Search

Rationale and Objectives Infectious encephalitis is a relatively common cause of morbidity and mortality. Treatment of infectious encephalitis with antiviral medication can be highly effective when administered promptly. Clinical mimics of encephalitis arise from a broad range of pathologic processes, including toxic, metabolic, neoplastic, autoimmune, and cardiovascular etiologies. These mimics need to be rapidly differentiated from infectious encephalitis to appropriately manage the correct etiology; however, the many overlapping signs of these various entities present a challenge to accurate diagnosis. A systematic approach that considers both the clinical manifestations and the imaging findings of infectious encephalitis and its mimics can contribute to more accurate and timely diagnosis. Materials and Methods Following an institutional review board approval, a health insurance portability and accountability act (HIPAA)-compliant search of our institutional imaging database (teaching files) was conducted to generate a list of adult and pediatric patients who presented between January 1, 1995 and October 10, 2013 for imaging to evaluate possible cases of encephalitis. Pertinent medical records, including clinical notes as well as surgical and pathology reports, were reviewed and correlated with imaging findings. Clinical and imaging findings were combined to generate useful flowcharts designed to assist in distinguishing infectious encephalitis from its mimics. Key imaging features were reviewed and were placed in the context of the provided flowcharts. Results Four flowcharts were presented based on the primary anatomic site of imaging abnormality: group 1: temporal lobe; group 2: cerebral cortex; group 3: deep gray matter; and group 4: white matter. An approach that combines features on clinical presentation was then detailed. Imaging examples were used to demonstrate similarities and key differences. Conclusions Early recognition of infectious encephalitis is critical, but can be quite complex due to diverse pathologies and overlapping features. Synthesis of both the clinical and imaging features of infectious encephalitis and its mimics is critical to a timely and accurate diagnosis. The use of the flowcharts presented in this article can further enable both clinicians and radiologists to more confidently differentiate encephalitis from its mimics and improve patient care.

The purpose of this study is to demonstrate the potential of diffusion tensor imaging (DTI) to reveal structural mechanisms underlying spinal ablative procedures, including percutaneous radiofrequency cordotomy (PRFC). PRFC is a surgical procedure that produces analgesia through focal ablation of the lateral spinothalamic tract (STT), thereby interrupting the flow of pain information from the periphery to the brain. To date, studies regarding mechanisms of analgesia after PRFC have been limited to postmortem cadaveric dissection and histology. However, with recent advances in DTI, the opportunity has arisen to study the STT non-invasively in vivo. In this technical note, an individual with successful pain relief following unilateral STT PRFC was examined using DTI, with the contralateral STT serving as an internal control. PRFC substantially reduced rostrocaudal directional DTI signal in the STT from the lesion in the cervical spinal cord through the pons and mesencephalon. Our findings confirm that focal ablation and anterograde degeneration accompany the analgesic effects of PRFC. In vivo imaging of the STT with DTI may contribute to surgical targeting for PRFC procedures, better understanding of the therapeutic and untoward effects of PRFC, and a deeper understanding of spinothalamic contributions to nociception.

Rationale and Objectives: To investigate for differences in metabolic concentrations and ratios between patients with systemic lupus erythematosus (SLE) without (group SLE) and those with neurological symptoms (group NPSLE) compared to a healthy control (group HC) in three normal-appearing brain regions: the frontal white matter, right insula (RI), and occipital gray matter and whether changes in any of the metabolites or metabolic ratios are correlated to disease activity and other clinical parameters. Materials and Methods: Twenty patients with SLE (18 women and 2 men, age range 23.4-64.6 years, mean age 43.9 years), 23 NPSLE patients (23 women, age range 23.7-69.8 years, mean age 42.4 years), and 21 HC (19 women and 2 men, age range 21.0-65.7 years, mean age 43.4 years) were included. All subjects had conventional brain magnetic resonance imaging and H-1 single-voxel spectroscopy, clinical assessment, and laboratory testing. Results: NPSLE patients had significantly reduced N-acetylaspartate (NAA)/creatine compared to HC (P = .02) and SLE patients (P = .01) in the RI. Lower glutamine/creatine levels were also detected in RI in both patient groups and in frontal white matter in NPSLE patients compared to HC (P = .01, P = .02). NAA/Cr ratio in the RI was significantly negatively correlated with the Systemic Lupus Erythematosus Disease Activity Index (r = -0.41; P = .008), and patients with active SLE symptoms also had a trend toward lower NAA/creatine ratios (1.02 vs 1.12; P = .07). Conclusions: The present data support previous findings of abnormal metabolic changes in normal-appearing regions in the brain of both SLE and NPSLE patients and raise the possibility that especially NAA, glutamine, and glutamate may be additional biomarkers for cerebral disease activity in SLE patients as these early metabolic changes occur in the brain of SLE patients before neurologic and imaging manifestations become apparent. (Less)

Undergoing voiding cystourethrogram (VCUG) can be distressing for children. To assess the efficacy of a cartoon and photograph montage storybook in preparing children for VCUG. Outpatient children (ages 2–14 years) who had VCUGs between December 2011 and June 2012 were randomly assigned to two groups; one group received the storybook a week before the procedure. Parents and guardians were asked to complete an anonymous survey rating their child’s tolerance of the exam from 1 to 5, worst to best, immediately after VCUG. The VCUG technologist also rated the child’s tolerance. Children prepared for VCUG with the storybook had less distress than those without. Results were analyzed by Cochran-Mantel-Haenszel and Cochran-Armitage Trend exact tests, a P value of both tests of 0.0092 indicating a statistically significant difference between the tolerance scores of children prepared with the storybook and those without. Effects of gender and history of VCUG were not statistically significant. Two-thirds of all children had no other source of information. The cartoon and photograph montage storybook format of preparing children for VCUG was effective in increasing their tolerance for the procedure. The storybook should be mailed out in advance because the majority of families did not pursue information on preparing their children for VCUG.

