scholarly journals Parotid carcinoma following chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: a case report

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yilun Deng ◽  
Bi Zhao ◽  
Bing Wei ◽  
Shihong Zhang ◽  
Ming Liu
Keyword(s):  
Clinical Symptoms ◽  
Brain Mri ◽  
Pulse Therapy ◽  
Inflammatory Disorder ◽  
Residual Symptoms ◽  
Parotid Carcinoma ◽  
Unsteady Gait ◽  
History Of ◽  
Nosologic Entity

Abstract Background Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder with unclear causes. Paraneoplastic etiology may be a cause. We report a case of CLIPPERS with parotid carcinoma. Case presentation A 54-year-old man with a history of lymphoma was hospitalized with a pontocerebellar syndrome. Brain MRI revealed that the pons and cerebellum were “peppered” with punctate and curvilinear enhancement lesions that supported the diagnosis of CLIPPERS. The relapse of lymphoma was excluded by a further cerebellum biopsy revealing predominantly CD3+ T cells in white matter. The patient was relieved after pulse therapy with intravenous methylprednisolone and a large dose of corticosteroids, but he complained of a worsening gait problem when corticosteroids were tapered to a lower dose. Although the clinical symptoms gradually improved again by increasing the dosage of corticosteroids with Azathioprine, the patient still had a slight unsteady gait during follow-up. At the 7-month follow-up, a parotid mass was detected by MRI and was verified as carcinoma by biopsy. After resection of parotid carcinoma, the residual symptoms and previous MRI lesions disappeared, and no relapse occurred. Conclusions CLIPPERS may not be a distinct nosologic entity but an overlapping diagnosis with other diseases. Some cases of CLIPPERS might be a subtype of paraneoplastic neurological syndromes (PNS) due to the similar mechanism of antibody-mediated encephalitis. Tumor screening and serum paraneoplastic autoantibody tests are recommended for patients with CLIPPERS, especially for those who relapse when corticosteroids treatment is stopped or tapered.

A Case of Ophthalmoplegic Migraine: Some Observations

2011 ◽  
Vol 24 (6) ◽  
pp. 862-866 ◽  
Author(s):  
S. Patro ◽  
T. Banerjee
Keyword(s):  
Differential Diagnosis ◽  
Rare Case ◽  
Clinical Symptoms ◽  
Brain Mri ◽  
Oculomotor Nerve ◽  
Root Exit Zone ◽  
Ophthalmoplegic Migraine ◽  
Characteristic Imaging ◽  
History Of

We describe a 43-year-old man with a long-standing history of headache and ophthalmoplegia. Brain MRI showed a nodular enhancing lesion at the root exit zone of the left oculomotor nerve. Contrast enhancement on follow-up MRI and clinical symptoms showed a partial resolution on starting prophylactic anti-migraine medications. This was confirmed to be a case of ophthalmoplegic migraine. Here we describe a rare case of OM in an adult patient with its characteristic imaging pattern and the differential diagnosis considered.

scholarly journals Chondrosternal Arthritis in Infant: An Unusual Entity

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Athina Nikolarakou ◽  
Dana Dumitriu ◽  
Pierre-Louis Docquier
Keyword(s):  
Clinical Symptoms ◽  
Therapeutic Option ◽  
White Blood Cells ◽  
Laboratory Data ◽  
C Reactive Protein ◽  
Reactive Protein ◽  
History Of ◽  
Local Tenderness ◽  
Wait And See

Primary arthritis of chondrosternal joint is very rare and occurs in infants less than 18 months of age. Presentation is most often subacute but may be acute. Child presents with a parasternal mass with history of fever and/or local signs of infection. Clinical symptoms vary from a painless noninflammatory to a painful mass with local tenderness and swelling, while fever may be absent. Laboratory data show low or marginally raised levels of white blood cells and C-reactive protein, reflecting, respectively, the subacute or acute character of the infection. It is a self-limiting affection due to the adequate immune response of the patient. Evolution is generally good without antibiotherapy with a progressive spontaneous healing. A wait-and-see approach with close follow-up in the first weeks is the best therapeutic option.

