Central Pons Research Articles

Hypophysitis and central nervous system involvement in association with Sjögren’s syndrome along with hypoparathyroidism: a case report

BackgroundPatients with autoimmune diseases can develop multiple autoimmune diseases over a long period of time, and the presence of more than one autoimmune disease in a single patient is defined as polyautoimmunity. Polyautoimmunity may be clinical evidence that autoimmune diseases share similar immunological mechanisms.Case presentationWe report a 30-year-old woman with a unique combination of autoimmune diseases predominantly affecting the central nervous system, with hypoparathyroidism, hypophysitis, medulla involvement, and pons and temporal lobe involvement associated with primary Sjögren's syndrome (pSS), occurring independently over a long period. The patient who had a history of muscle cramps and one seizure incident, presented with vomiting and blurred vision. She was diagnosed with hypophysitis and hypoparathyroidism with calcifications in the basal ganglia and cerebellum. She recovered after four months of corticosteroid treatment for hypophysitis and was started on treatment for hypoparathyroidism. Eight months later, she developed vomiting, hiccups, vertigo, and ataxia with a focal lesion in the medulla. She recovered with immunosuppressive treatment for 2 years. Fifty-eight months after the onset of hypophysitis, she developed diplopia and dry mouth and eyes. MRI showed infiltrative lesions in the left pons and left temporal lobe. Based on positive anti-Sjögren's syndrome-related antigen A antibodies and low unstimulated whole salivary flow rate, pSS was diagnosed. She received corticosteroids and continued mycophenolate mofetil treatment with recovery of neurological symptoms.ConclusionThis case highlights the need for long-term follow-up to detect autoimmune disease processes involving various organs.

Expanding the reach of the trans-middle cerebellar peduncle approach: pontine cavernous malformations, tissue transgression beyond the safe entry zone, and the invisible triangle.

In the authors' microsurgical experience, the trans-middle cerebellar peduncle (MCP) approach to the lateral and central pons has been the most common approach to brainstem cavernous malformations (BSCMs). This approach through a well-tolerated safe entry zone (SEZ) allows a wide vertical or posterior trajectory, reaching pontine lesions extending into the midbrain, medulla, and pontine tegmentum. Better understanding of the relationships among lesion location, surgical trajectory, and long-term clinical outcomes could determine areas of safe passage. A single-surgeon cohort study of all primary trans-MCP BSCM resections was conducted from July 1, 2017, to June 30, 2021. Preoperative and postoperative MR images were independently reviewed by 3 investigators blinded to the intervention, using a standardized rubric to define BSCM regions of interest (ROIs) involved with a lesion or microsurgical tract. Statistical testing, including the chi-square test with the Bonferroni correction, logistic regression, and structural equation modeling, was performed to analyze relationships between ROIs and clinical outcomes. Thirty-one patients underwent primary trans-MCP BSCM resection during the study period. The median age was 50 years (IQR 24-49 years); 19 (61%) patients were female, and 12 (39%) were male. Seven (23%) patients had familial cavernous malformation syndromes. The median follow-up was 9 months (range 6-37 months). At the last follow-up, composite neurological outcomes were favorable: 22 (71%) patients had 0 (n = 12, 39%) or 1 (n = 10, 32%) major persistent deficit, 5 patients (16%) had 2 deficits, 2 (7%) had 3 deficits, and 1 patient each (3%) had 4 or 6 deficits. Unfavorable composite outcomes were significantly associated with lesions (OR 7.14, p = 0.04) or surgical tracts (OR 12.18, p < 0.001) extending from the superior cerebellar peduncle (SCP) into the contralateral medial midbrain. The ipsilateral dorsal pons was the most frequently implicated ROI involving a surgical tract and the development of new postoperative deficits. This region involved the rhomboid pontine territory and transgression of the pontine tegmentum (OR 7.53, p < 0.001). Structural equation modeling supported medial midbrain and pontine tegmentum transgression as the primary drivers of morbidity. Trans-MCP resection is a safe and effective treatment for BSCMs, including lesions with marked superior or inferior ipsilateral extension. Two trajectories are associated with increased neurological risk: first, a superomedial trajectory to lesions extending into the midbrain that transgresses the SCP, its decussation, or both; and second, a posteromedial trajectory to lesions extending into the pontine tegmentum. The corticospinal tract, SCP, and pontine tegmentum form an invisible triangle within the pontine white matter tolerant of transgression. When the surgeon works within this triangle, most deep pontine BSCMs, including large lesions, those with contralateral or posterior extension, and others extending into the midbrain and medulla, can be resected safely with the trans-MCP approach.