The triad of leukoencephalopathy, cerebral calcifications, and ysts (LCC) represents a very rare, and distinct clinico-radiologic ntity of unknown etiology, first described in children by Labrune t al. [1] in 1996. The onset ranges from infancy to adults 1–5], with varying clinical presentations including a combinaion of mild cognitive dysfunction or normal intelligence, seizures, erebellar, pyramidal and extrapyramidal signs. The disorder s characterized by diffusely progressive basal ganglia, brain tem and subcortical white matter calcifications, cerebellar, and upratentorial parenchymal cysts, as well as diffusely abnormal 2-weighted sequences on MRI [1–4]. The white matter abnoralities were described as a leukoencephalopathy rather than demyelinating process (leukodystrophy) [2]. Histopathologic xamination reveals angiomatous-like blood vessels, gliosis, and eaded, corkscrew-shaped intracytoplasmic inclusions typically ssociated with highly gliotic tissue surrounding cysts and vascuar malformations (Rosenthal fiber formation) in the white matter 1,3,4]. Complications are typically a consequence of mass effect ncluding cerebral edema leading to intracranial hypertension, apilledema, syringomyelia and eventually tonsillar herniation [4].

Objective.Neuropsychiatric lupus (NPSLE) is a severe and potentially life-threatening condition, reported to occur in 25%–70% of patients with systemic lupus erythematosus (SLE). Brain imaging, especially magnetic resonance imaging, is frequently used to diagnose or exclude overt cerebral pathologies such as edema, hemorrhage, and central thrombosis. More advanced imaging techniques have been applied to demonstrate subtle changes in regional cerebral blood flow and brain structure. We investigated changes in regional gray-matter (GM) volume in SLE patients without neurological manifestations and NPSLE patients at an acute stage of the disease.Methods.Using high-resolution structural images and voxel-based morphometry (VBM), we investigated regional GM volume in 20 NPSLE patients (within 2 weeks of the acute manifestation), 18 SLE patients without neurologic and/or psychiatric manifestations, and 18 healthy controls.Results.VBM analyses revealed several regions of GM atrophy in various parts of the brain in NPSLE and SLE patients. GM atrophy was seen in both groups in the temporal and parietal lobes and was most pronounced in the posterior thalamus bilaterally. Both groups showed an increase in regional GM volume in the posterior parahippocampal gyrus.Conclusion.Our data suggest that changes in regional brain morphology are present in acute NPSLE, but also in SLE (as compared to controls), which might be indicative of a subclinical neurodegenerative process. Further research is needed to investigate whether specific neuropsychiatric symptoms are related to these changes.

Objectives/Hypothesis: To describe surgical outcomes and radiographic features of olfactory groove meningiomas treated by excision through the subcranial approach. Special emphasis is placed on paranasal sinus and orbit involvement. Study Design: Retrospective review of a series of patients. Methods: Nineteen patients underwent excision of olfactory groove meningioma (OGM) via the transglabellar/subcranial approach between December 1995 and November 2009. Nine patients had previously undergone prior resection at outside institutions, and four had prior radiotherapy in addition to a prior excision. Transglabellar/subcranial surgical approach to the anterior skull base was performed. Results: Tumor histology included three World Health Organization (WHO) grade III lesions, one WHO grade II lesion, and 15 WHO grade I lesions. Fourteen patients had evidence of extension into the paranasal sinuses, with the ethmoid sinus being most commonly involved. Kaplan-Meier estimates of mean overall and disease-free survival were 121.45 months and 93.03 months, respectively. The mean follow-up interval was 41.0 months, and at the time of data analysis three patients had recurrent tumors. Seven (36.8%) patients experienced a major complication in the perioperative period; there were no perioperative mortalities. Orbit invasion was observed in four patients, with optic nerve impingement in 11 patients. Of these, three patients had long-term diplopia. No patients experienced worsening of preoperative visual acuity. Conclusions: Olfactory groove meningiomas demonstrate a propensity to spread into the paranasal sinuses, particularly in recurrent cases. Given a tendency for infiltrative recurrence along the skull base, this disease represents an important area of collaboration between neurosurgery and otolaryngology. The subcranial approach offers excellent surgical access for excision, particularly for recurrences that involve the paranasal sinuses and optic apparatus.

Eight patients sustained a combination of clival epidural hematoma, traumatic sixth cranial nerve palsy (6 NP), and occipitocervical injury. This combination of features has been sparsely described. Whether the hematoma, which represents tectorial membrane injury, is merely a marker for 6 NP and occipitocervical injury or is causative is unresolved, but this imaging finding should alert examiners who note traumatic 6 NP to the need for detailed cervical imaging, as surgical stabilization of this region may be critical to prevent future spinal cord dysfunction.

