomotor nerve(white arrowhead); (f) cyststocisternotomy was conducted with visualization of the basilar artery((black arrow) and dorsum of the saddle.

KORELASI HIDROSEFALUS BERDASARKAN PEMERIKSAAN CT SCAN DENGAN KLINIS DI RSUD TIARA KASIH SEJATI PEMATANGSIANTAR

Hydrocephalus is a complex disorder of the formation, flow, and absorption of cerebrospinal fluid (CSF), characterized by increased intracranial pressure, pathological dilatation of the cerebral ventricles, and excessive accumulation of CSF. This study describes the correlation of hydrocephalus degrees in children aged 0-12 with clinical manifestation through a head CT scan to measure the degree of mild, moderate, and severe hydrocephalus using the linear measurement. Research subjects according to age group &lt; 6 years 62.8% and higher in males = 37.2%. Based on clinical manifestations, febrile seizures 48.5%, delayed development 31.3%, and decreased consciousness 20.1%. The average linear measurement values ​ with decreased consciousness were BCI = 0.650, VI = 0.38, FOHR = 0.829, FHR=0.045 and BFI=0.650. The average linear measurement values ​​compared to delayed development FHR, BCI and BFI were 0.500, 0.127, and 0.220, respectively, with the FOHR significance value = 0.109 and the VI significance value = 0.002. Linear measurement values compared to the degree of hydrocephalus with clinical manifestations of febrile convulsions were more in the moderate category, the decrease in consciousness was more in the mild category, while delayed development had more mild categories.

Deficits of Learning and Spatial Memory are Associated with Increased Pyknosis of Pyramidal Neurons of the Hippocampus of Adult Rats with Chronic Hydrocephalus

&#x0D; Background: The hippocampus is the brain's center for the consolidation of short-term and spatial memory that enables navigation. Hippocampal injury occurs in hydrocephalus and is associated with loss of memory.&#x0D; Objective: We assessed pyknotic changes in kaolin-induced chronic hydrocephalus in adult rats using qualitative and quantitative means, and related these to memory deficits in the rats.&#x0D; Methods: Adult rats were randomly divided into control and experimental groups. Hydrocephalus was induced by intracisternal injection of 0.1ml sterile kaolin suspension for 6weeks. Control rats received sham injections. Spatial memory was assessed with the Morris water maze test. Coronal sections of the brains were grouped into either mild or moderate hydrocephalus and then stained with H&amp;E and cresyl violet stains. Thus, there were three groups: control, mild hydrocephalus and moderate hydrocephalus (n=10), respectively.&#x0D; Results: Shrinking and thinning of the hippocampal tissue, distortion of the pyramidal layer and pyknotic cells were observed in the CA1 and CA3 regions of the hydrocephalic rats. The pyknotic indices in the mild hydrocephalic rat (Cornus Ammonis, CA) CA1, CA2, CA3 (54.30±1.38; 27.62±0.83; 57.61±0.74) and moderate hydrocephalic rat CA1, CA2, CA3 (48.18±0.67; 32.00±0.84; 42.41±1.19) regions were significantly increased compared to the controls' CA1, CA2, CA3 (12.14±0.56; 9.21±0.36; 13.04±0.59).&#x0D; Conclusion: Chronic hydrocephalus in adult rats was associated with cell death in all the regions of the hippocampus, irrespective of the degree of ventricular enlargement. The extent of cell death corresponded with the severity of learning and memory deficits.&#x0D; Background: The hippocampus is the center of the brain for the consolidation of short-term memory and spatial memory that enables navigation. Hippocampal damage occurs in hydrocephalus and is associated with memory loss.&#x0D; Objective: We assessed pyknotic changes in chronic kaolin-induced hydrocephalus in adult rats using qualitative and quantitative means, and related them to memory deficits in rats.&#x0D; Methods: Adult rats were randomly assigned to control and experimental groups. Hydrocephalus was induced by intracisternal injection of 0.1 ml of sterile kaolin suspension for 6 weeks. Control rats received sham injections. Spatial memory was assessed by the Morris water maze test. Coronal sections of brains were grouped into mild or moderate hydrocephalus and then stained with H&amp;E and cresyl violet. There were therefore three groups: control, mild hydrocephalus and moderate hydrocephalus (n=10), respectively.&#x0D; RESULTS: Shrinking and thinning of hippocampal tissue, distortion of the pyramidal layer, and pyknotic cells were observed in the CA1 and CA3 regions of hydrocephalic rats. The pyknotic indices in the CA1, CA2 and CA3 regions of mild hydrocephalic rats (Cornus Ammonis, CA) (54.30±1.38; 27.62±0.83; 57.61±0.74) and hydrocephalic rats moderate CA1, CA2 and CA3 (48.18±0.67; 32.00±0.84; 42.41±1.19) were significantly increased compared to the CA1, CA2, CA3 regions of controls (12.14±0.56; 9.21±0.36; 13.04±0.59).&#x0D; Conclusion: Chronic hydrocephalus in adult rats was associated with cell death in all regions of the hippocampus, regardless of the degree of ventricular enlargement. The extent of cell death corresponded to the severity of learning and memory deficits.&#x0D; Key words: Chronic hydrocephalus, hippocampus, adult rats, memory, pyknotic cells.&#x0D; &#x0D; Keywords: Adult rats; Chronic hydrocephalus; hippocampus; memory; pyknotic cells.