From wernicke-korsakoff to central pontine myelinolysis: the potentially irreversible risks of alcohol use

Introduction Sustained alcohol intake, when combined with incomplete treatment, can result in chronic structural changes in the Central Nervous System, including generalized cortical and cerebellar atrophy, amnesic syndromes like Korsakoff’s syndrome, and white matter disorders such as Central Pontine Myelinolysis and Marchiafava-Bignami syndrome. It is crucial to prevent these complications due to their potential for irreversible and debilitating consequences. For Wernicke-Korsakoff syndrome, early recognition and thiamine administration for prevention are paramount, as it arises from thiamine deficiency due to malnutrition caused by persistent alcohol use. In the case of Central Pontine Myelinolysis, which is caused by abrupt fluctuations in serum osmolality, controlled sodium correction is essential.ObjectivesThrough a clinical case and a review of published literature, this study aims to reflect on the importance of preventing neurological injuries associated with chronic alcohol consumption, specifically Wernicke-Korsakoff Syndrome and Central Pontine Myelinolysis.Methods A literature review was conducted by searching for articles on PubMed using the terms “Alcohol Use Disorder,” “Wernicke-Korsakoff syndrome,” and “Central pontine myelinolysis.” A clinical case is presented, featuring a 50-year-old patient with alcohol use disorder who developed Wernicke-Korsakoff syndrome and Central Pontine Myelinolysis. Considering this case, we reflect on the primary approaches that could have been beneficial in preventing these complications and propose a straightforward method for doing so.Results A 50-year-old patient presented with poor general condition, characterized by low weight, significant loss of strength in the limbs and arms, and incoherent speech with anterograde amnesia and confabulation. This condition had progressed to a point where the patient could no longer walk, perform basic self-care tasks such as bathing, dressing, and eating independently, underscoring the severity of his condition. The diagnoses of Wernicke-Korsakoff syndrome and Central Pontine Myelinolysis were established based on clinical manifestations and the presence of hyperintense lesions observed in the central pons on T2/FLAIR axial MRI scans. This clinical case highlights the importance of proper and precocious prevention of complications in patients with alcohol use disorder. The foremost step in preventing these complications is to treat alcohol dependence effectively, even when faced with patient resistance. It’s vital to remain vigilant about potential complications and implement suitable prophylactic measures.ConclusionsThe devastating effects of complications arising from Alcohol Use Disorder, such as Wernicke-Korsakoff syndrome and Central Pontine Myelinolysis, underscore the importance of enhanced attention that clinicians should provide when approaching these patients at all clinical interactions.Disclosure of InterestNone Declared

Harnessing axonal transport to map reward circuitry: Differing brain-wide projections from medial prefrontal cortical domains.

