Bartonella henselae Infection and Lymphadenopathy in a Patient With T Cell Acute Lymphoblastic Leukemia.

Patients undergoing therapy for T cell acute lymphoblastic leukemia are at risk of infections during their treatment course. Cat scratch disease caused by Bartonella hensalae can masquerade as leukemic relapse and cause systemic infection. Obtaining a thorough exposure history may aid clinicians in making the diagnosis.

The black rim susceptibility sign in the MRI evaluation of intracranial colloid cysts.

BACKGROUND AND PURPOSE: Colloid cysts are relatively rare intracranial lesions located in the rostral aspect of third ventricle. They may produce acute hydrocephalus, brain herniation, and death. On conventional MRI, the appearance of a colloid cyst varies depending on its composition. Small isointense cysts can be missed. The purpose of this study is to introduce a new sign, "black rim susceptibility" sign for the accurate diagnosis of colloid cyst on susceptibility weighted imaging (SWI). METHODS: A retrospective case-control study consisted of 100 MRI brain scans (19 cases and 81 controls) performed from January 2012 to September 2018. Two fellowship trained neuroradiologists individually interpreted SWI sequences for the presence of the "black rim susceptibility" sign (thin rim of dark signal along the periphery of a rounded, hyperintense focus). RESULTS: The sample was 43% male and 57% female, with an average age of 51.8 ± 17.7. Out of 19 cases, 9 had undergone surgery in which pathology had confirmed colloid cyst. Sensitivity, specificity, and accuracy for reader 1 was 94.8%, 98.8%, and 98% and for reader 2 was 89.5%, 100%, and 98%, respectively. Positive predictive value and negative predictive value for reader 1 was 94.7% and 98.8% and for reader 2 was 100% and 97.6%, respectively. Interrater correlation between the two readers was calculated with kappa of 0.93. CONCLUSION: The black rim susceptibility appearance of colloid cyst on SWI is a novel description and an effective sign that can be used by radiologists for accurate diagnosis.

Normal range of clivoaxial angle in adults using flexion and extension cervical magnetic resonance imaging scans.

OBJECTIVE: An abnormally decreased clivoaxial angle (CXA) is used during the clinical evaluation for corrective skull base surgery. Published normal ranges of CXA using x-ray, computed tomography, or magnetic resonance imaging (MRI) vary dramatically, especially with neck flexion or extension. The aim of this study was to use high-resolution MRI to determine the normal range of CXA in various neck positions using a reproducible measurement technique. METHODS: The CXA was measured in 10 healthy volunteers on sagittal T2 SPACE c-spine MRI in supine and prone positions and with the neck both neck and extended. CXA is strictly defined as the angle between a line along the inferior third of the dorsal clival cortex and a line from the superior/posterior cortex of the dens to the posterior/inferior corner of the C2 body. Statistical analysis was performed in all positions and included mean CXA, range, standard deviation (SD), inter-reader agreement, and group comparisons. RESULTS: The mean CXA overall was 156.92° (SD=4.23°; range 134-179°). The mean value for extension CXA was 169.20° (SD=5.81°), and the mean value for flexion CXA was 144.73° (SD=5.71°), the difference being statistically significant (p<0.0001) regardless of supine or prone position. Concordant correlations of reader measurements showed substantial agreement in the supine position at 0.96, with lower agreement in the prone position at 0.87. CONCLUSIONS: We report normal ranges for CXA in various neck positions based on 3D T2-weighted MRI, using a reproducible measurement method. There was a significant difference in the CXA values between neck extended and neck flexed positions but not between supine and prone positions.

Arteriovenous Malformations Treated With Frameless Robotic Radiosurgery Using Non-Invasive Angiography: Long-Term Outcomes of a Single Center Pilot Study.

OBJECTIVE: CT-guided, frameless robotic radiosurgery is a novel radiotherapy technique for the treatment of intracranial arteriovenous malformations (AVMs) that serves as an alternative to traditional catheter-angiography targeted, frame-based methods. METHODS: Patients diagnosed with AVMs who completed single fraction frameless robotic radiosurgery at Medstar Georgetown University Hospital between July 20, 2006 - March 11, 2013 were included in the present study. All patients received pre-treatment planning with CT angiogram (CTA) and MRI, and were treated using the CyberKnife radiosurgery platform. Patients were followed for at least four years or until radiographic obliteration of the AVM was observed. RESULTS: Twenty patients were included in the present study. The majority of patients were diagnosed with Spetzler Martin Grade II (35%) or III (35%) AVMs. The AVM median nidus diameter and nidal volume was 1.8 cm and 4.38 cc, respectively. Median stereotactic radiosurgery dose was 1,800 cGy. After a median follow-up of 42 months, the majority of patients (81.3%) had complete obliteration of their AVM. All patients who were treated to a total dose of 1800 cGy demonstrated complete obliteration. One patient treated at a dose of 2,200 cGy developed temporary treatment-related toxicity, and one patient developed post-treatment hemorrhage. CONCLUSIONS: Frameless robotic radiosurgery with non-invasive CTA and MRI radiography appears to be a safe and effective radiation modality and serves as a novel alternative to traditional invasive catheter-angiography, frame-based methods for the treatment of intracranial AVMs. Adequate obliteration can be achieved utilizing 1,800 cGy in a single fraction, and minimizes treatment-related side effects.