On The Alice In Wonderland Syndrome With A Case Report

Alice in Wonderland syndrome is a rare neurological disorder characterized by transient distorsion of visual perception, impaired perception of one’s own body and time sometimes along with derealization and depersonalization. One of the rare visual symptoms of this syndrome is inversion of visual perception, consisting of rotating or inverting the image of the observed environment lasting several seconds or minutes. The transient inversion of visual perception is thought to be the result of dysfunction of the parieto-insular vestibular or parieto-occipital cortex. We present a 6-year-old boy (М.М.К.) who informed his parents about three episodes of “rotation of the images” in front of him in the last several weeks, lasting 1-2 minutes and abruptly disappearing. Conducted neurological status, neuro-ophthalmological examination, electro-encephalography did not show the presence of abnormalities. MRT of the brain revealed the only pathologic finding – mild unilateral (left) lateral ventricular dilatation due to left foramen of Monro stenosis, interpreted as congenital. We hypothesize that mild unilateral hydrocephalus may be an etiological factor in our patient and cause transient cortical dysfunction and visual-spatial imbalance with impaired verticality.

DIPG-23. SINGLE CASE REPORT OF LOW DOSE RADIOTHERAPY AND CHEMOTHERAPY IN THE TREATMENT OF DIPG

A 3 year-old girl, was noted to have progressive gait problem since Nov. 2017 and brought to hospital for checkup. Brain MRI on Jan. 2018 showed T2 hyperintensity infiltrative pontine lesion, favoring diffuse infiltrative pontine glioma and mild obstructive hydrocephalus, received VP-shunting on Jan. 15, 2018. Due to the refusal of surgical biopsy for tissue proof, we started the radiotherapy from Jan. 24, 2018, using Rapidarc technique with 6MV photon energy to treat the brain stem lesion, ended on Feb. 24, 2018 with total dose of 25.5Gy in 17 fractions as our usual practice. Following the completion of radiotherapy, we started the adjuvant chemotherapy using 1-week on, 1-week off regimen of temozolomide using dosage of 75 mg/sq-m/day, and this patient’s general condition returned back to nearly normal. Serial follow-up images of brain MRI on 04/30/2018, 08/01/2018, 11/30/2018, 02/26/2019, 05/30/2019, 08/28/2019 showed slow progression of the pontine lesion, without the development of contrast enhanced new lesion. She maintained the functional independent until Sep. 2019, she was noted to have symptoms of ataxic gait, esotropia and choking on drinking liquid. We started the retreatment of radiotherapy from Oct.7, 2019, using same technique, ended on Nov. 5, 2019 with total dose of 30Gy in 20 fractions. The symptoms improved partially after the treatment, with residual weakness over left extremity. We are still treating the patient with adjuvant temozolomide, and she has survived most of time functionally independent in these 2 years.