Neurons project long axons that contact other distant neurons. Neurons in the medial prefrontal cortex project into the limbic system to regulate responses to reward or threat. Diminished neural activity in prefrontal cortex is associated with loss of executive function leading to drug use, yet the specific circuitry that mediate these effects is unknown. Different regions within the medial prefrontal cortex may project to differing limbic system nuclei. Here, we exploited the cell biology of intracellular membrane trafficking, fast axonal transport, to map projections from two adjacent medial prefrontal cortical regions. We used Mn(II), a calcium analog, to trace medial prefrontal cortical projections in the living animal by magnetic resonance imaging (MRI). Mn(II), a contrast agent for MRI, enters neurons through voltage-activated calcium channels and relies on kinesin-1 and amyloid-precursor protein to transport out axons to distal destinations. Aqueous MnCl2 together with fluorescent dextran (3--5nL) was stereotactically injected precisely into two adjacent regions of the medial prefrontal cortex: anterior cingulate area (ACA) or infralimbic/prelimbic (IL/PL) region. Projections were traced, first live by manganese-enhanced MRI (MEMRI) at four time points in 3D, and then after fixation by microscopy. Data-driven unbiased voxel-wise statistical maps of aligned normalized MR images after either ACA or IL/PL injections revealed statistically significant progression of Mn(II) over time into deeper brain regions: dorsal striatum, globus pallidus, amygdala, hypothalamus, substantia nigra, dorsal raphe and locus coeruleus. Quantitative comparisons of these distal accumulations at 24h revealed dramatic differences between ACA and IL/PL injection groups throughout the limbic system, and most particularly in subdomains of the hypothalamus. ACA projections targeted dorsomedial nucleus of the hypothalamus, posterior part of the periventricular region and mammillary body nuclei as well as periaqueductal gray, while IL/PL projections accumulated in anterior hypothalamic areas and lateral hypothalamic nuclei as well as amygdala. As hypothalamic subsegments relay CNS activity to the body, our results suggest new concepts about mind-body relationships and specific roles of distinct yet adjacent medial prefrontal cortical segments. Our MR imaging strategy, when applied to follow other cell biological processes in the living organism, will undoubtedly lead to an expanded perspective on how minute details of cellular processes influence whole body health and wellbeing.

Osmotic Demyelination Syndrome: A Rare Clinical Image.

The term "Osmotic Demyelination Syndrome" (ODS) is synonymous with central pontine myelinolysis (CPM), denoting a condition characterised by brain damage, particularly affecting the white matter tracts of the pontine region. This damage arises due to the rapid correction of metabolic imbalances, primarily cases of hyponatremia. Noteworthy triggers encompass severe burns, liver transplantations, anorexia nervosa, hyperemesis gravidarum, and hyperglycaemia, all linked to the development of CPM. Clinical manifestations encompass a spectrum of signs and symptoms, including dysphagia, dysarthria, spastic quadriparesis, pseudobulbar paralysis, ataxia, lethargy, tremors, disorientation, catatonia, and, in severe instances, locked-in syndrome and coma. A recent case involving a 45-year-old woman illustrates these complexities. Upon admission to the Medicine Intensive Care Unit, she presented with symptoms indicative of diminished responsiveness and bilateral weakness in the upper and lower limbs. Of significance, the patient had a pre-existing medical history of hyperthyroidism. Extensive diagnostic investigations were undertaken, revealing compelling evidence of profound hyponatremia through blood analyses. Furthermore, magnetic resonance imaging (MRI) was performed, unveiling conspicuous areas of abnormal hyperintensity located in the central pons, intriguingly accompanied by spared peripheral regions. These radiological findings align with the characteristic pattern associated with osmotic demyelination syndrome, illuminating the underlying pathology.

52 Stable Cognitive Impairment and Increased Psychiatric Symptoms in a Patient with Neuropathologically Progressive Fahr’s Disease After 2-Year Repeated Neuropsychological Assessment

Objective:Fahr’s disease is a rare genetic neurological disorder characterized by abnormal idiopathic calcification of the basal ganglia, typically with extrapyramidal symptoms, speech difficulty, behavioral disturbances, and progressive neurologic dysfunction. A small number of case reports have explored the neuropsychological profile of Fahr’s disease patients, and even fewer have followed the course of neuropsychological functioning over time.Participants and Methods:A 53-year-old Asian woman presented for a neuropsychological reevaluation (2021) after experiencing a recurrence of memory difficulties and mood changes. Relevant medical history was significant for systemic lupus erythematosus (SLE) and Fahr’s disease. Following an episode of acute confusion, the patient underwent a head CT (2019) which revealed extensive calcification throughout the cerebellum, central pons, and periventricular and subcortical white matter, suggestive of Fahr’s disease. Two months later, she underwent an initial neuropsychological evaluation (2019), which demonstrated prominent attention and processing speed deficits contributing to variably impaired new learning and memory along with spatial planning and problem-solving difficulties. The etiology of her cognitive deficits was determined to likely reflect metabolic and immune instability, consistent with her history of SLE and Fahr’s disease. An updated CT (2021) revealed increased calcification throughout the bilateral corona radiata, basal ganglia, cerebellar hemispheres, and midbrain, which was determined to be compatible with progressive Fahr’s disease.Results:The patient’s neurocognitive profile from current neuropsychological testing (2021) was marked by notable deficits in attention and processing speed, delayed memory, problem solving, visuospatial reasoning, and motor dexterity. Compared to her initial evaluation, her cognitive profile remained stable save for a slight decline in processing speed. The largest change was seen within the psychiatric domain. Self-reported depressive symptoms involving anhedonia, concentration difficulties, and anxiety symptoms involving nervousness and tension were more pronounced in her current evaluation. In addition, she endorsed an increase in apathy compared to her initial evaluation.Conclusions:The cognitive profile seen in this patient is consistent with the current literature relating to the clinical sequelae of Fahr’s disease in patients that eventually went on to develop dementia. Despite an increase in brain calcification seen on CT imaging over an 18-month interval, the patient’s neurocognitive profile remained relatively stable. An increase in psychiatric symptoms appeared to be the most prominent change over repeated neuropsychological assessment, which elucidates the heterogenous course of Fahr’s disease from a neuropsychological perspective. Further exploration of this disorder is warranted to better understand the clinical progression of symptoms over time.

