Antiglutamate acid decarboxylase seropositive brain stem encephalitis.

This is a case of a previously healthy female in her fourties presenting with a subacute presentation of bilateral horizontal gaze restriction, with bilateral lower motor facial palsy. The patient's daughter has type 1 diabetes. On investigation, the patient's MRI revealed a lesion in the dorsal medial pons. Cerebrospinal fluid analysis revealed albuminocytological dissociation, with a negative autoimmune panel. The patient was treated with intravenous immunoglobulin, and methylprednisolone for a total of 5 days and showed mild improvement. The patient had raised serum antiglutamic acid decarboxylase (anti-GAD) levels, and the final diagnosis of GAD seropositive brain stem encephalitis was made.

Eye pain caused by brainstem infarction.

Sudden pain of the eye, nose or face can be a symptom of stroke located to the pons. This case report is about a 73-year-old women with acute debut of right-sided hemiparesis, ataxia, gait disturbance, dysarthria, hemisensory defects and contralateral burning eye pain. MRI showed acute ischaemia of the left pons. The case adds to the growing literature of this rare presentation of posterior circulation stroke.

Nonpainful Trigeminal Neuropathy Associated with a Solitary Pontine Lesion: A Case Series.

A solitary pontine lesion (SPL) is a single brainstem lesion on the trigeminal nerve pathway without any other central nervous system lesion. This research aimed to investigate the demographic and clinical features of nonpainful TNO patients with SPL and identify the most frequently affected anatomical areas using lesion mapping techniques. Demographic and clinical features were retrospectively reviewed from the patients' charts. Brain lesions were mapped using MRIcroGL software. The study included 6 patients (three females and three males) with an SPL. The median age of the patients was 57 (range: 46-68) years. Cranial MRI displayed lesions in the dorsolateral pons and the cerebellar peduncle. The lesion mapping revealed that the lesions were on the trigeminal nerve pathway. SPL is an uncommon cause of TNO. Nonpainful SPL patients have demographic, clinical, and radiological features similar to those of painful SPL patients. The lesion mapping showed that the same brainstem areas are affected in painful and nonpainful SPL patients.

Occult Breast Cancer With Anti-Ri Antibody Positivity and Pontine Hot Cross Bun Sign.

This case report describes a 40-year-old woman with occult breast cancer with anti-Ri antibody positivity and pontine hot cross bun sign.

CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent.

OBJECTIVE: We report a case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) who achieved durable and steroid-free remission after IV cladribine. METHODS: A 25 year-old man presented with progressively worsening headaches, polydipsia, dysarthria, diplopia and vertigo, and obtundation requiring respiratory support. CSF revealed lymphocytosis, and MRI revealed a perivascular pattern of punctate enhancement involving the pons. An extensive workup for inflammatory, autoimmune, infective, and malignant explanations was unrevealing. He responded dramatically to steroids, compatible with CLIPPERS as a diagnosis of exclusion. Attempts to wean prednisone over the ensuing year resulted in 2 clinical relapses and persistent punctate enhancement. Given significant steroid side effects, steroid-sparing agents were considered. RESULTS: IV cladribine IV (0.0875 mg/kg adjusted body weight daily × 4 days at 0, 4, 8, and 16 months) was selected, given its favorable side effect profile including lower risks of malignancy and infertility and the potential for long-lasting effects. The only side effect was short-term fatigue at the time of infusion. At 20 months after cladribine initiation, he was able to wean-off prednisone altogether. Now at 33 months, he remains in clinical and MRI remission. DISCUSSION: Cladribine is a rational candidate steroid-sparing treatment for presumed neurologic autoimmune conditions such as CLIPPERS. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that cladribine is a steroid-sparing treatment consideration in CLIPPERS.

MULTIPLE ACUTE POSTERIOR CIRCULATION STROKE WITH LESIONS IN THE PONS AND BOTH HEMISPHERES OF THE CEREBELLUM ASSOCIATED WITH OVARIAN HYPERSTIMULATION SYNDROME: A CASE REPORT OF A WHITE EUROPEAN ADULT IN UKRAINE.

