Neuromyelitis Optica Spectrum Disorders and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

For over two centuries, clinicians have been aware of various conditions affecting white matter which had come to be grouped under the umbrella term multiple sclerosis. Within the last 20 years, specific scientific advances have occurred leading to more accurate diagnosis and differentiation of several of these conditions including, neuromyelitis optica spectrum disorders and myelin oligodendrocyte glycoprotein antibody disease. This new understanding has been coupled with advances in disease-modifying therapies which must be accurately applied for maximum safety and efficacy.

A Comparison of Pediatric- and Adult-Onset Aquaporin-4 Immunoglobulin G-Positive Neuromyelitis Optica Spectrum Disorder: A Review of Clinical and Radiographic Characteristics.

BACKGROUND: The identification of immunoglobulin G antibodies against the aquaporin-4 channel (AQP-IgG) in the majority of adult patients differentiates neuromyelitis optica as a distinct disease entity. The high specificity of AQP4-IgG for neuromyelitis optica has allowed the identification of seropositive patients with atypical presentations of this disease. Neuromyelitis optica spectrum disorder has been increasingly recognized in children who demonstrate patterns of clinical involvement beyond the traditional boundaries of the optic pathways and spinal cord. METHODS: This is a single-center, retrospective review comparing demographic, clinical/paraclinical, and laboratory features of children and adults with a serologically confirmed diagnosis of AQP4-IgG-positive neuromyelitis optica spectrum disorder. RESULTS: Of 151 reviewed patient charts, 12 pediatric-onset and 31 adult-onset patients had AQP4-IgG-positive neuromyelitis optica spectrum disorder. The mean age of pediatric-onset neuromyelitis optica spectrum disorder was 12 ± 3.58 years with a female predilection (3:1). Pediatric patients showed more frequent involvement of the brainstem (6/12 [50%]); P = .008) and diencephalon (3/12 [25%]; P = .018). A preceding infection was identifiable in only 3 of 12 (25%) pediatric-onset patients. Moreover, disability as calculated on the expanded disability status scale was less severe in pediatric-onset cases compared to adult-onset cases in their most recent assessment (0 [0-9]) vs 6.5 [0-10]; P = .005). Pediatric-onset patients were also more likely to respond to treatment of acute episodes with corticosteroids ± intravenous immunoglobulin and/or plasmapheresis (Clinical Global Impression-Change scale: 2.5 [1-4] vs 4 [1-6], P = .009). INTERPRETATION: This retrospective study was able to compare and contrast pediatric- and adult-onset neuromyelitis optica spectrum disorder. Relative to their adult counterparts, pediatric-onset neuromyelitis optica spectrum disorder patients were more likely to respond to treatment and less likely to be disabled from their disease at follow-up. Therefore, pediatric-onset disease may represent a less virulent form of neuromyelitis optica spectrum disorder.

Non-invasive MR imaging of human brain lymphatic networks with connections to cervical lymph nodes.

Meningeal lymphatic vessels have been described in animal studies, but limited comparable data is available in human studies. Here we show dural lymphatic structures along the dural venous sinuses in dorsal regions and along cranial nerves in the ventral regions in the human brain. 3D T2-Fluid Attenuated Inversion Recovery magnetic resonance imaging relies on internal signals of protein rich lymphatic fluid rather than contrast media and is used in the present study to visualize the major human dural lymphatic structures. Moreover we detect direct connections between lymphatic fluid channels along the cranial nerves and vascular structures and the cervical lymph nodes. We also identify age-related cervical lymph node atrophy and thickening of lymphatics channels in both dorsal and ventral regions, findings which reflect the reduced lymphatic output of the aged brain.

Detailed imaging of abducens nerve anatomy using contrast-enhanced 3D-TOF MR angiography.

PURPOSE: Cranial nerves (CNs), particularly CN IV and VI are difficult to visualize with conventional MRI techniques, particularly within the cavernous sinus region. The aim of this study was to evaluate the capacity of high-resolution contrast enhanced 3D time-of-flight (TOF) MR angiography using new generation 3 T imaging technology to provide detailed visualization of CN VI anatomy, particularly within the cavernous sinus and petroclival regions. METHODS: Two neuroradiologists conducted bilateral evaluation of CN VI visibility in 23 patients for nerve segments located in the petroclival segment (dural cave and Dorello's canal), and three divisions of the cavernous sinus. All images were collected using contrast enhanced TOF MR angiography using a new generation 3 T machine. RESULTS: Of the CN VI segments assessed, average visibility of CN VI was best achieved in Dorello's canal. Overall visibility of CN VI within the regions inspected was best achieved in the axial view, with the exception of the dural cave, which was best assessed using the coronal view. We also identified strong agreement in assessment of nerve visibility between the two reviewers. We also identified a putative CN6 duplication and a small schwannoma, highlighting the fidelity of our approach. CONCLUSION: Contrast enhanced 3D TOF MR angiography can visualize CN VI anatomy, particularly within the petrocavernosal region and cavernous sinus with simultaneous visualization of arterial and venous structures. This cannot be easily achieved using traditional MRI techniques. This imaging technique might be used with new generation machines to evaluate CN VI anatomy and pathologies within the petrocavernosal region and cavernous sinus, especially relating to vascular pathologies.