Pathologically- confirmed Osmotic Demyelination in the Case of Clinically and Radiographically Progressive Encephalopathy due to Liver Disease and Malnutrition. (P4-4.007)

<h3>Objective:</h3> To report a case of osmotic demyelination syndrome (ODS) in a patient with alcoholic liver cirrhosis and normal sodium levels. <h3>Background:</h3> Correction of hyponatremia and chronic alcoholism have been identified as the most frequent conditions associated with osmotic demyelination syndrome (ODS). <h3>Design/Methods:</h3> N/A <h3>Results:</h3> A 77 year old female with a history of gastric bypass surgery complicated by copper deficiency, and alcoholic liver cirrhosis was hospitalized for failure to thrive and encephalopathy. Initial MRI brain showed non-specific white matter hyperintensities. Mental status continued to worsen despite treatment with IV thiamine, copper (low 41 mcg/dL, normal 80–155), and antibiotics for presumed peritonitis. Repeat MRI brain showed T2 signal abnormality within the central pons and midbrain with sparing of the anterior pontine periphery. Serum sodium on presentation was 138 mEq/L (normal 135–145) and fluctuated during hospitalization between 133–150. She subsequently developed hypoxemic respiratory failure requiring intubation and kidney failure requiring hemodialysis. Despite all aggressive measures she remained severely encephalopathic with repeat MRI brain revealing new confluent large areas of T2 hyperintensity in the centrum semiovale and corona radiata. T2 hyperintensity in the midbrain and central pons remained unchanged. The family declined lumbar puncture and pursued comfort measures. Autopsy was performed which revealed extensive demyelination in the midbrain tegmentum with relative preservation of axons; capillary and arterial cerebral amyloid angiopathy most prominent in the cerebellar leptomeninges, extending into the cerebellar cortex; and moderate Alzheimer’s type 2 astrocytosis in the basal ganglia consistent with prior history of hepatic encephalopathy. <h3>Conclusions:</h3> Our patient was diagnosed with toxic-metabolic, hepatic encephalopathy and had progressive radiological findings suggestive of ODS. Demyelination was confirmed on autopsy with copathology including cerebral amyloid angiopathy and findings of hepatic encephalopathy. ODS must be considered with progressive MRI findings in the setting of encephalopathy with liver disease and malnutrition despite absence of significant serum sodium fluctuations. <b>Disclosure:</b> Dr. Hanson has nothing to disclose. Dr. Bronstein has nothing to disclose. Dr. Fong has nothing to disclose. Dr. Ashikian has nothing to disclose.