Background The basal ganglia, particularly caudate, are hypothesized to play a role in affective and obsessive-compulsive disorders. The depressive syndrome is a feature of untreated Cushing's disease. The objective of this study was to test the hypothesis that after treatment of Cushing's disease reduces elevated cortisol, improvement in mood and related ideations are associated with increase in caudate volume. Methods In this longitudinal, interventional study of 23 patients with Cushing's disease, 24-hour urinary free cortisol, structural magnetic resonance imaging and behavioral measures were obtained prior to treatment and approximately one year after pituitary microadenomectomy. Five SCL-90-R subscales measuring change in mood, related ideations and physical symptoms were utilized. Results Partial correlations (adjusted for age and time since surgery) showed change in caudate, but not hippocampal, volume was significantly associated with change in behavioral SCL-90-R subscales, indicating selectivity for structure. Right but not left caudate showed associations, suggesting selectivity for lateralization. Right caudate volume increase was significantly associated with decreases in Depression, Anxiety, Obsessive-Compulsive, and Paranoid scores, but not with Somatization (physical symptoms), indicating specificity for behavioral but not physical variables. Limitations A limitation is that relatively low-resolution scans were utilized. Although most likely not diminishing the significant findings, less sensitive methodology could lead to an increased probability of a type 2 error. Conclusions These findings support the concept that caudate, and likely right caudate, participates in human brain circuitry regulating mood.

Purpose: To investigate whether apparent diffusion coefficient (ADC), fractional anisotropy (FA), and eigenvalues in neuropsychiatric systemic lupus erythematosus (NPSLE) patients differ from those of healthy controls. Material and Methods: Eight NPSLE patients (aged 23–55 years, mean 42.9 years) and 20 healthy age-matched controls (aged 22–59 years, mean 44.4 years) underwent conventional brain magnetic resonance (MR) and diffusion tensor imaging (DTI). The ADC, FA, principal eigenvalue (λ∥), and the corresponding average perpendicular eigenvalue (λ⊥) ( = (λ2+λ3)/2) were measured in selected regions of normal appearing gray and white matter brain parenchyma. For statistical evaluation of differences between the two groups, a Student's t-test was used. The P value for statistical significance was set to P = 0.0025 after Bonferroni correction for multiple measurements. Results: Significantly increased ADC values were demonstrated in normal-appearing areas in the insular cortex ( P⊥ was significantly higher in several of these regions in NPSLE patients compared to healthy controls. Conclusion: Our study demonstrates alterations in normal-appearing gray and white matter brain parenchyma of patients with NPSLE by means of abnormal ADC, FA, and eigenvalues. These alterations may be based on loss of tissue integrity in part due to demyelination. It is possible that DTI in the future could assist in the diagnosis of NPSLE and possibly help to further elucidate the pathogenesis of NPSLE.

Infectious encephalitis is a relatively common cause of morbidity and mortality. Treatment of infectious encephalitis with antiviral medication can be highly effective when administered promptly. Clinical mimics of encephalitis arise from a broad range of pathologic processes, including toxic, metabolic, neoplastic, autoimmune, and cardiovascular etiologies. These mimics need to be rapidly differentiated from infectious encephalitis to appropriately manage the correct etiology; however, the many overlapping signs of these various entities present a challenge to accurate diagnosis. A systematic approach that considers both the clinical manifestations and the imaging findings of infectious encephalitis and its mimics can contribute to more accurate and timely diagnosis.Following an institutional review board approval, a health insurance portability and accountability act (HIPAA)-compliant search of our institutional imaging database (teaching files) was conducted to generate a list of adult and pediatric patients who presented between January 1, 1995 and October 10, 2013 for imaging to evaluate possible cases of encephalitis. Pertinent medical records, including clinical notes as well as surgical and pathology reports, were reviewed and correlated with imaging findings. Clinical and imaging findings were combined to generate useful flowcharts designed to assist in distinguishing infectious encephalitis from its mimics. Key imaging features were reviewed and were placed in the context of the provided flowcharts.Four flowcharts were presented based on the primary anatomic site of imaging abnormality: group 1: temporal lobe; group 2: cerebral cortex; group 3: deep gray matter; and group 4: white matter. An approach that combines features on clinical presentation was then detailed. Imaging examples were used to demonstrate similarities and key differences.Early recognition of infectious encephalitis is critical, but can be quite complex due to diverse pathologies and overlapping features. Synthesis of both the clinical and imaging features of infectious encephalitis and its mimics is critical to a timely and accurate diagnosis. The use of the flowcharts presented in this article can further enable both clinicians and radiologists to more confidently differentiate encephalitis from its mimics and improve patient care.

Subependymomas are benign intraventricular tumors with an indolent growth pattern, which are usually asymptomatic, and most commonly occur in the fourth and lateral ventricles. When symptomatic, subependymomas often obstruct critical portions of the cerebrospinal fluid (CSF) pathway, causing hydrocephalus, and range from 3 cm to 5 cm in size. We report a case of an unusually massive subependymoma of the lateral ventricles treated with subtotal resection, ventriculoperitoneal shunt, and post-surgical radiation. The clinical course, radiographic and pathologic characteristics of this massive intraventricular subependymoma are discussed, as well as the differential diagnosis of lateral ventricular masses and a review of the literature concerning subependymomas.