We have presented a brief literature overview of the disease, supported by a clinical case of multiple acute posterior circulation strokes with lesions in the pons and both hemispheres of the cerebellum associated with ovarian hyperstimulation syndrome in a white young European adult in Ukraine. Specific features of posterior circulation stroke associated with ovarian hyperstimulation syndrome were determined, analyzed, and described. Complex posterior circulation cerebral infarction in the pons and both hemispheres of the cerebellum associated with ovarian hyperstimulation syndrome has not been reported before but has devastating consequences for both mother and fetus. Strokes in patients with OHSS must be timely prevented, promptly diagnosed, and treated to avoid high morbidity and mortality associated with it.

Morphometric examination of trigeminal nerve and its adjacent structures in patients with trigeminal neuralgia: a case-control study.

BACKGROUND: Morphological differences that can lead the trigeminal nerve to neurovascular conflict and a new solitary pontine lesion are associated with the pathogenesis of trigeminal neuralgia (TN). In this case-control study, we aimed to contribute to the current discussions about the pathogenesis of TN by investigating the anatomical structures that may have an effect on the morphometric parameters of the trigeminal nerve. METHODS: This study included 25 patients with TN followed up for pain in the Department of Algology, Faculty of Medicine, and 25 age- and gender-matched controls. We performed morphometric measurements including the length and volume of the trigeminal nerve, cerebellopontine cistern, pons, and posterior fossa in the MRIs of these individuals. Comparative analyses were performed for the mean of the affected and unaffected sides of the TN patients and the right, left, and both sides of the control group. RESULTS: In patients with TN, on the affected side, length and volume of the trigeminal nerve and cerebellopontine cistern volume were found smaller than controls (p < 0.05). Pons volume was higher in patients with TN compared to controls (p < 0.05). The length of the affected nerve was significantly related to prepontine cistern length and cerebellopontine cistern volume (p < 0.05). DISCUSSION: The cerebellopontine cistern volume has a significant impact on the morphometric characteristics of the trigeminal nerve. Especially, whether the increase in the volume of pons causes a decrease in the volume of cerebellopontine cistern should be clarified with further research.

Do hypokyphotic adolescent idiopathic scoliosis patients treated with Ponte osteotomy obtain a better clinical efficacy? A preliminary retrospective study.

STUDY DESIGN: A retrospective case-control study. OBJECTIVE: To evaluate whether Ponte osteotomy improves thoracic kyphosis and to determine its clinical efficacy in hypokyphotic adolescent idiopathic scoliosis (AIS). METHODS: Eighty consecutive Lenke type 1 AIS patients with hypokyphotic curves who underwent posterior spinal fusion by one spine surgeon at a single institution were recruited. According to whether Ponte osteotomy was performed, the patients were divided into two groups. The preoperative, immediate, one-year postoperative, and two-year postoperative radiographs were analyzed. The demographic characteristics, surgical information, radiographic parameters, Scoliosis Research Societye-22 (SRS-22) questionnaire, and complications were compared. RESULTS: The sagittal alignment and coronal alignment were both improved in the Ponte group and the control group postoperatively. There was no significant difference in the preoperative parameters between the two groups, except the TL/L, CB, and LL. Significant differences were found in the MT (15.18° ± 2.84° vs. 20.33° ± 3.75°, P < 0.001) and TK (24.23° ± 2.71° vs. 19.93° ± 2.38°, P < 0.001) at the two-year follow-up. The Ponte group had a longer operation time and more intraoperative blood loss. No significant difference was observed between the groups in the SRS-22 scores at the final follow-up. CONCLUSIONS: Ponte osteotomy could obtain better coronal correction and sagittal contour restoration in AIS patients with hypokyphosis. However, Ponte osteotomies might lead to more intraoperative blood loss and longer operation time. Moreover, no discrepancy was found in the postoperative health-related quality of life of the included patients. Therefore, we considered that the Ponte osteotomy may be an alternative method to restore the desired thoracic kyphosis, which needs further study.