Myélinolyse centropontine réversible sans hyponatrémie chez un enfant atteint de leucémie aigue lymphoblastique: à propos d’un cas

Central pontine myelinolysis is a demyelinating disorder mainly affecting the central pons. In some cases, it is associated with extrapontine myelinolysis. It is usually caused by rapid correction of hyponatremia and osmotic shock. We here report the case of a 3.5-year-old girl diagnosed with acute lymphoblastic leukemia admitted to our Oncology Unit with neutropenic fever and diarrhea. Laboratory tests showed mild neutropenia, normochromic normocytic anemia. Electrolyte tests were normal without hyponatremia. She received antibiotic therapy with Metronidazole. Five days later, she developed flaccid quadriparesis with mutism. Computerized tomography (CT) scan was normal, cerebrospinal fluid (CSF) examination was normal (there was no evidence of leukemic cells) and ophthalmological examination did not show any abnormalities. Brain MRI found hyperintense signal in the pons. The child improved without specific treatment, and clinical and complete neurological recovery was noted. This case highlights that myelinolysis can occur under some circumstances not related with hyponatremia such as malignancy, chemotherapy.

Radiation-induced Brain Calcification Leads to L-dopa-resistant Parkinsonism and Cerebellar Ataxia

We experienced a young patient who presented with progressive parkinsonism and cerebellar ataxia. Brain magnetic resonance imaging revealed progressive brain calcification, expanding from the bilateral basal ganglia to the central pons, caused by a delayed reaction to the radiation therapy that she had received to treat craniopharyngioma 14 years earlier. Heterogeneous clinical symptoms due to radiation-induced brain calcification have been described, but parkinsonism has never been reported. While dopamine transporter-single photon emission computed tomography revealed only slight damage to the dopaminergic striatal pathway, the extension of calcification to the periventricular white matter was likely responsible for her parkinsonism.

With a grain of salt; A cautionary reminder. A case report of osmotic demyelination syndrome

Osmotic Demyelination Syndrome (ODS) is a demyelinating disease typically seen in the central pons, often associated with a rapid correction of hyponatraemia.

How I do it: horizontal fissure approach to the middle cerebellar peduncle.

The horizontal fissure approach is a workhorse for brainstem lesions in the central and dorsolateral pons and middle cerebellar peduncle (MCP). The cerebellopontine fissure is a V-shaped fissure with a superior and inferior limb between the cerebellum, pons, and MCP. The horizontal or petrosal fissure is at the apex of the cerebellopontine fissure and extends laterally to divide the petrosal surface of the cerebellum into superior and inferior parts. Splitting this fissure exposes the posterolateral aspect of the MCP without excessive retraction or transgression of the cerebellum. We demonstrate and describe the horizontal fissure operative approach to the middle cerebellar peduncle for resection of a pontine cavernoma with illustrative figures and operative video. Splitting the horizontal (petrosal) fissure of the cerebellum brings the middle cerebellar peduncle into view behind the root entry zone of the trigeminal nerve, providing an expanded, safe corridor to the central and dorsolateral pons.

Extra-pontine osmotic demyelination syndrome and membranous nephropathy

Rapid correction of hyponatremia can increase the risk of osmotic demyelination syndrome (ODS) involving the central pons and extrapontine (basal ganglia) brain areas. We described a patient who developed extrapontine ODS after rapid correction of profound hyponatremia in the setting of severe nephrotic syndrome (NS).

Abstract P717: Central Midbrain Strokes Are More Liable for Respiratory Failure Then Other Brain Stem Strokes

Background: Respiratory centers are known to be present in the central medulla oblongata and pons. There are multiple complex respiratory networks involving these centers. The midbrain periaqueductal grey is believed to act as a regulator of the respiratory function. The effect of brain-stem strokes on respiration remains understudied. There is a lack of clear understanding of the anatomical influence of such strokes on respiration. We attempted to identify brain-stem locations with the highest liability for respiratory failure in case of stroke. Methods: We included all ischemic and hemorrhagic brain-stem strokes from our stroke-registry between 2016 and 2018 then performed univariate/multivariate regression-analyses on variables that might predict respiratory failure and the need for intubation. The brain stem was divided into nine locations (right lateral, central, left lateral in each of the midbrain, pons, and medulla oblongata). Results: Out of 128 brain-stem strokes of different sizes and etiologies, central midbrain strokes were the only significant and independent affected location associated with respiratory failure and endotracheal intubation (coefficient= 0.1256, 95%-CI= 0.0175, 0.2338, p= 0.023). R-squared was equal to 15% when only central midbrain strokes stayed in the model. Conclusions: While one might assume that central medullary and pontine strokes have the most impact on respiration; our results show that central midbrain is the most impactful, accounting for about 15% of respiratory instability associated with brain-stem strokes. This can be explained by the adaptive nature of respiratory circuits within the medulla and pons. Central periaqueductal grey within the midbrain controls the rate and depth of respiration and might not have the same flexibility present elsewhere.