OBJECTIVE AND IMPORTANCE: We present a case of transient signal alterations along the brainstem path of the trigeminal nerve after microvascular decompression in a patient with trigeminal neuralgia. Such signal changes have been previously reported to represent infarction, but in our patient, they seemed to represent reactive neural and nuclear changes attributable to operative manipulation. CLINICAL PRESENTATION: A 43-year-old woman presented with gradually increasing, medically refractory recurrent trigeminal neuralgia 16 months after initial successful microvascular decompression for trigeminal neuralgia. Repeat microvascular decompression resulted initially in complete pain relief; however, her pain suddenly returned on the 3rd postoperative day. Magnetic resonance imaging (MRI) of this recurrent pain revealed new signal alterations along the brainstem path of the trigeminal nerve. Other than marked reduction in her trigeminal neuralgia, the patient had no postoperative alterations of trigeminal sensory or motor function. INTERVENTION: The patient's residual postoperative trigeminal neuralgia was successfully treated with carbamazepine. Because of the absence of worrisome signs and symptoms corresponding to the MRI signal alterations, no additional therapy was elected. Follow-up MRI in 1 month revealed near-complete resolution of the postoperative signal alterations along the brainstem path of the trigeminal nerve. CONCLUSION: MRI signal alterations along the brainstem path of the trigeminal nerve after microvascular decompression may be only reactive and do not always represent infarction. Depending on the clinical picture, observation may be all that is necessary in such cases.

A 38-year-old woman developed incoherent mentation, tremor, ataxia, and bilateral optic disc edema with mildly depressed visual acuity, nerve fiber bundle defects, and a left afferent pupillary defect. Magnetic resonance imaging of the brain disclosed striking linear contrast enhancement radiating from the ventricular borders. Lumbar puncture showed a normal opening pressure with a lymphocytic pleocytosis and elevated protein. On the basis of these findings, the initial diagnosis was viral or post-viral meningoencephalitis and the patient was not treated. During the next 4 weeks, her condition worsened. A brain and meningeal biopsy disclosed findings typical of primary angiitis of the central nervous system. With aggressive treatment, her neurologic status and magnetic resonance imaging normalized and her optic neuropathy improved markedly. Optic neuropathy and linear magnetic resonance imaging enhancement should be recognized as features of primary angiitis of the central nervous system.

Patients with spontaneous Cushing's syndrome are exposed to elevated levels of endogenous cortisol for months to years. We previously reported that hippocampal formation volume (HFV) increased in such patients after treatment lowered cortisol to normal concentrations. In the present study, we examined whether the structural increase was associated with improvement in cognition.Twenty-four patients with Cushing's disease were studied before treatment and following treatment. Magnetic resonance imaging was used to measure HFV and caudate head volume. Neuropsychologic tests of verbal cognition, learning, and memory were also administered.Patients showed variability in improvement on neuropsychologic test performance. After partialing out age, education, duration of illness, and time since surgical treatment, greater improvement in word list learning, as measured by the Selective Reminding Test was associated with greater increase in HFV (r =.59, p.02). There were no significant associations between improvement in paragraph or paired-word learning or memory tasks and increase in HFV. Improvement in other verbal tasks not strongly dependent on the hippocampus were not significantly associated with increase in HFV.After cortisol levels decline to normal concentrations, structural volumetric increase in HFV is accompanied by functional improvement in learning of unrelated words.

We report the case of a composite malignant neoplasm consisting of germ cell tumor and B-cell non-Hodgkin's lymphoma occurring in the sella turcica of a young girl who presented with hypopituitarism. Routine hematoxylin and eosin-stained sections of a resected suprasellar tumor demonstrated a neoplasm composed of 2 distinct morphologies. A panel of immunohistochemical markers was used to confirm the morphologic impression of germinoma (cytokeratin AE1/AE3-CAM 5.2, cytokeratin 7, neuron-specific enolase, and focally placental alkaline phosphatase positive) and mature B-cell lymphoma (CD20 positive; pancytokeratin, placental alkaline phosphatase, and terminal deoxynucleotidyl transferase negative). To the best of our knowledge, this is the first reported case of such a composite tumor in the central nervous system.

A patient who had achieved marked improvement in vision and shrinkage of a prolactinoma following treatment with pergolide (0.1 mg/day) suffered a marked worsening of vision 7 months after continued treatment at the same dose. Brain magnetic resonance imaging (MRI) at the time of visual loss showed further shrinkage of the tumor and prolapse of the chiasm into the pituitary fossa. The dose of pergolide was cut in half (0.05 mg/day); 12 months later, vision had completely recovered. Brain MRI at the time of visual recovery showed no change in the position of the prolapsed chiasm. This is the 11th reported case of delayed visual loss following dopaminergic treatment of prolactinoma. Recovery of vision always occurs with reduction of the medication dosage. Many patients whose prolactinomas are treated in this fashion display chiasmal prolapse, and few suffer visual loss. Considering that visual recovery occurs without a visible change in the position of the chiasm, traction is an unlikely cause of delayed visual loss. Therefore, the term chiasmal traction syndrome, used to describe visual loss with prolapsed chiasm following surgical and radiation treatment of sellar tumors, should not be applied in this setting lest it prompt consideration of surgical chiasmapexy. The proper management is reduction of the dopaminergic agonist dosage.