Ralstonia pickettii bacteremia: An emerging infection in a Tertiary care hospital setting

Background: Ralstonia species are Gram-negative bacilli that have increasingly been recognized as emerging nosocomial pathogens particularly in immunocompromised hosts. Ralstonia pickettii is the most clinically important pathogen from the Ralstonia genus. Nosocomial outbreaks of Ralstonia pickettii infections brought about by use of contaminated medical solutions including saline, sterile water as well as disinfectants have been reported. There have been case reports of invasive infections with variable presentations. Here we describe three cases of Ralstonia pickettii bacteremia during a period of one year in a tertiary care hospital in Karachi, Pakistan. Case description: Case1: A 76 year old male known case of Type 2 Diabetes Mellitus, Hypertension and Amyotrophic lateral sclerosis presented with complaints of burning micturition, hematuria and fever. Patient had history of multiple hospital admissions in the recent past. His blood culture was found to be positive for Ralstonia pickettii. CT KUB was suggestive of pyelonephritis. Patient improved on intravenous Meropenem. Case 2: A 47 year old gentleman with no prior co-morbids was admitted with gunshot injury to the neck resulting in complete cervical cord resection and mild hydrocephalus with intraventricular haemorrhage. Patient had a prolonged ICU stay which was complicated by ventilator associated pneumonia with Acinetobacter and central line associated bloodstream infection with Ralstonia pickettii. He was treated with Meropenem and colistin but continued to deteriorate and expired. Case 3: 46 year old lady, known case of end stage renal disease, admitted with prosthetic valve endocarditis, had prolonged hospital stay complicated by central line associated blood stream infection with Ralstonia pickettii, improved on Meropenem but later died due to fungemia. Discussion: Ralstonia pickettii is an emerging gram-negative opportunistic pathogen. Ralstonia pickettii has been rarely known to cause severe invasive infections, including pneumonia, endocarditis, osteomyelitis, meningitis, and septic arthritis. It is a waterborne organism of low virulence, with a propensity to cause severe infections in the immunocompromised. Among our cases, two of the patients had chronic debilitating illnesses, which could have predisposed them to infection with this organism. Conclusion: Ralstonia pickettii is an emerging cause of nosocomial infection in patients particularly those with prolonged hospital stay and can cause invasive and severe infections.