Anterior pituitary gland T1 signal intensity is influenced by time delay after injection of gadodiamide

To test the hypothesis of washout from the anterior pituitary (AP) gland after serial injections of gadodiamide. We included 59 patients with history of at least 5 injections of gadodiamide. Values of mean signal intensity of the AP and of the central pons were measured on unenhanced sagittal T1-weighted images. AP-to-pons signal intensity ratios were calculated dividing the values of the AP by those of the pons. The measurements were performed using MR images acquired at four different time points including baseline (prior to any gadodiamide injection), minimum post-injection time delay, maximum post-injection time delay, and last available MR scans. Normalized ratios (i.e. ratios divided total volume of injected gadodiamide) were also calculated. To assess the difference between ratios, non-parametric Wilcoxon signed-rank test was applied. The correlations were tested with non-parametric Spearman correlation coefficient. A p-value < 0.05 was considered as statistically significant. A statistically significant increase of AP signal intensity was found by comparing the baseline scans with both the minimum time delay (p = 0.003) and maximum time delay scans (p = 0.005). We found significant higher normalized ratios for minimum post-injection time delay with respect to maximum post-injection time delay (p < 0.001). The normalized ratios demonstrated a statistically significant negative correlation with the post-injection time delay (r = − 0.31; p = 0.006). The findings of this study suggest that washout phenomena of retained/deposited gadolinium from the AP are influenced by the total injected volume and post-injection time delay.

The role of pontine lesion location in differentiating multiple sclerosis from vascular risk factor-related small vessel disease

Background: Differentiating multiple sclerosis (MS) from vascular risk factor (VRF)-small vessel disease (SVD) can be challenging. Objective and Methods: In order to determine whether or not pontine lesion location is a useful discriminator of MS and VRF-SVD, we classified pontine lesions on brain magnetic resonance imaging (MRI) as central or peripheral in 93 MS cases without VRF, 108 MS patients with VRF and 43 non-MS cases with VRF. Results: MS without VRF were more likely to have peripheral pons lesions (31.2%, 29/93) than non-MS with VRF (0%, 0/43) (Exp(B) = 29.8; 95% confidence interval (CI) = (1.98, 448.3); p = 0.014) but there were no significant differences regarding central pons lesions between MS without VRF (5.4%, 5/93) and non-MS with VRF patients (16.3%, 7/43) (Exp(B) = 0.89; 95% CI = (0.2, 3.94); p = 0.87). The presence of peripheral pons lesions discriminated between MS and VRF-SVD with 100% (95% CI = (91.8, 100)) specificity. The proportion of peripheral pons lesions in MS with VRF (30.5%, 33/108) was similar to that seen in MS without VRF (31.2%, 29/93, p = 0.99). Central lesions occurred in similar frequency in MS with VRF (8.3%, 9/108) and non-MS with VRF (16.3%, 7/43, p = 0.15). Conclusion: Peripheral pons lesion location is a good discriminator of MS from vascular lesions.

NCMP-04. OPSOCLONUS-MYOCLONUS SYNDROME ASSOCIATED WITH CONTACTIN-ASSOCIATED PROTEIN-LIKE 2 AUTOANTIBODY IN THE SETTING OF NON-SMALL CELL LUNG CARCINOMA