Background: Decreased hippocampal volume is observed in patients with Cushing’s syndrome and other conditions associated with elevated cortisol levels, stress, or both. Reversibility of hippocampal neuronal atrophy resulting from stress occurs in animals. Our study investigated the potential for reversibility of human hippocampal atrophy. Methods: The study included 22 patients with Cushing’s disease. Magnetic resonance brain imaging was performed prior to transsphenoidal microadenomectomy and again after treatment. Results: Following treatment, hippocampal formation volume (HFV) increased by up to 10%. The mean percent change (3.2 ± 2.5) was significantly greater (p < .04) than that of the comparison structure, caudate head volume (1.5 ± 3.4). Increase in HFV was significantly associated with magnitude of decrease in urinary free cortisol (r = −.61, p < .01). This relationship strengthened after adjustments for age, duration of disease, and months elapsed since surgery (r = −.70, p < .001). There was no significant correlation between caudate head volume change and magnitude of cortisol decrease. Conclusions: Changes in human HFV associated with sustained hypercortisolemia are reversible, at least in part, once cortisol levels decrease. While many brain regions are likely affected by hypercortisolemia, the human hippocampus exhibits increased sensitivity to cortisol, affecting both volume loss and recovery.

BACKGROUND Computerized tomography (CT) of metastatic adenocarcinoma to the brain usually shows low-to-moderate attenuation. However, mucinous adenomas may appear with high attenuation, mimicking hemorrhage. CASE DESCRIPTION A 68-year-old man with a history of metastatic esophageal adenocarcinoma presented to the emergency room complaining of a chronic, progressive right occipital headache. A head CT demonstrated a moderate-to-high attenuation, homogenous mass in the right cerebellar hemisphere consistent with an intracerebral hemorrhage. There was no frank calcification in the mass by CT criteria. An emergent posterior fossa craniectomy revealed nonhemorrhagic metastatic mucinous adenocarcinoma. CONCLUSION Moderate-to-high attenuation, noncalcified brain masses should raise the possibility of mucin-containing neoplasm.

Purpose: To analyze the failure patterns for patients with high-grade astrocytomas treated with high-dose conformal radiotherapy (CRT) using a quantitative technique to calculate the dose received by the CT- or MR-defined recurrence volume and to assess whether the final target volume margin used in the present dose escalation study requires redefinition before further escalation. Methods and Materials: Between 4/89 and 10/95, 71 patients with high-grade supratentorial astrocytomas were entered in a phase I/II dose escalation study using 3-D treatment planning and conformal radiotherapy. All patients were treated to either 70 or 80 Gy in conventional daily fractions of 1.8–2.0 Gy. The clinical and planning target volumes (CTV, PTV) consisted of successively smaller volumes with the final PTV defined as the enhancing lesion plus 0.5 cm margin. As of 10/95, 47 patients have CT or MR evidence of disease recurrence/progression. Of the 47 patients, 36 scans obtained at the time of recurrence were entered into the 3-D radiation therapy treatment planning system. After definition of the recurrent tumor volumes, the recurrence scan dataset was registered with the pretreatment CT dataset so that the actual dose received by the recurrent tumor volumes during treatment could be accurately calculated and then analyzed dosimetrically using dose–volume histograms. Recurrences were divided into several categories: 1) "central," in which 95% or more of the recurrent tumor volume ( V recur ) was within D 95 , the region treated to high dose (95% of the prescription dose); 2) "in-field," in which 80% or more of V recur was within the D 95 isodose surface; 3) "marginal," when between 20 and 80% of V recur was inside the D 95 surface; 4) "outside," in which less than 20% of V recur was inside the D 95 surface. Results: In 29 of 36 patients, a solitary lesion was seen on recurrence scans. Of the 29 solitary recurrences, 26 were central, 3 were marginal, and none were outside. Multiple recurrent lesions were seen in seven patients: three patients had multiple central and/or in-field lesions only, three patients had central and/or in-field lesions with additional small marginal or outside lesions, and one patent had 6 outside and one central lesion. Since total recurrence volume was used in the final analysis, 6 of the 7 patients with multiple recurrent lesions were classified into central/in-field category. Conclusion: Analysis of the 36 evaluable patients has shown that 32 of 36 patients (89%) failed with central or in-field recurrences, 3/36 (8%) had a significant marginal component to the recurrence, whereas only 1/36 (3%) could be clearly labeled as failing mainly outside the high-dose region. Seven patients had multiple recurrences, but only 1 of 7 had large-volume recurrences outside the high-dose region. This study shows that the great majority of patient recurrences that occur after high-dose (70 or 80 Gy) conformal irradiation are centrally located: only 1/36 patients (with 7 recurrent lesions) had more than 50% of the recurrence volume outside the region previously treated to high dose. Further dose escalation to 90 Gy (and beyond) thus seems reasonable, based on the same target volume definition criteria.