Anti-MOG associated disorder-Clinical and radiological characteristics compared to AQP4-IgG+ NMOSD-A single-center experience.

BACKGROUND: The discovery of two immunoglobulin G (IgG) antibodies against aquaporin 4 (anti-AQP4) and myelin oligodendrocyte glycoprotein (anti-MOG) has led to the distinction of the disorders anti-AQP4 immunoglobulin G positive neuromyelitis spectrum disorder (AQP4-IgG+ NMOSD) and anti-MOG associated disorder (MOGAD). Different clinical and radiological features have been proposed to distinguish these two demyelinating CNS diseases. METHODS: This is a single-center retrospective review at the University of Florida (UF) including all patients with the diagnostic code ICD G36 ("other acute disseminated demyelination") from October 2015 to January 2020 (n=141) and all charts included in the clinical NMOSD database of the UF Division of Neuroimmunology (n=36). A total of 151 cases were reviewed for presence of anti-MOG and anti-AQP4 antibodies and NMOSD diagnostic criteria. Differences in MOGAD and AQP4-IgG+ NMOSD were compared. RESULTS: Of the 151 reviewed patient charts, 11 were consistent with MOGAD and 43 with AQP4-IgG+ NMOSD. Patients with MOGAD were significantly younger at symptom onset compared to patients with AQP4-IgG+ NMOSD (14 [1-33] years vs. 37 [6-82] years; p=0.005). In comparison with AQP4-IgG+ NMOSD, optic neuritis in MOGAD was more frequently associated with bilateral optic nerve involvement: (6/11 [54.5%] vs. 6/43 [13.9%]; p=0.009) and fundoscopic presence of optic disc edema (5/11 [45.5%] vs. 3/43 [7%]; p=0.006). Perineuritis was a common radiological feature in MOGAD (present in 4 cases). In case of myelitis, there was more frequent involvement of the conus medullaris in MOGAD (4/11 [36.4%] vs. 2/43 [4.7%]; p=0.012). Symptomatic cerebral syndrome with supratentorial white matter lesions was seen in MOGAD patients with pediatric onset (pediatric onset: 4/6 [66.7%] vs. adult onset: 0/5 [0%]. In MOGAD, evidence for combined central and peripheral demyelination and increased intracranial pressure was present in one patient each. A preceding inciting event (illness/postpartum) was more frequently identifiable in MOGAD (4/11 [36.4%] vs. 4/43 [7%]; p=0.045). Disability as calculated on the Expanded Disability Status Scale was less severe in MOGAD compared to AQP-IgG+ NMOSD (most severe presentation: 5 [2-7] vs. 7 [1-10]; p=0.015; most recent assessment: 2 [0-5] vs. 5 [0-10]; p=0.045) and patients were more likely to respond to treatment of acute attacks with corticosteroids and/or plasmapheresis (Clinical Global Impression-Global Change scale: 1 [1-4] vs. 3 [1-6]; p=0.001). INTERPRETATION: The study confirms that simultaneous bilateral optic neuritis, presence of optic disc edema, transverse myelitis with conus involvement and a less severe disease course are distinctive features of MOGAD.

Corticosteroids lead to short-term improvement in cerebral amyloid angiopathy-related inflammation.

BACKGROUND: Cerebral amyloid angiopathy - related inflammation (CAA-ri) is an uncommon manifestation of CAA. METHODS: Single-center, retrospective review of all charts with ICD-code I68.0 (CAA) from 2/2/2016-1/1/2020. RESULTS: Of 152 CAA cases, 13 (8.6%) were consistent with CAA-ri. Corticosteroid-treatment led to short-term reduction in modified Rankin Scale scores (2.6 ± 1.4 vs. 1.6 ± 1.5; p = 0.01) and T2/FLAIR lesion volume (78.1 ± 52.2 cm3 vs. 30 ± 30.9 cm3, p < 0.01) as well as short-term improvement in post-treatment Clinical Global Impression - Global Change scores compared to pre-treatment scores (clinical: 6 ± 1 vs. 2.6 ± 1.3, p = 0.03; radiological: 4.6 ± 1.9 vs. 1.2 ± 0.4, p = 0.03). INTERPRETATION: Corticosteroid-treatment leads to clinical and radiological short-term improvement (class IV evidence).

Novel diagnostic imaging features of facial lupus panniculitis: ultrasound, CT, and MR imaging with histopathology correlate.