Abstract INTRODUCTION Opsoclonus–myoclonus syndrome (OMS) is a rare neurological disorder characterized by spontaneous multidirectional saccadic eye movements, myoclonus, and ataxia. It is suspected to be an autoimmune mediated process precipitated by a paraneoplastic or infectious trigger. Among the pediatric population, OMS is often associated with neuroblastoma and anti-Hu antibody. Paraneoplastic syndromes in adults manifesting as OMS are most commonly described in breast, gynecological and small cell lung cancer with associated anti-neuronal antibodies (ANNA-2; anti-Ri). We describe the first case of OMS associated with lung adenocarcinoma and Contactin-associated protein-like 2 (Caspr2) antibody. CASE REPORT: A 46-year-old-woman with history of limited small cell lung cancer (SCLC) status-post chemotherapy with cisplatin and etoposide and prophylactic whole brain radiation, presented with new onset gait instability, nausea, vomiting, vertigo, and opsoclonus. Two months prior to her presentation, a chest computed topography (CT) revealed no evidence of recurrent disease. Brain MRI revealed T2 hyperintensities in the white matter and central pons presumably related to chronic small vessel ischemic changes, unchanged from prior imaging. CSF studies revealed normal cell count, glucose, and protein. Gram stain, culture and meningoencephalitis panel were negative. Her paraneoplastic panel revealed a positive Caspr2 antibody. A repeat CT chest for cancer surveillance revealed a new lung mass. Immunotherapy treatment included plasmapheresis, corticosteroids, and intravenous immunoglobulin therapy. She underwent a biopsy of the new pulmonary lesion and the pathology was consistent with non-small cell lung carcinoma. Her symptoms slowly improved after treatment. Unfortunately, her disease progressed on repeat PET scan despite surgical resection of the lung mass. She ultimately elected for hospice. CONCLUSION We report a novel antibody, Caspr2, occurring in association with paraneoplastic OMS. While SCLC is the most common neoplasm associated with OMS among adults, non-small cell lung carcinoma should be considered. Prompt immunotherapy and neoplasm treatment may result in improvement.

Increased T1 Signal Intensity of the Anterior Pituitary Gland on Unenhanced Magnetic Resonance Images After Chronic Exposure to Gadodiamide.

The aim of this study was to assess the signal intensity of the anterior pituitary (AP) gland on unenhanced T1-weighted images in patients with history of serial intravenous injections of gadodiamide and normal renal function. We included 53 patients who had undergone at least 5 injections of gadodiamide and a control group of 15 subjects who underwent at least 5 brain magnetic resonance imaging without gadolinium-based contrast agents. Using unenhanced sagittal T1-weighted images, values of mean signal intensity of the AP and of the central pons were obtained. Anterior pituitary-to-pons signal intensity ratios were calculated dividing the values of the AP by those of the pons. Then, the ratios were compared between the first and the last magnetic resonance imaging scans for all the subjects. To assess the difference between the first and the last ratios, nonparametric Wilcoxon signed-rank test with Monte Carlo resampling was applied. A P value less than 0.05 was considered as statistically significant. The comparison between the first and the last scan revealed a statistically significant increase of AP-to-pons ratio in the last scan for the gadolinium-exposed group (P < 0.001), whereas nonsignificant results were found for the control group (P = nonsignificant). We found an increased signal intensity of the AP on unenhanced T1-weighted images in patients with history of serial intravenous injections of gadodiamide and normal renal function, suggesting gadolinium deposition or long-term retention within the AP gland. Our findings need to be confirmed by further histochemical analysis of AP gland tissue samples.

Central Pontine Myelinolysis After Non-Aneurysmal Perimesencephalic Subarachnoid Hemorrhage: Case Report and Review of the Literature

Central pontine myelinolysis (CPM) is one of the encephalopathy that results from extreme fluctuations in serum sodium concentration and plasma osmolality. CPM after non aneurysmal perimesencephalic subarachnoid hemorrhage (NPSAH) is very rare. A 53-year-old female patient aggravated her instabilty 3 weeks after treatment of after NPSAH. Brain CT showed a prominent low-density lesion in the central pons. Vasospasm, pontine infarct, multiple sclerosis must be excluded after subarachnoid hemorrhage. Her brain magnetic resonance imaging (MRI) of the brainstem revealed CPM. The peripheral fiber sparing, central trident appearance was observed. Peripheral fiber sparing is more prominent, but central trident is disappearing at long-term follow-up MRI. CPM can develop even after NPSAH as well as aneurysmal subarachnoid hemorrhage. Trident pattern in pons area and peripheral fiber sparing is differential diagnosis with vasospasm, cerebral infarct and multiple sclerosis after NPSAH. Keywords: Subarachnoid hemorrhage; Hyponatremia; Central pontine demyelination; Demyelination.

