Rituximab Versus Cyclophosphamide for Central Nervous System Involvement of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Case Series.

OBJECTIVE: To compare the effectiveness of rituximab (RTX) with cyclophosphamide (CYC) in patients who have central nervous system (CNS) involvement in antineutrophil cytoplasmic antibody-associated vasculitis (AAV). METHODS: A computer-assisted search was conducted to identify all adults who received a diagnosis of AAV with CNS involvement from January 1, 1997, through July 1, 2017, at our institution. RESULTS: Of the 17 patients identified, 11 had received RTX, and 6 had received CYC. Age at diagnosis of CNS involvement was similar in both groups. In the RTX group, 91% of the patients were women; in the CYC group, 33% were women (p = 0.03). At the time of CNS presentation, orbital involvement had occurred in 6 patients in the RTX group and in none of the patients in the CYC group. Initial remission of induction was achieved in all patients (100%) in the CYC group and in 10 patients (91%) in the RTX group. Two patients had no response to RTX: 1 patient when RTX was used for remission induction at the time of diagnosis and the second patient when RTX was used for remission induction after relapse. The median follow-up was 38 months (range, 9-127 months). Central nervous system relapse occurred in 4 patients in the RTX group and in 1 patient in the CYC group. Of the 4 patients in the RTX group with relapse, 3 had marked ocular involvement. Both nonresponder patients in the RTX group had ocular involvement. CONCLUSION: Rituximab is as effective as CYC in remission induction in patients with CNS involvement in AAV.

Spontaneous Regression of a Symptomatic Intramedullary Spinal Cord Lesion.

Intramedullary spinal cord tumors and cavernous malformations are rare lesions that can lead to progressive neurologic deficits, impaired quality of life, and even death. Early diagnosis and surgical resection of spinal cord tumors and cavernous malformations are often quoted as essential to optimizing a patient's functional outcome. Unfortunately, these are high-risk operations, with many patients having worse neurological deficits after surgery - sometimes permanent. We present a case of a patient with a cervical intramedullary spinal cord lesion that almost completely resolved spontaneously at short-term follow-up and remained stable at longe-term follow up. Conservative management with careful observation and sequential imaging should be considered in patients with intramedullary spinal cord lesions presenting with acute onset, stable symptoms, especially if the lesion has a hemorrhagic component.

Congenital hemangioma of the face-Value of fetal MRI with prenatal ultrasound.

We report the presentation, workup, and pre/perinatal management of a fetus with a large congenital hemangioma of the face. Hemangiomas are benign vascular neoplasms frequently encountered in neonates and infants. The less common congenital variant develops in utero and can present on prenatal ultrasound with diagnostic uncertainty as well as clinical implications for delivery. The differential diagnosis for these solid vascular masses when located in the head and neck includes myofibroma, kaposiform hemangioendothelioma, teratoma, and encephalocele. Potential clinical issues relate to size and location of the mass and include airway obstruction, disruption in development or compression of the globe, invasion into the cranial vault, bleeding and ulceration, and high output heart failure. Prenatal ultrasound and MRI play an important role in the diagnosis of congenital hemangiomas and planning for delivery and immediate postnatal cares.

Multiple myeloma of the thyroid cartilage.

A 60-year-old male presented with hoarseness. His past medical history was remarkable for a plasmacytoma of the left maxillary sinus having been resected without systemic evidence of plasma cell myeloma (PCM), also known as multiple myeloma (MM), at the time. This maxillary sinus disease recurred and was treated with radiation. Workup for PCM was conducted. Treatment included melphalan and autologous stem cell transplant. Because of the therapeutic and prognostic implications, a Plasma cell neoplasm (PCN) in a neck mass must be carefully evaluated by clinical and pathological criteria in order to distinguish plasmacytoma from PCM. PCN involvement of the thyroid cartilage is very rare, with only 5 previously reported cases.

Brooke-spiegler syndrome with multiple scalp cylindromas and bilateral parotid gland adenomas.

A 62-year-old female presented with numerous soft tissue lesions of her scalp and bilateral preauricular region. Several of these have been biopsied or removed with a diagnosis of cylindromas. Cylindromas are benign tumors with a differentiation towards apocrine sweat glands that increase in number and size throughout life. Multiple scalp cylindromas may coalesce and cover the entire scalp, resulting in the "turban tumor." These are often associated with the autosomal dominant Brooke-Spiegler syndrome with coexistent facial trichoepitheliomas and spiradenomas. There is a very rare association between cylindromas and basal cell adenoma and adenocarcinoma of the parotid gland, with only 17 reported cases. Ours is the first CT demonstration of both the scalp and parotid gland findings in this uncommon situation.

Pediatric cerebellar hemorrhagic glioblastoma multiforme.

We report the case of an 11 year old boy who presented with nausea, vomiting and ataxia. He was evaluated with computed tomography (CT) and magnetic resonance imaging (MRI). Imaging demonstrated minimal enhancement and hemorrhage of a cerebellar mass. Cerebellar glioblastoma multiforme (GBM) is extremely rare in the cerebellum at any age but especially in children. The atypical findings of minimal enhancement, cerebellar location and hemorrhagic presentation combine to make the prospective diagnosis of GBM a difficult one. This rare combination of findings has not been previously reported.