Characteristics of cerebral hemodynamics in patients with chronic brain ischemia

Background. Literature sources report conflicting results regarding the severity of clinical symptoms of hydrocephalus (HC) and their association with cerebral arterial blood flow (CABF); most studies do not show a direct relationship, while some suggest a link between clinical severity and progressive decrease in CABF. The study of hemodynamic changes in the brain of patients with chronic brain ischemia (CBI), elucidation of their relationship with cognitive impairments helps to improve diagnostic approaches and optimize the prognosis of the disease. The purpose of this study is to investigate the state of cerebral hemodynamics and to establish its relationship with changes in cognitive functions in patients with CBI and HC. Materials and Methods. A comprehensive examination of 110 patients with CBI and HC was performed. The localization of the HC and results of Montreal Cognitive Test (MoCA scale) were taken into account. Computed tomography of the brain was performed with subsequent determination of morphometric parameters and indices. The state of cerebral blood flow and structural changes of blood vessels were studied using transcranial color-coded duplex ultrasonography (TCCS) of intracranial vessels and extracranial divisions. Microsoft Excel 2011 and Statistica were used for statistical processing of the results. Results. It was found that there was a significantly larger diameter of both common carotid arteries (CCA), thickness of complex intima\media (CIM), maximum systolic velocity (Vs), velocity at the end of the diastolic cycle (Vd), as well as peripheral resistance (IR) in left CCA (p &lt;0,05) in patients with CBI and HC compared with patients with CBI without HC. Significantly larger diameter of left internal carotid artery (ICA), Vs, Vd and IR was revealed; Vs and Vd in the right ICA; Vd in the extracranial devision of vertebral artery (VA) in patients with HC. A significant relationship was found between the following blood flow parameters and the values of the MoCA scale: Vs MCA/MoCA (r = 0,45, p &lt;0,05), Vs ACA/MoCA (r = 0,38, p &lt;0.05), ICA diameter/MoCA (r = -0,51, p &lt;0,05). It was found that the diameter of CCA and the thickness of CIM were significantly larger in patients with CBI with internal HC in comparison with patients with CBI with external HC (p &lt;0,01). Vs and Vd in patients with internal hydrocephalus were significantly lower, and IR was significantly higher compared with patients with external hydrocephalus (p &lt;0,01). The diameter of ICA and IR in patients with CBI with internal HC was significantly higher, and Vs was significantly lower compared with patients with CBI and external HC (p &lt;0,01). Significantly different values were found in Vs and IR meanings in middle cerebral artery (MCA) and Vs in anterior cerebral artery (ACA) in patients with CBI with internal HC compared with patients with CBI with external HC.Conclusions. Functional parameters of blood flow and structural changes of cerebral arteries in patients with CBI with concomitant hydrocephalus differed significantly from those in patients without hydrocephalus and depended on the type and severity of hydrocephalus. ICA occlusion, ICA stenosis &gt;50%, and intracranial venous stasis were significantly more common in patients with severe and moderate HC compared with those with mild HC. A weak relationship was found between the structure of carotid arteries and blood flow in intracranial arteries in patients with CBI and HC. There was a significant relationship between blood flow indices and the values of the MoCA scale, which indicated the effect of changes in the vessels of the anterior circular basin on the cognitive functions and the lack of such connection with blood flow indices in vertebrobasilar basin.

Neuroimage-Based Consciousness Evaluation of Patients with Secondary Doubtful Hydrocephalus Before and After Lumbar Drainage.

Hydrocephalus is often treated with a cerebrospinal fluid shunt (CFS) for excessive amounts of cerebrospinal fluid in the brain. However, it is very difficult to distinguish whether the ventricular enlargement is due to hydrocephalus or other causes, such as brain atrophy after brain damage and surgery. The non-trivial evaluation of the consciousness level, along with a continuous drainage test of the lumbar cistern is thus clinically important before the decision for CFS is made. We studied 32 secondary mild hydrocephalus patients with different consciousness levels, who received T1 and diffusion tensor imaging magnetic resonance scans before and after lumbar cerebrospinal fluid drainage. We applied a novel machine-learning method to find the most discriminative features from the multi-modal neuroimages. Then, we built a regression model to regress the JFK Coma Recovery Scale-Revised (CRS-R) scores to quantify the level of consciousness. The experimental results showed that our method not only approximated the CRS-R scores but also tracked the temporal changes in individual patients. The regression model has high potential for the evaluation of consciousness in clinical practice.

SAT-269 A Rare Case of Functioning Gonadotroph Producing Pituitary Macroadenoma in Teenage Male