Lupus panniculitis (LP), also referred to as lupus erythematosus profundus (LEP), is a chronic recurrent inflammation condition of the subcutaneous fat. It occurs in 1 to 3% of patients with systemic lupus erythematosus (SLE) and in 10% of patients with discoid lupus erythematosus (DLE), but can also occur as an entity of its own. Patients with lupus panniculitis usually present with persistent, often tender and painful skin lesions, or subcutaneous nodules, that range from 1 to 5 cm in diameter. The overlying skin may appear erythematous; lesions may become ulcerated, and heal with atrophy, skin depression, dimpling and scaring. Lesions tend to resolve spontaneously and may follow a chronic course of remission and exacerbation that persists for months to years. The imaging features of facial LP are extremely scarce in the literature. We present a case of facial lupus panniculitis and describe the associated characteristic ultrasound, CT, and MR imaging findings along with histopathologic correlation.

Detection of bifid mandibular condyle using computed tomography

Objective: To determine the frequency and characteristics of bifid mandibular condyle (BMC) using computed tomography (CT) evaluation. Study Design: A retrospective study was carried out using the CT records of 550 patients referred to the Medical School of Erciyes University (Kayseri, Turkey) between 2007 and 2010. T-tests were used to compare frequency of BMC between the left and right sides and between female and male patients. Statistical analysis was performed using SPSS software and a chi-squared test. Results: Of the 550 Patients, 10 patients (1.82%) were found to have BMCs. Five patients were female (50%) and five were male (50%). Of these 10 patients, 7 (70%) had unilateral and 3 (30%) had bilateral BMCs. As a result, a total of 13 BMCs were found in 10 patients. No statistically significant differences were found between either the right- and left-sided BMCs or between female and male patients (p >.05). Conclusions: To our knowledge, this is the first retrospective study investigating the prevalence and characteristics of BMC using computed tomography. Although BMC is an uncommon anomaly, it may be a more frequent condition in the Turkish population. Further studies and research on the orientation of duplicated condylar heads should be carried out (AU)

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Detection of bifid mandibular condyle using computed tomography.

OBJECTIVE: To determine the frequency and characteristics of bifid mandibular condyle (BMC) using computed tomography (CT) evaluation. STUDY DESIGN: A retrospective study was carried out using the CT records of 550 patients referred to the Medical School of Erciyes University (Kayseri, Turkey) between 2007 and 2010. T-tests were used to compare frequency of BMC between the left and right sides and between female and male patients. Statistical analysis was performed using SPSS software and a chi-squared test. RESULTS: Of the 550 Patients, 10 patients (1.82%) were found to have BMCs. Five patients were female (50%) and five were male (50%). Of these 10 patients, 7 (70%) had unilateral and 3 (30%) had bilateral BMCs. As a result, a total of 13 BMCs were found in 10 patients. No statistically significant differences were found between either the right- and left-sided BMCs or between female and male patients (p >.05). CONCLUSIONS: To our knowledge, this is the first retrospective study investigating the prevalence and characteristics of BMC using computed tomography. Although BMC is an uncommon anomaly, it may be a more frequent condition in the Turkish population. Further studies and research on the orientation of duplicated condylar heads should be carried out.

Value of multislice computed tomography in the diagnosis of acute mesenteric ischemia.

OBJECTIVE: To define the value of multislice computed tomography (CT) in the diagnosis of acute mesenteric ischemia (AMI). MATERIALS AND METHODS: Two hundred patients (age range: 20-92 years) who were referred to the emergency CT department with a clinical suspicion of AMI were prospectively included in the study. CT examinations were performed with a multislice (16) CT scanner and the protocol included pre-contrast, arterial and venous phase acquisitions. Images were evaluated by using multiplanar reconstruction, maximum intensity projection and volume-rendering techniques at the CT workstation. RESULTS: Ninety-four patients (47%) underwent surgery for AMI or for other causes of acute abdominal pain. One hundred-six patients (53%) were followed conservatively according to clinical, radiologic and laboratory findings. Of the 94 patients who underwent surgery, 49 (25%) were found to have AMI. All of these 49 patients with a proven AMI diagnosis were diagnosed with CT. In the other 45 patients who underwent surgery, CT findings were negative for AMI. None of the patients, who were followed conservatively, were eventually diagnosed as having AMI except 1 patient. This patient was unfit for surgery although his clinical and radiologic findings were consistent with AMI and died in 3 days. The sensitivity and specificity values of CT for the detection of AMI were calculated to be 100% for each. CONCLUSIONS: Multislice CT is an effective imaging technique for the diagnosis of AMI with excellent sensitivity and specificity values.

Primary lumbar hydatid cyst: a case report.

A five-year-old male patient presented with swelling in the lumbar region that had persisted for two weeks. Ultrasonography and magnetic resonance imaging revealed a cystic lesion inside his right back muscles. Intra-abdominal organs within the site of inspection were normal. The lumbar region was explored, and a 3x4x5 cm hydatid cyst was detected inside the latissimus dorsi muscles. The cyst was incised and the germinative membrane was removed. Albendazole was used for three months after surgery to prevent recurrences. No problem was detected in a five-year follow-up period. Primary hydatid cyst in the lumbar area is very rare. In hydatid cyst treatment, it is necessary to remove the cyst without contaminating adjacent organs. Medical treatment as an adjunct to surgery increases the chance of full recovery.