FDG PET/CT Aiding in Early Diagnosis of Central Pontine Myelinolysis.

ABSTRACT: We report a 70-year-old man with central pontine myelinolysis who presented in the emergency department with recent onset altered sensorium and tremors (Glasgow Coma Scale score, 13). Laboratory findings revealed hyponatremia and hypokalemia. MRI brain was unremarkable. Subsequently, patient was referred for FDG PET/CT to rule out malignancy or paraneoplastic syndrome. FDG PET revealed focal radiotracer uptake in the pons without any underlying CT abnormality consistent with central pontine myelinolysis, thus aiding in early diagnosis where conventional imaging modality was unremarkable.

Sixteen-Syndrome in a Young Patient with Systemic Lupus Erythematosus.

Sixteen syndrome is rare variant of one and a half syndrome resulting from lesion affecting bilateral dorsal pontine structures. This report describes a case of 16 syndrome in systemic lupus erythematosus (SLE). A 28 year old woman with underlying lupus was presented with sudden left side body weakness and diplopia. Examination showed features of 16 syndrome with one and a half syndrome, facial diplegia and left hemiparesis. Neuroimaging revealed an acute infarct of bilateral dorsal pons. The patient was placed on antiplatelet therapy. However, she developed left leg deep vein thrombosis and pulmonary embolism after one week. She was then given subcutaneous low molecular weight heparin, followed by warfarin and cycles of cyclophosphamide. Her hemiparesis, ocular symptoms, and facial diplegia improved after one year. SLE increases the risk of stroke and thrombotic events in a young patient. Achieving disease remission is important to prevent stroke in SLE patients.

A rare case report of primary Sjögren's syndrome with clinical characteristics similar to those of CLIPPERS.

BACKGROUND: Primary Sjögren's syndrome (pSS) is an autoimmune inflammatory disease characterized by dryness of the eyes, mouth and other mucous membranes. Patients with pSS can also present with extraglandular manifestations, such as pulmonary, kidney and nervous system involvement. Central nervous system (CNS) manifestations have rarely been described in pSS. CASE PRESENTATION: A 33-year-old man was admitted with a one-month history of dizziness, speech disturbance, and walking instability. His brain enhanced magnetic resonance imaging (MRI) showed symmetrical, enhanced "salt-and-pepper-like" speckled lesions in the brainstem, basal ganglia, and subcortical regions, and his diagnosis was considered possible chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Further examination revealed that anti-SSA antibody was positive, and the Schirmer test and labial salivary gland histopathology were abnormal, which supported the diagnosis of pSS. CONCLUSION: pSS is a chronic systemic autoimmune disease that involves neurological complications. This case suggests that CNS lesions of pSS can present with clinical and MRI findings similar to those of CLIPPERS.

Fentanyl effects on respiratory neuron activity in the dorsolateral pons.