Objectives/Hypothesis: To determine the mode of inheritance of familial nonsyndromic Mondini dysplasia. Study Design: Correlative clinical genetic analysis of a single kindred. Methods: Clinical history, physical examination, audiologic analysis, computed tomography of the temporal bones, and cytogenetic analysis. Results: The male proband, three affected sisters, and an affected brother are offspring of unaffected parents. The mother and an unaffected brother have audiologic findings suggestive of heterozygous carrier status for a recessive hearing loss gene. Conclusions: Pedigree analysis indicates autosomal recessive inheritance in this family. The observed inheritance and clinical, audiologic, and radiologic findings are different from those previously described for another family with nonsyndromic Mondini dysplasia.1 The phenotype in this study family therefore represents a distinct subtype, indicating clinical and genetic heterogeneity of this disorder. This information should facilitate future molecular linkage analyses and genetic counselling of patients with inner ear malformations. Key Words: Mondini, sensorineural, hearing loss, familial, inner ear.

Using an illustrative case of a presumed pontine unidentified bright object (UBO) with spontaneous lesion regression over 2 years, we review the importance of including UBOs in the differential diagnosis of children with confirmed or possible neurofibromatosis type 1 (NF1) who present with diffuse pontine enlargement and T2-weighted changes on MRI. Asymptomatic children with presumed NF1 and diffuse pontine lesions should not be treated with radiation and should not be biopsied. Prior reports of good prognosis associated with pontine glioma in patients with NF1 may have been unrecognized UBOs in some cases.

Purpose : To minimize xerostomia in patients receiving bilateral head and neck irradiation (RT) by using conformal RT planning to spare a significant volume of one parotid gland from radiation. Methods and Materials : The study involved 15 patients with head and neck tumors in whom bilateral neck radiation was indicated. The major salivary glands and the targets (tumor, surgical bed, metastases to lymph nodes, and the locations of lymph nodes at risk for metastases) were outlined on axial computed tomography images. Beam's-eye view (BEV) displays were used to construct conformal beams that delivered the prescribed doses to the targets while sparing from direct radiation most of one parotid gland. The gland that was planned to be spared resided in the neck side that was judged in each patient to be at a lesser risk of metastatic disease. Major salivary gland flow rates and the responses to a subjective xerostomia questionnaire were assessed before, during, and after radiation. Results : Radiation planning for patients with central oropharyngeal tumors required the generation of multiple axial nonopposed beams. The resulting isodoses encompassed the targets, including the retropharyngeal nodes and the jugular nodes up to the base of skull bilaterally, while limiting the dose to the oral cavity, spinal cord, and one parotid gland. For patients with lateralized tumors, the ipsilateral neck side was treated up to the base of the skull ; in the contralateral neck side, the treatment included the subdigastric nodes but excluded the jugular nodes at the base of the skull and most of the parotid gland. This was accomplished by a moderate gantry angle that was chosen using the BEV displays. Three months following the completion of radiation, the spared parotid glands retained on average 50% of their unstimulated and stimulated flows. In contrast, no saliva flow was measured from the unspared glands in any of the patients. Subjective xerostomia was absent, mild, or not different from that reported before radiation in 10 of 15 patients (67%). Conclusion : Partial parotid gland sparing is feasible by using three-dimensional planning in patients undergoing bilateral head and neck radiation. Approximately 50% of the saliva flow from the spared glands may be retained, and most patients thus treated have no or mild xerostomia in the early period after the completion of radiation. Whether tumor control and late complications are comparable to standard radiation will be assessed as more experience is gained.

The large vestibular aqueduct syndrome (LVAS) is a distinct clinical entity characterized by stepwise progressive sensorineural hearing loss associated with isolated enlargement of the vestibular aqueduct. A correlative clinical, audiologic, vestibular, cytogenetic, and radiographic analysis of a family with inherited LVAS was performed. The male proband and his affected brother are offspring of unaffected parents, and have no other abnormalities. Pedigree analysis suggests autosomal recessive or X-linked inheritance with variable expressivity of LVAS in this family. This study is the first description of familial inheritance of LVAS. LVAS may account for a significant number of patients with nonsyndromal, genetic sensorineural hearing loss. Future molecular analyses of this study family may identify the causative gene(s) in LVAS.

Activating mutations of the TSH receptor (TSHR) are rare, with few reported cases of long-term follow-up.We present a follow-up report on a patient with neonatal thyrotoxicosis known to have a rare activating mutation of the TSHR, a heterozygous substitution in exon 10 (p.Ile568Thr). Initial treatment included total thyroidectomy at age 2 ½ years, resulting in iatrogenic hypothyroidism and hypoparathyroidism. The patient was treated with levothyroxine replacement to maintain TSH levels within normal range, as well as calcitriol and calcium carbonate to treat postsurgical hypoparathyroidism. However, 4 years later, while euthyroid, he developed a palpable 1-cm midline neck mass.Functional imaging with 123-I thyroid scan demonstrated active thyroid tissue within the thyroglossal duct remnant and in the tracheoesophageal groove. Surgical removal of the neck mass revealed cytologically bland thyroid follicular cells.These findings suggest that even after total thyroidectomy, patients with TSHR-activating mutations are at risk to develop significant quantities of functional thyroid tissue related to the hypertrophy of residual foci in the thyroid bed and in the thyroglossal duct remnant. These residual foci may enlarge and secrete thyroid hormones autonomously, decreasing the patient's levothyroxine requirement. Surveillance with serial physical examination and biochemical monitoring is recommended; suspicious findings can be further evaluated with functional thyroid imaging (99-m technetium or radioiodine 123-I thyroid scans) to adequately identify residual foci of thyroid tissue, which may require further treatment with surgical excision or radioablation.