Background: Unlike nonfunctioning gonadotroph pituitary adenomas, functioning gonadotroph pituitary adenomas (FGA) are an uncommon type of pituitary tumors that secrete biologically active gonadotropins (LH, FSH, or both). Clinical Case: A 23-year-old man with no previous medical history presented to the emergency department with three months history of progressive decreased vision and decreased libido. He denied headache, seizure, erectile dysfunction, or weakness. On physical examination, his visual acuity was significantly reduced on the right eye and was only able to perceive light. He had a visual field narrowing on the left eye. Gynecomastia was noted bilaterally and testicles were found to be enlarged (Orchidometer >25 mL). Complete blood count was significant for hemoglobin of 19.2 g/dL (N, 13.7-17.5 g/dL), and hematocrit of 57.0% (N, 40.1-51.0%). Pituitary function tests were as follow: FSH >200.0 mIU/mL (N, 1.5-12.4 mIU/mL), LH 17.0 mIU/mL (N, 1.7-8.6 mIU/mL), total testosterone 41.3 nmol/L (N, 8.6-29.0 nmol/L), free testosterone 1.263 nmol/L (N, 0.148-0.718 nmol/L), and bioavailable testosterone 29.609 (N, 2.110-8.920 nmol/L). Prolactin, TSH, GH, and ACTH were all within the normal range. Magnetic resonance imaging (MRI) showed a sellar mass involving the planum sphenoidal measuring 5.8 x 5.2 x 5.6 cm with optic chiasm displaced superiorly, in addition, subfalcine herniation with mild hydrocephalus was also noted. The patient underwent orbitozygomatic approach resection of the pituitary tumor. Histological examination was consistent with a pituitary adenoma. Cells stained positive for transcription factor steroidogenic factor 1 (SF 1), FSH, LH, and alpha-subunit consistent with a gonadotroph adenoma. They were negative for transcription factor Pit 1 stain and the remaining pituitary hormones including ACTH, GH, prolactin, and TSH stain. Postsurgical hormone assessment showed a significant decline in FSH and LH to 2.3 and 0.4 mIU/mL, respectively and testosterone level decreased to < 0.087 nmol/L on postoperative day 18. The patient’s vision improved postoperatively prior to discharge but he lost follow up thereafter. Conclusion: Most patients with functioning gonadotroph pituitary adenoma present with large tumors that are detected based on the occurrence of symptoms of compression that result from the enlarging sellar mass. Most patients, particularly men and postmenopausal women, do not develop symptoms of hormone excess and the lack of symptomatology results in delay in diagnosis. Our patients presented with significant polycythemia which resulted from excess testosterone and could have prompted earlier tumor detection if he had presented in an earlier stage. The incidence of polycythemia in male patients with functioning gonadotroph pituitary adenoma has previously been described in the literature in a few case reports.

Cerebellar Hemangioblastoma Management in Pregnancy: A Case Report [20H

INTRODUCTION: We present a rare case of sporadic cerebellar hemangioblastoma diagnosed in pregnancy. METHODS: A 37-year-old gravida 3 para 2002 with no significant history reported nausea and throbbing, bitemporal headaches at her 15 week visit. They were most bothersome in the evenings and upon waking. At her 21 week visit she reported persistent headaches with new onset dizziness. An echocardiogram and laboratory studies were normal. She was referred to neurology. At 23 weeks she had right and left beating nystagmus on exam and an unsteady gait. MRI revealed a 4 cm cystic lesion within the right cerebellum, mass effect on the fourth ventricle, and mild hydrocephalus with tonsillar and upward transtentorial herniation consistent with a hemangioblastoma. Neurosurgery planned urgent craniotomy and resection. RESULTS: The patient underwent uncomplicated surgery and pathology confirmed hemangioblastoma. Her postoperative course was uneventful with reassuring fetal status throughout admission. Her balance was markedly improved and neurologic exam was normal four weeks postoperatively. Testing for Von Hippel-Lindau syndrome was negative. The remainder of her pregnancy was uncomplicated. The team determined that she was a candidate for vaginal delivery. She underwent elective induction of labor at 39 weeks and progressed to vaginal delivery of a healthy female infant weighing 3370 g with Apgar scores of 9 and 9. She was discharged home with her infant on postpartum day one. CONCLUSION: Headaches, vomiting, and dizziness are common in pregnancy. Patients with severe, unrelenting symptoms require imaging and neurology consultation. Excellent outcomes are possible with a multidisciplinary approach involving obstetrics, maternal fetal medicine, neurology, and neurosurgery.