A Longitudinal Study of Atlanto-Dens Relationships in Asymptomatic Individuals with Down Syndrome

PEDIATRICS ◽  
1992 ◽  
Vol 89 (6) ◽  
pp. 1194-1198 ◽  
Author(s):  
Siegfried M. Pueschel ◽  
Francis H. Scola ◽  
John C. Pezzullo
Keyword(s):  
Down Syndrome ◽  
Clinical Symptoms ◽  
Age Groups ◽  
Atlantoaxial Instability ◽  
History Of ◽  
Child Development Center ◽  
Asymptomatic Individuals ◽  
Development Center ◽  
Over Time

This study was designed to investigate the natural history of atlantoaxial instability in individuals with Down syndrome and to determine whether significant changes in C1-C2 relationship are taking place over time. Although more than 400 patients with Down syndrome who are presently followed at the Child Development Center had cervical spine radiographic examinations in the past, only 141 patients who had serial radiological examinations and whose radiographs were available for reevaluation participated in this study. The results of our investigations revealed that there were only minor changes (1 to 1.5 mm) of atlanto-dens interval measurements over time in 130 (92%) patients with Down syndrome. Eleven patients (8%) had changes of atlanto-dens interval measurements between 2 and 4 mm over time; however, none of these patients had any clinical symptoms. The analyses of data obtained from several subgroups (males and females, various age groups, and patients with and without atlantoaxial instability) did not show any significant changes of atlanto-dens interval measurements of successively obtained radiographs. Our recommendations for and rationale of routine screening for atlantoaxial instability and follow-up examinations are discussed in detail.

Indolent Neuroendocrine Tumor Involving the Bone Marrow

2003 ◽  
Vol 127 (4) ◽  
pp. 488-491
Author(s):  
Ellen Schlette ◽  
L. Jeffrey Medeiros ◽  
Miloslav Beran ◽  
Carlos E. Bueso-Ramos
Keyword(s):  
Bone Marrow ◽  
Neuroendocrine Tumor ◽  
Bone Marrow Biopsy ◽  
Clinical Symptoms ◽  
Eosinophilic Cytoplasm ◽  
Hydroxyindoleacetic Acid ◽  
History Of ◽  
Cardiac Valve Disease ◽  
Immunohistochemical Studies

Abstract We report a unique case of a patient with a neuroendocrine tumor localized to the bone marrow. The patient had a history of hairy cell leukemia, and the neuroendocrine tumor was detected in a bone marrow biopsy specimen obtained to assess response to 2-chlorodeoxyadenosine therapy. The neuroendocrine tumor was present as nodules that replaced approximately 15% of the bone marrow medullary space and was composed of round cells with fine chromatin, indistinct nucleoli, and relatively abundant, granular, eosinophilic cytoplasm. Histochemical stains showed cytoplasmic reactivity with Grimelius and Fontana-Masson stains, and immunohistochemical studies showed positivity for keratin and chromogranin. The histologic, cytochemical, and immunohistochemical features resembled a carcinoid tumor, and metastasis to the bone marrow was considered initially. The patient was asymptomatic without diarrhea, flushing, or cardiac valve disease. Serotonin production, assessed by the measurement of serum 5-hydroxyindoleacetic acid and substance P levels, was normal. Extensive clinical and radiologic work-up and endoscopy of the gastrointestinal tract to detect a primary site other than the bone marrow were negative. Follow-up bone marrow biopsy 7 years after the initial diagnosis was positive for persistent neuroendocrine tumor. The patient has not received any therapy specific for the neuroendocrine tumor and has had no clinical symptoms or evidence of progression after 9 years of clinical follow-up. We suggest that this neuroendocrine tumor may have arisen in the bone marrow.

scholarly journals MON-073 Hyperinsulinemic Hypoglycemia Responsive to Diazoxide Due to a Previously Unknown ABCC8 Dominant Mutation

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Veronica M Figueredo ◽  
Alejandro Diaz ◽  
Pedro Pagan Banchs
Keyword(s):  
Brain Mri ◽  
Poor Response ◽  
White Matter Injury ◽  
Response To Treatment ◽  
Hyperinsulinemic Hypoglycemia ◽  
Dominant Mutation ◽  
Suspected Sepsis ◽  
Intravenous Glucose ◽  
History Of