Rationale and Objective: Neuropsychiatric systemic lupus erythematosus (NPSLE) is a diagnostically challenging, severe, and life-threatening condition, which is currently lacking a "gold standard." Our aim with this study is to look for magnetic resonance (MR) perfusion differences in NPSLE, SLE, and healthy control (HC) patients and correlate our findings with clinical parameters. Materials and Methods: Twenty-four NPSLE patients, 21 SLE patients, and 21 HC underwent dynamic susceptibility contrast enhanced MR perfusion using a 3-T scanner. Nine prospectively selected intracranial regions of interest were placed in white and gray matter and the cerebral blood flow (CBF), cerebral blood volume (CBV), and mean transit time (MU) values were calculated. Subjects underwent clinical evaluation with SLEDAI and serum antibodies. Results: The SLE patients had higher CBF and CBV compared to the HC overall (P =.01) and in specific areas (P =.03-.048). SLE patients with signs of active disease (elevated SLEDAI and anti-double-stranded DNA) had significantly elevated CBV, CBF, and MU in the posterior cingulate gyrus (P =.01-.02). No significant difference was seen in the magnetic resonance perfusion measurements of NPSLE patients compared to SLE and HC, although the NPSLE patients also showed higher CBV variability compared to the SLE (P =.0004) and HC cohort (P

Background: Although the major neuropathologic changes in Friedreich's ataxia (FA) affect the spinal cord and peripheral nerves, we previously found abnormally increased glucose metabolism in the cerebral hemispheres in ambulatory patients and a return toward normal metabolism in nonambulatory patients. Objective: To determine whether brain atrophy accompanies the decline in cerebral glucose metabolism in FA and whether the degree of atrophy and the extent of decline in cerebral glucose metabolism are related to clinical severity. Design: Prospective series. Setting: University referral center. Patients: Twenty-two patients with FA and 26 patients with dizziness, headache, or minor acute head trauma, serving as control subjects, who underwent computed tomographic scans that were interpreted as normal. Measures: In patients with FA and control subjects, regional atrophy was assessed using subjective and objective measures on computed tomographic scans. In patients with FA, local cerebral glucose metabolism was measured with positron emission tomography, and clinical severity was assessed with a clinical rating scale. Results: Atrophy in the cerebral hemispheres, cerebellum, and brain stem was significantly greater in patients with FA than in control subjects, and the degree of atrophy correlated with the clinical severity. Local cerebral metabolic rate for glucose declined significantly from the initally elevated levels in the thalamus, cerebellum, and brain stem in correlation with increasing clinical severity. Conclusions: The structure and function of wide-spread brain regions including the cerebral hemispheres are abnormal in FA, and these abnormalities correlate with the clinical severity.

Stereotactic subcaudate capsulotomy (SC) is used to treat medically intractable obsessive compulsive disorders (OCD). Although clinical improvement has been observed, post-SC structural correlates are lacking in this biological disorder. Our study provides imaging evidence for local and distant alterations in structures which may have an important role in the manifestations of OCD. Five OCD patients treated with SC received MR imaging for pre-SC planning, early (1-7 days) post-SC assessment, and late (5-12 months) follow-up. The volumes of the anterior limb of the internal capsules, caudate heads, third ventricle, mamillary bodies, thalami, and hippocampal formations were digitally computed. Volumes from each of the serial imaging studies were compared. At 5-12 months post-SC, all patients showed reduction in volume of the anterior limbs of the internal capsules, caudate heads, thalami, and increased volume of the third ventricle (reflecting thalamic/caudate atrophy). 2-5 patients showed reduction in hippocampal formation volume. The post-SC reduction in volume of these structures, some far distant to the stereotactic lesion, suggests that the interrelationships of the anterior limb of the internal capsule, the caudate/thalamic nuclei, and possibly the pallidal and limbic systems are necessary for the manifestations of OCD and their variants.

To describe surgical outcomes and radiographic features of olfactory groove meningiomas treated by excision through the subcranial approach. Special emphasis is placed on paranasal sinus and orbit involvement.Retrospective review of a series of patients.Nineteen patients underwent excision of olfactory groove meningioma (OGM) via the transglabellar/subcranial approach between December 1995 and November 2009. Nine patients had previously undergone prior resection at outside institutions, and four had prior radiotherapy in addition to a prior excision. Transglabellar/subcranial surgical approach to the anterior skull base was performed.Tumor histology included three World Health Organization (WHO) grade III lesions, one WHO grade II lesion, and 15 WHO grade I lesions. Fourteen patients had evidence of extension into the paranasal sinuses, with the ethmoid sinus being most commonly involved. Kaplan-Meier estimates of mean overall and disease-free survival were 121.45 months and 93.03 months, respectively. The mean follow-up interval was 41.0 months, and at the time of data analysis three patients had recurrent tumors. Seven (36.8%) patients experienced a major complication in the perioperative period; there were no perioperative mortalities. Orbit invasion was observed in four patients, with optic nerve impingement in 11 patients. Of these, three patients had long-term diplopia. No patients experienced worsening of preoperative visual acuity.Olfactory groove meningiomas demonstrate a propensity to spread into the paranasal sinuses, particularly in recurrent cases. Given a tendency for infiltrative recurrence along the skull base, this disease represents an important area of collaboration between neurosurgery and otolaryngology. The subcranial approach offers excellent surgical access for excision, particularly for recurrences that involve the paranasal sinuses and optic apparatus.