EFFECT OF MEMANTINE ON BEHAVIORAL AND PSYCHOLOGICAL SYMPTOMS OF DEMENTIA (BPSD) OF ALZHEIMER'S DISEASE - STUDY OF CHANGES IN CEREBRAL BLOOD FLOW BY SPECT IMAGING-

Introduction The mechanism of the onset of BPSD associated with Alzheimer's disease (AD) is unclear, and the incidence and frequency of these symptoms are difficult. We conducted a retrospective study on sites of decreased cerebral blood flow(CBF) during onset along with sites of increased blood flow following administration of memantine (MEM) for three BPSD parameters consisting of “agitation/irritability”, “hallucinations/delusions” and “wandering/appeal to return home” using SPECT imaging. Methods The study was targeted at 41 outpatients diagnosed with AD and presenting with BPSD (agitation/irritability: 11 cases, hallucinations/delusions: 19 cases, wandering/appeal to return home: 11 cases). Sites of decreased CBF during onset of BPSD were extracted using the statistical parametric mapping (SPM8) followed by an examination of their responsible lesions. SPECT was repeated about six months after additional administration of MEM followed by an examination of the correlation between sites of increased CBF attributable to MEM and improvement of symptoms. Results • 1 With respect to agitation/irritability, decreases in CBF were observed in the dorsolateral prefrontal cortex bilaterally, angular gyrus, right-dominant parietal association area and posterior cingulate gyrus, while increases in CBF following administration of MEM were observed in the orbital gyri bilaterally, right occipital lobe and left ventral prefrontal cortex. • 2 With respect to hallucinations/delusions, decreases in CBF were observed in the posterior cingulate gyrus, occipital lobe and lt.-dominant parietal association area, while increases in CBF following administration of MEM were observed in the parietal lobe, orbital gyrus and prefrontal cortex bilaterally. • 3 With respect to wandering/appeal to return home, decreases in CBF were observed in the mid-cerebellum, occipital lobe and parietal lobe, while increases in CBF following administration of MEM were observed in mid-cerebellum, left parietal lobe, occipital lobe, posterior cingulate gyrus, prefrontal cortex and ventral central pons. Conclusions There was suggested to be a correlation between sites of CBF following administration of MEM and improvement of BPSD.

Evaluation of Gadolinium Deposition in the Brain After MR Arthrography.

Although significant investigation has been done into the deposition of gadolinium in the brains of patients receiving IV gadolinium, there is little research concerning nonintravenous uses of gadolinium, specifically in conjunction with musculoskeletal MR arthrography. Although small in volume, intraarticular administration is an off-label use of gadolinium, necessitating careful scrutiny for patient safety. Thus, we investigated the relationship between intraarticular gadolinium administration during MR arthrography and the presence of intracranial gadolinium deposition on subsequent brain MRI. An institutional review board-approved retrospective study was performed of patients with no history of gadolinium exposure who underwent MR arthrography from 2006 to 2016 followed by an unenhanced brain MRI examination. ROIs were manually placed within bilateral dentate nuclei (DN), bilateral globus pallidi (GP), bilateral thalami, bilateral middle cerebral peduncles (MCP), and the central pons (CP) on T1-weighted sequences. The left and right ROIs were averaged, and ratios of signal intensity were calculated for DN/MCP, DN/CP, GP/MCP, GP/CP, thalamus/MCP, and thalamus/CP. Similar ROIs were placed and ratios calculated for age-matched control subjects who had a history of brain MRI but no prior gadolinium exposure. We used t testing to compare signal intensity ratios between patients and control subjects. A total of 31 patients met the inclusion criteria. We found no significant difference in signal intensity between patients and control subjects for DN/MCP (p = 0.40), DN/CP (p = 0.76), GP/MCP (p = 0.51), GP/CP (p = 0.86), thalamus/MCP (p = 0.93), and thalamus/CP (p = 0.30). No association was found between intraarticular gadolinium administration for MR arthrography and detectable gadolinium deposition within the brain.