Abstract Background: Hyperinsulinism is the most common cause of persistent hypoglycemia. It results from different genetic defects, the most common being recessive and dominant mutations in the ABCC8/KCNJ11 genes. The majority of recessive mutations have a poor response to Diazoxide, while dominant mutations are highly variable in both clinical presentation and response to treatment. Prompt recognition and management is critical to avoid irreversible brain damage. Clinical case: A 38-week gestation male, born via emergent c-section due to decreased fetal movement, presented with neonatal hypoglycemia. Pregnancy was uncomplicated and mother had a normal OGTT. Patient had a history of suspected sepsis, seizures, pulmonary hypertension and respiratory distress requiring intubation. Blood glucose was undetectable at birth and required multiple dextrose 10% boluses. A critical sample with a glucose of 47 mg/dL showed an elevated insulin at 30.3 m IU/mL with undetectable ketone levels. Lactic acid, ammonia, cortisol, GH, plasma amino acids, acylcarnitine profile and uric organic acids where all normal for a hypoglycemic state. He required intravenous glucose infusion with GIR up to 17 mg/kg/min to maintain normoglycemia. A brain MRI at 11 days of life showed findings of white matter injury. Subsequent genetic testing identified a heterozygous c.4051G>A (p.Val1351Met) variant in ABCC8, classified as “of uncertain significance”. However, an entry in the ClinVar database (RCV000714711.1) exists from a research lab and was classified as likely pathogenic. Analysis of parental samples showed that the mother was heterozygous for the same genetic variant. She did not have a history of hypoglycemia. Patient was started on diazoxide (8 mg/kg/day) and chlorothiazide with resolution of hypoglycemia. At a follow up visit at 5 months of age, there was no history of hypoglycemia, and no need for adjustments of the diazoxide dose by weight (dose at that time of 7.4 mg/kg/day). Conclusion: The ABCC8 reported here is a dominant mutation causing hyperinsulinemic hypoglycemia responsive to diazoxide with a milder phenotype later in infancy. Longitudinal follow up of the case is warranted to understand the long term progress in patients with this particular mutation. Reference: Adam MP, Ardinger HH, Pagon RA, Wallace SE, et al. None. 1993. Familial hyperinsulinism.

scholarly journals First-line ibrutinib for Bing-Neel syndrome

2018 ◽  
pp. bcr-2018-226102 ◽  
Author(s):  
Aaron Tallant ◽  
Daniel Selig ◽  
Sam O Wanko ◽  
Joseph Roswarski
Keyword(s):  
Kinase Inhibitor ◽  
Spinal Column ◽  
Lower Extremity Weakness ◽  
Cerebrospinal Fluid Flow ◽  
Leptomeningeal Enhancement ◽  
Unsteady Gait ◽  
History Of ◽  
Spinal Mri ◽  
Gait Abnormalities

The authors present a case of an elderly man with a history of Waldenstrom macroglobulinaemia in remission who presented with progressively worsening gait abnormalities and falls for several months. His examination was notable for bilateral lower extremity weakness and an unsteady gait. Brain and spinal MRI showed focal leptomeningeal enhancement in the brain and spinal column. Lumbar puncture was performed and cerebrospinal fluid flow cytometry demonstrated a monoclonal CD5/CD10-negative, CD20-positive B-cell lymphocyte population consistent with a diagnosis of Bing-Neel syndrome. He was started on ibrutinib, an oral Bruton’s tyrosine kinase inhibitor, and had marked improvement in his weakness and gait. Repeat imaging 2 months after starting ibrutinib showed improvement in his leptomeningeal enhancement. During subsequent follow-up, he continued to tolerate ibrutinib and had a sustained clinical response.

scholarly journals Flakka-Induced Prolonged Psychosis

2016 ◽  
Vol 2016 ◽  
pp. 1-2 ◽  
Author(s):  
Craig Crespi
Keyword(s):  
Hot Spot ◽  
Symptomatic Treatment ◽  
Altered Mental Status ◽  
Mental States ◽  
South Florida ◽  
Residual Symptoms ◽  
History Of ◽  
Synthetic Drug ◽  
Paranoid Psychosis