Nilotinib Effects on Safety, Tolerability, and Biomarkers in Alzheimer's Disease.

OBJECTIVE: Preclinical evidence with nilotinib, a US Food and Drug Administration (FDA)-approved drug for leukemia, indicates improvement in Alzheimer's disease phenotypes. We investigated whether nilotinib is safe, and detectable in cerebrospinal fluid, and alters biomarkers and clinical decline in Alzheimer's disease. METHODS: This single-center, phase 2, randomized, double-blind, placebo-controlled study investigated the safety, tolerability, and pharmacokinetics of nilotinib, and measured biomarkers in participants with mild to moderate dementia due to Alzheimer's disease. The diagnosis was supported by cerebrospinal fluid or amyloid positron emission tomography biomarkers. Nilotinib 150 mg versus matching placebo was taken orally once daily for 26 weeks followed by nilotinib 300 mg versus placebo for another 26 weeks. RESULTS: Of the 37 individuals enrolled, 27 were women and the mean (SD) age was 70.7 (6.48) years. Nilotinib was well-tolerated, although more adverse events, particularly mood swings, were noted with the 300 mg dose. In the nilotinib group, central nervous system (CNS) amyloid burden was significantly reduced in the frontal lobe compared to the placebo group. Cerebrospinal fluid Aß40 was reduced at 6 months and Aß42 was reduced at 12 months in the nilotinib group compared to the placebo. Hippocampal volume loss was attenuated (-27%) at 12 months and phospho-tau-181 was reduced at 6 months and 12 months in the nilotinib group. INTERPRETATION: Nilotinib is safe and achieves pharmacologically relevant cerebrospinal fluid concentrations. Biomarkers of disease were altered in response to nilotinib treatment. These data support a larger, longer, multicenter study to determine the safety and efficacy of nilotinib in Alzheimer's disease. ANN NEUROL 2020 ANN NEUROL 2020;88:183-194.

Morphological classification of the tubercle of insertion of the transverse atlantal ligament: A computer tomography-based anatomical study of 200 subjects.

BACKGROUND AND PURPOSE: The atlantal tubercle is the attachment point of the transverse atlantal ligament, the main stabilizer of the atlantoaxial complex. No system of classification of the tubercle exists in the literature. We aimed to develop a morphologically based classification system of the atlantal tubercle to aid clinicians who deal with craniocervical pathology. MATERIALS AND METHODS: A retrospective review of computed tomography (CT) scans of the cervical spine was performed. The morphology of the atlantal tubercle was classified into four variants: rounded (classical), pointed, flattened, and hypoplastic. Age, presence, and morphological type were recorded. RESULTS: A total of 200 CT scans were identified and reviewed. The tubercle was present bilaterally in all patients. Patients were equally distributed over various age ranges. The following morphological types were recorded: rounded (227/400; 56.8%), pointed (13/400; 3.3%), flattened (126; 31.5%), and hypoplastic (34/400; 8.5%). The same type was seen bilaterally in 68% (135/200) of patients. Morphological types appear equally on the right and left side of the atlas. CONCLUSIONS: The first morphologically based classification system of the atlantal tubercle utilizing CT is presented. Morphology type, especially hypoplastic type, may confer an increased risk for subsequent need for posterior fusion.

Intracranial dural chondroma in a child-conventional and advanced neuroimaging characteristics and differential diagnosis.

Intracranial chondromas are rare tumors, especially in the pediatric population. We describe the conventional and advanced neuroimaging characteristics of this rare convexity dura-based chondroma in a young adolescent. In particular we demonstrate that diffusion-weighted imaging (DWI) facilitates differentiation between a dura-based chondroma and the more frequent classical meningioma. Chondromas are typically DWI hypointense with high apparent diffusion coefficient (ADC) values while meningiomas are typically DWI hyperintense with low ADC values. We also discuss the relevant additional differential diagnoses of dura based focal lesions for the pediatric population as well as the diagnostic significance of additional imaging modalities, including computed tomography, magnetic resonance imaging and cerebral angiography.