Spinal epidural hematoma in an infant as the initial presentation of severe hemophilia.

We describe the clinical manifestations, radiographic features, and management options of an extensive spontaneous spinal epidural hematoma in a 7-month boy who had severe hemophilia that had not been previously diagnosed, despite a baseline factor VIII level less than 1% of normal. We believe this to be the youngest reported case of a symptomatic spontaneous spinal epidural hemorrhage in an infant subsequently initially establishing a diagnosis of hemophilia.

Lyme disease of the brainstem.

Lyme disease is a multisystem infectious disease caused by the tick-borne spirochete, Borrelia burgdorferi. Central nervous system (CNS) involvement typically causes local inflammation, most commonly meningitis, but rarely parenchymal brain involvement. We describe a patient who presented with clinical findings suggesting a brainstem process. Magnetic resonance imaging (MRI) and positron emission tomography (PET) suggested a brainstem neoplasm. Prior to biopsy, laboratory evaluation led to the diagnosis of Lyme disease. Clinical and imaging abnormalities improved markedly following antimicrobial therapy. We describe Lyme disease involvement of the cerebellar peduncles with hypermetabolism on PET. Although MRI is the primary imaging modality for most suspected CNS pathology, the practical applications of PET continue to expand.

Perineural extension of facial melanoma.

A 64-year-old man presented with a pigmented cutaneous lesion on the right side of his face along with right facial numbness. Histological examination revealed malignant melanoma. Magnetic resonance imaging (MRI) revealed perineural extension along the entire course of the maxillary division of the right trigeminal nerve. This is a rare but important manifestation of the spread of head and neck malignancy.

Intrathecal injection of epidural blood patch: a case report and review of the literature.

Epidural blood patch (EBP) is a commonly performed procedure for the treatment of persistent severe post- dural-puncture headache (PDPH). It has a high success rate with a low incidence of complications. We report the case of a 27-year-old woman who developed progressive back pain and radicular symptoms after an EBP was performed for PDPH. An emergency MRI showed a subarachnoid hematoma. Gradual recovery occurred without the need for intervention. To our knowledge, this is the only case demonstrating the MRI findings of a rare complication of a common procedure. Radiologists may benefit from familiarity with epidural blood patching, including the technique, risks, benefits, and potential complications

Newborn brain infarction: clinical aspects and magnetic resonance imaging.

OBJECTIVE: We describe the clinical and imaging studies of 11 full-term babies with neonatal stroke. We classify the neonatal non-hemorrhagic strokes as thrombotic, embolic, or global vascular insufficiency and determine if this classification is improved by adding magnetic resonance spectroscopy (MRS) and diffusion-weighted imaging (DWI) to conventional magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). METHODS: Clinically, eight of the 11 babies presented with seizures, one with apnea, and two with lethargy. Conventional MRI and DWI were used to classify each infarct as being either borderzone or vascular distribution. The location of infarction revealed the presumed vascular pathophysiology. RESULTS: Infants were classified as having either embolic (bilateral middle cerebral artery,n=1), global ischemic (bilateral borderzone, n=2), or thrombotic infarction (unilateral middle cerebral artery, n=7; bilateral posterior cerebral arteries, n=1). DWI and MRS detected a small infarct better than conventional MRI in one patient. MRA showed abnormal intracranial arteries in three, all of who were in the thrombotic group. Even though MRS was more sensitive than conventional MRI in detecting ischemia/infarction in one patient, in another there was no detectable lactate in the stroke region found on conventional MRI. Clinical presentation was similar in global ischemia and focal infarctions, but newborn stroke was more likely to present with lateralizing focal motor seizures. Seizures were the most common presenting sign, with a paucity of other focal neurological deficits. CONCLUSION: MRI is the best approach to determine stroke pathophysiology. Brain infarction frequently presents with seizures. We speculate that the location and distribution of infarction might determine stroke timing, pathophysiology and outcome. Ongoing clinical studies will likely clarify this speculation.

Correlation of diffusion-weighted magnetic resonance imaging with neuropathology in Creutzfeldt-Jakob disease.

BACKGROUND: Although the diagnosis of Creutzfeld-Jakob disease (CJD) is straightforward in fully developed cases, a definitive diagnosis can be difficult early in the course of the illness. T2-weighted magnetic resonance imaging (MRI) signal abnormalities, and recently, diffusion-weighted MRI abnormalities, have been described in patients with CJD, suggesting the utility of MRI in the early recognition of CJD. OBJECTIVE: To correlate diffusion-weighted MRI signal abnormalities with neuropathologic changes in CJD. MATERIALS AND METHODS: Diffusion-weighted MRI and neuropathologic changes of 2 patients with autopsy-proven CJD were examined in a blinded fashion by a neuroradiologist and a neuropathologist. RESULTS: Areas of bright signal on diffusion-weighted MRI correlated with a higher degree of spongiform changes. CONCLUSION: Diffusion-weighted MRI in CJD demonstrates specific-signal abnormalities that correlate well with areas of the most severe and characteristic neuropathologic changes.