In South Florida, there has been a highly addictive new synthetic drug flooding the streets for people looking for a cheap high. Alpha-PVP, better known as Flakka, is an illegal substance that sells on the streets for as little as $5 a hit and delivers an instant high that can last from hours to days with lingering effects for weeks after it has been ingested. Although people use Flakka for its potential euphoric high, symptoms are known to easily escalate into frightening delusions, paranoid psychosis, extreme agitation, and a multitude of other altered mental states. According to the National Institute on Drug Abuse, Florida appears to be the nation’s hot spot for reports of Flakka. In this case report, a 17-year-old female with no prior psychiatric diagnosis presents to the hospital under a 72-hour involuntary placement for altered mental status with agitation and psychotic behaviors. After multiple days of symptomatic treatment with benzodiazepines and antipsychotics, the patient became coherent enough to give a history of a “friend” putting Flakka in her food at school as a joke. Although she continues to have residual symptoms including psychomotor agitation and slowing of cognition, she was alert, oriented, and able to be discharged home with proper follow-up.

scholarly journals Spontaneous ARIA-like Events in Cerebral Amyloid Angiopathy–Related Inflammation: A Multicenter Prospective Longitudinal Cohort Study

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012778
Author(s):  
Laura Antolini ◽  
Jacopo C. DiFrancesco ◽  
Marialuisa Zedde ◽  
Gianpaolo Basso ◽  
Andrea Arighi ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Cerebral Amyloid Angiopathy ◽  
Pulse Therapy ◽  
Amyloid Angiopathy ◽  
Longitudinal Cohort ◽  
History Of ◽  
Corticosteroid Pulse Therapy ◽  
Cerebral Amyloid

Background and Objectives:To investigate the natural history and outcomes following treatment for spontaneous amyloid-related imaging abnormalities (ARIA)-like in cerebral amyloid angiopathy-related inflammation (CAA-ri).Methods:A multicenter, hospital-based, longitudinal, prospective observational study of inpatients meeting CAA-ri diagnostic criteria, recruited through the iCAβ International Network, in the period January 2013 - March 2017. A protocol for systematic data collection at first-ever presentation and at subsequent in-person visits, including T1-weighted, GRE-T2*, fluid-suppressed T2-weighted (FLAIR), and T1 post-gadolinium contrast-enhancement images aquired on 1.5T MRI, was employed at 3, 6, 12, 24-months follow-up. Centralized reads of MRI images were performed blinded to clinical, therapeutic, and time-points information. Main outcomes were survival, clinical and radiological recovery, intracerebral hemorrhage (ICH), and recurrence of CAA-ri.Results:The study enrolled 113 participants (10.6% definite, 71.7% probable, and 17.7% possible CAA-ri), mean age 72.9 years, 43.4% female, 37.1% APOEε4 carriers. 36.3% had a history of Alzheimer’s disease, 33.6% of ICH. A history of ICH, as well as the occurrence of new ICH at follow-up, was more common in patients with cortical superficial siderosis at baseline (52.6% vs 14.3%; p< 0.0001 and 19.3% vs 3.6%; p<0.009, respectively). After the first-ever presentation of CAA-ri, 70.3% (95% CI, 61.6-78.5) and 84.1% (95% CI, 76.2-90.6) clinically recovered within three and twelve months, followed by radiological recovery in 45.1% (95% CI, 36.4 - 54.8) and 77.4% (95% CI, 67.7 - 85.9), respectively. After clinicoradiological resolution of the first-ever episode, 38,3% (95% CI, 22.9 - 59.2) had at least one recurrence within the following 24 months. Recurrence was more likely if intravenous high dose corticosteroid pulse therapy was suddenly stopped compared to slow oral tapering-off (Hazard Ratio 4.68; 95% CI, 1.57-13.93; p=0.006).Discussion:These results from the largest longitudinal cohort registry of patients with CAA-ri support the transient and potentially relapsing inflammatory nature of the clinical-radiological acute manifestations of the disease and the effectiveness of slow oral tapering-off after intravenous corticosteroid pulse therapy in preventing recurrences. Our results highlight the importance of differential diagnosis for spontaneous ARIA-like events in Aβ-driven diseases, including treatment-related ARIA in Alzheimer’s disease patients exposed to immunotherapy drugs.

scholarly journals Advanced acromegaly: successful disease control for more than 16 years using octreotide LA