Opioids suppress breathing through actions in the brainstem, including respiratory-related areas of the dorsolateral pons, which contain multiple phenotypes of respiratory patterned neurons. The discharge identity of dorsolateral pontine neurons that are impacted by opioids is unknown. To address this, single neuronal units were recorded in the dorsolateral pons of arterially perfused in situ rat preparations that were perfused with an apneic concentration of the opioid agonist fentanyl, followed by the opioid antagonist naloxone (NLX). Dorsolateral pontine neurons were categorized based on respiratory-associated discharge patterns, which were differentially affected by fentanyl. Inspiratory neurons and a subset of inspiratory/expiratory phase-spanning neurons were either silenced or had reduced firing frequency during fentanyl-induced apnea, which was reversed upon administration of naloxone. In contrast, the majority of expiratory neurons continued to fire tonically during fentanyl-induced apnea, albeit with reduced firing frequency. In addition, pontine late-inspiratory and postinspiratory neuronal activity were absent from apneustic-like breaths during the transition to fentanyl-induced apnea and the naloxone-mediated transition to recovery. Thus, opioid-induced deficits in respiratory patterning may occur due to reduced activity of pontine inspiratory neurons, whereas apnea occurs with loss of all phasic pontine activity and sustained tonic expiratory neuron activity.NEW & NOTEWORTHY Opioids can suppress breathing via actions throughout the brainstem, including the dorsolateral pons. The respiratory phenotype of dorsolateral pontine neurons inhibited by opioids is unknown. Here, we describe the effect of the highly potent opioid fentanyl on the firing activity of these dorsolateral pontine neurons. Inspiratory neurons were largely silenced by fentanyl, whereas expiratory neurons were not. We provide a framework whereby this differential sensitivity to fentanyl can contribute to respiratory pattern deficits and apnea.

Consider the pons: bridging the gap on sensory prediction abnormalities in schizophrenia.

A shared mechanism across species heralds the arrival of self-generated sensations, helping the brain to anticipate, and therefore distinguish, self-generated from externally generated sensations. In mammals, this sensory prediction mechanism is supported by communication within a cortico-ponto-cerebellar-thalamo-cortical loop. Schizophrenia is associated with impaired sensory prediction as well as abnormal structural and functional connections between nodes in this circuit. Despite the pons' principal role in relaying and processing sensory information passed from the cortex to cerebellum, few studies have examined pons connectivity in schizophrenia. Here, we first briefly describe how the pons contributes to sensory prediction. We then summarize schizophrenia-related abnormalities in the cortico-ponto-cerebellar-thalamo-cortical loop, emphasizing the dearth of research on the pons relative to thalamic and cerebellar connections. We conclude with recommendations for advancing our understanding of how the pons relates to sensory prediction failures in schizophrenia.

Restless legs syndrome due to brainstem stroke: A systematic review.

Restless Legs Syndrome (RLS) is a sleep-related movement disorder, which can also result from brainstem pathology. A systematic review of articles published in the electronic databases PubMed and Web of Science was conducted to summarize the existent literature on RLS associated with a brainstem stroke. We identified eight articles including 19 subjects with RLS due to brainstem ischemic lesion. The symptoms occurred simultaneously with the infarction (66.7%) or few days after (33.3%). The most common location of infarction was pons and less commonly medulla. In most cases (68.4%), symptoms were unilateral. In the majority of those cases (92.3%), the contralateral limb was affected due to a lateral pons infarction. RLS symptoms after infarction improved or resolved in almost 90% of cases within a few days up to 3 months. In almost all patients who received dopaminergic treatment (11 out of 13, 91.7%), the symptoms improved significantly or resolved completely. Screening for RLS has to be considered in patients suffering a brainstem stroke, particularly anteromedial pontine infarction. The appearance of acute unilateral RLS symptoms, usually in association with other sensorimotor deficits, should prompt the clinician to consider a vascular event in the brainstem. RLS in these cases seem to have a favorable outcome and respond well to dopaminergic treatment.

Fetal Pontine Tegmental Cap Dysplasia- A Case Report.

Pontine tegmental cap dysplasia (PTCD) is a very rare hindbrain malformation recently described and the affected children show a bad prognosis. We present this case to increase the awareness of this rare condition and to highlight the importance of early prenatal diagnosis. A 25 years old female with 22 weeks gestation was referred after sonography for fetal magnetic resonance imaging (MRI) in the evaluation of cerebellar hypoplasia. Prenatal MRI confirmed cerebellar hypoplasia. Follow up postnatal MRI showed flattening of the ventral pons, beak-like tissue in the posterosuperior pons suggesting the diagnosis of PTCD. In retrospect the fetal MR images revealed features consistent with PTCD. To the best of our knowledge, this is the fifth prenatal case and with the earliest gestational age of 22 weeks.