Gram-Positive Rods on a Cerebrospinal Fluid Gram Stain.

Cerebrospinal fluid (CSF) access device placement in the pediatric population presents challenges due to the development of infections following placement, access or revision, and/or shunt malfunctions. Here we report an unusual pediatric case of L. monocytogenes ventriculitis/VP shunt (VPS) infection and associated pseudocyst with an emphasis on the importance of VPS removal in clearing the infection due to biofilm formation.

Pediatric Dental Clinic-Associated Outbreak of Mycobacterium abscessus Infection.

BACKGROUND: Mycobacterium abscessus is an uncommon cause of invasive odontogenic infection. METHODS: M abscessus-associated odontogenic infections occurred in a group of children after they each underwent a pulpotomy. A probable case-child was defined as a child with facial or neck swelling and biopsy-confirmed granulomatous inflammation after a pulpotomy between October 1, 2013, and September 30, 2015. M abscessus was isolated by culture in confirmed case-children. Clinical presentation, management, and outcomes were determined by medical record abstraction. RESULTS: Among 24 children, 14 (58%) were confirmed case-children. Their median age was 7.3 years (interquartile range, 5.8-8.2 years), and the median time from pulpotomy to symptom onset was 74 days (range, 14-262 days). Clinical diagnoses included cervical lymphadenitis (24 [100%] of 24), mandibular or maxillary osteomyelitis (11 [48%] of 23), and pulmonary nodules (7 [37%] of 19). Each child had ≥1 hospitalization and a median of 2 surgeries (range, 1-6). Of the 24 children, 12 (50%) had surgery alone and 11 (46%) received intravenous (IV) antibiotics. Nineteen of the 24 (79%) children experienced complications, including vascular access malfunction (7 [64%] of 11), high-frequency hearing loss (5 [56%] of 9), permanent tooth loss (11 [48%] of 23), facial nerve palsy (7 [29%] of 24), urticarial rash (3 [25%] of 12), elevated liver enzyme levels (1 [20%] of 5), acute kidney injury (2 [18%] of 11), incision dehiscence/fibrosis (3 [13%] of 24), and neutropenia (1 [9%] of 11). CONCLUSIONS: M abscessus infection was associated with significant medical morbidity and treatment complications. Unique manifestations included extranodal mandibular or maxillary osteomyelitis and pulmonary nodules. Challenges in the identification of case-children resulted from an extended incubation period and various clinical manifestations. Clinicians should consider the association between M abscessus infection and pulpotomy in children who present with subacute cervical lymphadenitis. The use of treated/sterile water during pulpotomy might prevent further outbreaks.

An Unusual Case of Post-Traumatic Headache Complicated by Intracranial Hypotension.

We present a case of post-traumatic headache complicated by intracranial hypotension resulting in an acquired Chiari malformation and myelopathy with syringomyelia. This constellation of findings suggest a possible series of events that started with a traumatic cerebral spinal fluid (CSF) leak, followed by descent of the cerebellar tonsils and disruption of CSF circulation that caused spinal cord swelling and syrinx. This unusual presentation of post-traumatic headache highlights the varying presentations and the potential sequelae of intracranial hypotension. In addition, the delayed onset of upper motor neuron symptoms along with initially normal head computerized tomography scan (CT) findings, beg the question of whether or not a post-traumatic headache warrants earlier magnetic resonance imaging (MRI).

Effectiveness of a Rapid Lumbar Spine MRI Protocol Using 3D T2-Weighted SPACE Imaging Versus a Standard Protocol for Evaluation of Degenerative Changes of the Lumbar Spine.