2020 ◽  
Vol 2020 ◽  
Author(s):  
Omayma Elshafie ◽  
Nicholas Woodhouse
Keyword(s):  
Clinical Symptoms ◽  
Tumour Size ◽  
Uncontrolled Hypertension ◽  
Therapeutic Trial ◽  
List Type ◽  
Residual Tumour ◽  
History Of ◽  
Successful Control ◽  
Long Acting

Summary A 79-year-old male presented with a 10-year history of intermittent headache, sweating, persistent hand numbness and uncontrolled hypertension. He was receiving Nifedipine and Hydrochorothizide. On examination (O/E), his BP was 180/100 he was acromegalic. His growth hormone (GH) was 10 mIU/L (0.0–0.1) and his insulin-like growth factor (IGF-1): 952 µg/L (76–160). An MRI of the pituitary revealed a 3 × 2 cm pituitary macroadenoma. Surgery was refused and the family agreed for a therapeutic trial of octreotide. His GH levels fell immediately. Two weeks later he was switched to long-acting monthly octreotide in September 2003. During his 16-year follow-up, he has remained well and asymptomatic off medications for hypertension. His BP and IGF-1 levels were also normal until octreotide Long acting (LA) octrotide was stopped for 3 months at age 96. During this period the IGF-1 level returned to pretreatment levels 500 ng/L (50–141), GH 24 mIU/L (0.0–0.1), and a small residual tumour 0.5–0.8 cm was seen on the MRI. Octreotide LA was restarted and the IGF-1 and GH levels returned to normal. He continues the same treatment to date age 97 without side effects. We conclude that the successful control of IGF-1, GH levels, hypertension, tumour size and clinical symptoms for more than 16 years occurred using octreotide LA in an elderly advanced acromegalic patient. To the best of our knowledge, this is the first report of the successful use of octreotide LA for more than 16 years. Learning points: The value of a therapeutic trial of octreotide to identify responders. Control of GH and IGF-1 secretion using octreotide LA. The report of the successful use of octreotide for more than 16 years irrespective of age.

Prevalence and natural history of arachnoid cysts in adults

2013 ◽  
Vol 118 (2) ◽  
pp. 222-231 ◽  
Author(s):  
Wajd N. Al-Holou ◽  
Samuel Terman ◽  
Craig Kilburg ◽  
Hugh J. L. Garton ◽  
Karin M. Muraszko ◽  
...  
Keyword(s):  
Natural History ◽  
Brain Mri ◽  
Arachnoid Cysts ◽  
Consecutive Series ◽  
Middle Fossa ◽  
Presenting Symptoms ◽  
History Analysis ◽  
Number Of Patients ◽  
History Of

Object Arachnoid cysts are a frequent finding on intracranial imaging. The prevalence and natural history of these cysts in adults are not well defined. Methods We retrospectively reviewed the electronic medical records of a consecutive series of adults who underwent brain MRI over a 12-year interval to identify those with arachnoid cysts. The MRI studies were reviewed to confirm the diagnosis. For those patients with arachnoid cysts, we evaluated presenting symptoms, cyst size, and cyst location. Patients with more than 6 months' clinical and imaging follow-up were included in a natural history analysis. Results A total of 48,417 patients underwent brain MRI over the study period. Arachnoid cysts were identified in 661 patients (1.4%). Men had a higher prevalence than women (p < 0.0001). Multiple arachnoid cysts occurred in 30 patients. The most common locations were middle fossa (34%), retrocerebellar (33%), and convexity (14%). Middle fossa cysts were predominantly left-sided (70%, p < 0.001). Thirty-five patients were considered symptomatic and 24 underwent surgical treatment. Sellar and suprasellar cysts were more likely to be considered symptomatic (p < 0.0001). Middle fossa cysts were less likely to be considered symptomatic (p = 0.01. The criteria for natural history analysis were met in 203 patients with a total of 213 cysts. After a mean follow-up of 3.8 ± 2.8 years (for this subgroup), 5 cysts (2.3%) increased in size and 2 cysts decreased in size (0.9%). Only 2 patients developed new or worsening symptoms over the follow-up period. Conclusions Arachnoid cysts are a common incidental finding on intracranial imaging in all age groups. Although arachnoid cysts are symptomatic in a small number of patients, they are associated with a benign natural history for those presenting without symptoms.

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