OBJECTIVE: Reducing lumbar spine MRI scanning time while retaining diagnostic accuracy can benefit patients and reduce health care costs. This study compares the effectiveness of a rapid lumbar MRI protocol using 3D T2-weighted sampling perfection with application-optimized contrast with different flip-angle evolutions (SPACE) sequences with a standard MRI protocol for evaluation of lumbar spondylosis. MATERIALS AND METHODS: Two hundred fifty consecutive unenhanced lumbar MRI examinations performed at 1.5 T were retrospectively reviewed. Full, rapid, and complete versions of each examination were interpreted for spondylotic changes at each lumbar level, including herniations and neural compromise. The full examination consisted of sagittal T1-weighted, T2-weighted turbo spin-echo (TSE), and STIR sequences; and axial T1- and T2-weighted TSE sequences (time, 18 minutes 40 seconds). The rapid examination consisted of sagittal T1- and T2-weighted SPACE sequences, with axial SPACE reformations (time, 8 minutes 46 seconds). The complete examination consisted of the full examination plus the T2-weighted SPACE sequence. Sensitivities and specificities of the full and rapid examinations were calculated using the complete study as the reference standard. RESULTS: The rapid and full studies had sensitivities of 76.0% and 69.3%, with specificities of 97.2% and 97.9%, respectively, for all degenerative processes. Rapid and full sensitivities were 68.7% and 66.3% for disk herniation, 85.2% and 81.5% for canal compromise, 82.9% and 69.1% for lateral recess compromise, and 76.9% and 69.7% for foraminal compromise, respectively. CONCLUSION: Isotropic SPACE T2-weighted imaging provides high-quality imaging of lumbar spondylosis, with multiplanar reformatting capability. Our SPACE-based rapid protocol had sensitivities and specificities for herniations and neural compromise comparable to those of the protocol without SPACE. This protocol fits within a 15-minute slot, potentially reducing costs and discomfort for a large subgroup of patients.

Periodic Lateralized Epileptiform Discharges (PLEDs) in Patients With Neurosyphilis and HIV Infection.

Periodic lateralized epileptiform discharges (PLEDs) are an electroencephalographic pattern recorded in the setting of a variety of brain abnormalities. It is best recognized for its association with acute viral encephalitis, stroke, tumor, or latestatus epilepticus. However, there are other conditions that have been recognized as the underlying pathology for PLEDs such as alcohol withdrawal, Creutzfeldt-Jacob disease, anoxic brain injury, and hemiplegic migraine. However, there are only rare case reports of PLEDs in patients with neurosyphilis. Here, we report 2 patients presenting with encephalopathy and seizures with PLEDs, ipsilateral or contralateral to their main brain magnetic resonance imaging abnormalities. Further workup revealed neurosyphilis in both patients, one in association with human immunodeficiency virus (HIV) infection. Given the increasing incidence of neurosyphilis with or without HIV infection, these cases suggest neurosyphilis as a consideration in the differential for patients presenting with PLEDs.

Giovanni Battista Morgagni (1682-1771), the First Pediatric Pathologist.

During the age of enlightenment in the 18th century, radical changes were occurring in the Western world in science, medicine, philosophy, religion, and socio-economic concepts. In medicine, major advances had already been underway since the days of Vesalius.

Evidence of staphylococcal toxic shock syndrome caused by MRSA in a mother-newborn pair.

A neonate and his mother presented with fever and erythroderma. The mother met full diagnostic criteria for staphylococcal toxic shock syndrome, whereas the neonate lacked hypotension and multiorgan dysfunction. A wound culture from the neonate's circumcision site grew methicillin-resistant Staphylococcus aureus containing the tst gene. This provides evidence of the first reported case of toxic shock syndrome caused by methicillin-resistant Staphylococcus aureus in a mother-newborn pair.

Effect of multimodality treatment on overall survival for patients with metastatic or recurrent HPV-positive head and neck squamous cell carcinoma.

BACKGROUND: The optimal treatment for patients with recurrent human papillomavirus (HPV)-positive head and neck cancer is poorly understood. METHODS: We investigated treatments and outcomes in patients with recurrent head and neck cancer. Treatments included salvage neck surgery, metastasectomy, hypofractionated reirradiation, chemoembolization, and chemotherapy. Treatment outcomes were compared based on HPV status. RESULTS: A total of 37 patients were identified (12 HPV positive and 25 HPV negative). Demographics were similar. Overall, there was a trend toward a higher number of total treatment interventions in patients with HPV-positive disease (4.5 vs 2.6), but this was statistically insignificant (p=.066). After a mean follow-up of 21 months, median survival in HPV-negative patients was 10.6 months, whereas the median survival had not been reached for HPV-positive patients. Of the 12 HPV-positive patients, 7 were still alive (58%) after a mean follow-up period of 33 months. CONCLUSION: Multimodality aggressive therapy may improve overall survival in patients with recurrent HPV-positive disease. Further prospective research is warranted.

Bow hunter's syndrome: the use of dynamic magnetic resonance angiography and intraoperative fluorescent angiography.

Bow hunter's syndrome is a diagnosis typically made using dynamic digital subtraction angiography. The authors present the case of a 68-year-old woman who presented with symptoms consistent with bow hunter's syndrome that was accurately diagnosed utilizing noninvasive dynamic MR angiography. The dynamic MR angiogram clearly illustrated unilateral vertebral artery compression upon turning of the head. A subsequent CT of the cervical spine showed a ventral C-1 osteophyte within the foramen. The patient underwent posterior surgical decompression of the left vertebral artery. Sufficient decompression was confirmed using intraoperative fluorescent angiography with the patient's head turned. This case report is the first to illustrate that dynamic MR angiography can be a reliable and less invasive diagnostic tool. It can also be used to confirm sufficient postoperative decompression and monitor for recurrence. Intraoperative fluorescent angiography has been previously used in the evaluation of intracranial and extracranial vascular patency. This report is the first to show that fluorescent angiography can offer rapid and reliable intraoperative evaluation of vertebral artery decompression in bow hunter's syndrome.

Clinical presentation and imaging findings in patients with pulsatile tinnitus and sigmoid sinus diverticulum/dehiscence.

OBJECTIVE: Sigmoid sinus diverticulum/dehiscence (SSDD) is an increasingly recognized venous cause for pulsatile tinnitus (PT). SSDD is amenable to surgical/endovascular intervention. We aim to understand the clinical and imaging features of patients with PT due to SSDD. STUDY DESIGN: Retrospective CT study and chart review. SETTING: Tertiary-care, academic center. PATIENTS: Cohort 1: 200 consecutive unique temporal bone CT were blindly reviewed for anatomic findings associated with PT. Cohort 2: 61 patients with PT were evaluated for otologic manifestations. INTERVENTION(S): All patients underwent a temporal bone CT for evaluation of PT. Clinical information was gathered using electronic medical records. MAIN OUTCOME MEASURE(S): Otologic symptoms and physical findings (including body mass index (BMI), mastoid/neck bruits) were analyzed. Temporal bone CT scans were evaluated for the presence of SSDD and other possible causes of PT. RESULTS: Cohort 1: 35 cases of SSDD were identified (18%); 10 (29%) true diverticula; and 25 (71%) dehiscence. Sixty-six percent were right sided. Twelve patients had PT (34%). Patients with SSDD are more likely to have PT (p = 0.003). A significant association between right SSDD and PT was found (p = 0.001). Cohort 2: 15 out of 61 patients had PT and CT-confirmed SSDD. All were female subjects; average age was 45 years (26-73 yr). Radiologic evaluation revealed 10 SSDD cases on the right (66.7%), 2 on the left (13.3%%), and 3 bilateral (20%). Sensorineural hearing loss was seen in 8 (53%), aural fullness in 12 (80%). Average BMI was 32.2 (21.0-59.82), and 4 (26%) had audible mastoid bruits. CONCLUSION: SSDD may be the most common identifiable cause for PT from venous origin and is potentially treatable. Temporal bone CT scans should be included in a complete evaluation of PT.

Five fraction image-guided radiosurgery for primary and recurrent meningiomas.

PURPOSE: Benign tumors that arise from the meninges can be difficult to treat due to their potentially large size and proximity to critical structures such as cranial nerves and sinuses. Single fraction radiosurgery may increase the risk of symptomatic peritumoral edema. In this study, we report our results on the efficacy and safety of five fraction image-guided radiosurgery for benign meningiomas. MATERIALS/METHODS: Clinical and radiographic data from 38 patients treated with five fraction radiosurgery were reviewed retrospectively. Mean tumor volume was 3.83 mm(3) (range, 1.08-20.79 mm(3)). Radiation was delivered using the CyberKnife, a frameless robotic image-guided radiosurgery system with a median total dose of 25 Gy (range, 25-35 Gy). RESULTS: The median follow-up was 20 months. Acute toxicity was minimal with eight patients (21%) requiring a short course of steroids for headache at the end of treatment. Pre-treatment neurological symptoms were present in 24 patients (63.2%). Post treatment, neurological symptoms resolved completely in 14 patients (58.3%), and were persistent in eight patients (33.3%). There were no local failures, 24 tumors remained stable (64%) and 14 regressed (36%). Pre-treatment peritumoral edema was observed in five patients (13.2%). Post-treatment asymptomatic peritumoral edema developed in five additional patients (13.2%). On multivariate analysis, pre-treatment peritumoral edema and location adjacent to a large vein were significant risk factors for radiographic post-treatment edema (p = 0.001 and p = 0.026 respectively). CONCLUSION: These results suggest that five fraction image-guided radiosurgery is well tolerated with a response rate for neurologic symptoms that is similar to other standard treatment options. Rates of peritumoral edema and new cranial nerve deficits following five fraction radiosurgery were low. Longer follow-up is required to validate the safety and long-term effectiveness of this treatment approach.