Objective Discussion of a rare case of angioleiomyoma of the internal auditory canal. Patient Thirteen-year-old female patient with a 1-year history of progressive hearing loss. Intervention Middle cranial fossa approach providing complete surgical extirpation. Main outcome measure Surgical pathology. Results Radiography and history suggestive of vestibular schwannoma; pathology revealed angioleiomyoma. Conclusion Angioleiomyoma is a rare lesion of the internal auditory canal that has many similar clinical and radiographic features of a vestibular schwannoma. There are no previous reports of this tumor occurring within the internal auditory canal in this age group.

Patients with chronic hypercortisolemia due to Cushing's syndrome (CS) exhibit cognitive dysfunction. Because glucocorticoid excess is associated with hippocampal damage in animals, and the hippocampus participates in learning and memory, we explored the relationships between hippocampal formation (HF) volume, memory dysfunction, and cortisol levels in 12 patients with CS. After magnetic resonance imaging, HF volume was determined using digital sum of track ball traces of dentate gyrus, hippocampus proper and subiculum, correcting for total intracranial volume. For 27% of the patients, HF volume fell outside the 95% confidence intervals for normal subject volume given in the literature. In addition, there were significant and specific correlations between HF volume and scores for verbal paired associate learning, verbal recall, and verbal recall corrected for full-scale IQ (r = 0.57 to 0.70, p < 0.05). HF volume was negatively correlated with plasma cortisol levels (r = -0.73, p < 0.05). These studies suggest an association between reduced HF volume, memory dysfunction, and elevated cortisol in patients with CS.

We describe a patient with multiple congenital anomalies including deafness, lacrimal duct stenosis, strabismus, bilateral cervical sinuses, congenital cardiac defects, hypoplasia of the corpus callosum, and hypoplasia of the cerebellar vermis. Mutation analysis of EYA1, SIX1, and SIX5, genes that underlie otofaciocervical and/or branchio-oto-renal syndrome, was negative. Pathologic diagnosis of the excised cervical sinus tracts was revised on re-examination to heterotopic salivary gland tissue. Using high resolution chromosomal microarray analysis, we identified a novel 2.52 Mb deletion at 19p13.12, which was confirmed by fluorescent in situ hybridization and demonstrated to be a de novo mutation by testing of the parents. Overall, deletions of chromosome 19p13 are rare.

We describe a 73-year-old man who developed diplopia as the initial manifestation of a left thalamic infarction. By the time he reached the emergency department, clouded consciousness precluded localization of the lesion. Results of brain MRI were initially interpreted as negative. Ophthalmologic examination several hours later disclosed a small vertical ocular misalignment attributed to skew deviation. This finding led to careful scrutiny of the upper brainstem on MRI. Comparison of the diffusion, apparent diffusion coefficient, and exponential apparent diffusion coefficient MRI studies allowed a diagnosis of subtle left thalamic infarction. The recognition of skew deviation in this setting is important because it may be the most specific indicator of a brainstem lesion.

In the 1950's it was first observed that mammalian cells exposed to the halogenated deoxyuridines were more sensitive to ultraviolet light and radiation than untreated cells. This prompted early clinical trials with bromodeoxyuridine (BUdR) which showed mixed results. More recently, several Phase I studies, while establishing the feasibility of continuous intravenous (IV) infusion of BUdR, have reported significant dose limiting skin and bone marrow toxicities and have questioned the optimal method of BUdR delivery. To exploit the high mitotic activity of malignant gliomas relative to surrounding normal brain tissue, we have developed a permanently implantable infusion pump system for safe, continuous intraarterial (IA) internal carotid BUdR delivery. Since July 1985, 23 patients with malignant brain tumors (18 grade 4, 5 grade 3) have been treated in a Phase I clinical trial using IA BUdR ( 400−600 mg/m 2 /day × 8 1 2 weeks) and focal external beam radiotherapy (59.4Gy at 1.8 Gy/day in 6 1 2 weeks ). Following initial biopsy/surgery the infusion pump system was implanted; BUdR infusion began 2 weeks prior to and continued throughout the 6 1 2 week course of radiotherapy. There have been no vascular complications. Side-effects in all patients have included varying degrees of anorexia, fatigue, ipsilateral forehead dermatitis, blepharitis, and conjunctivitis. Myelosuppression requiring dose reduction occurred in one patient. An overall Kaplan-Meier estimated median survival of 20 months has been achieved. As in larger controlled series, histologic grade and age are prognostically significant. We have shown in a Phase I study that IA BUdR radiosensitization is safe, tolerable, may lead to improved survival, and appears to be an efficacious primary treatment of malignant gliomas.

To determine whether ventriculomegaly is associated with ongoing increased intracranial pressure (ICP), physicians often rely on corroborative imaging features such as altered periependymal signal, distortion of ventricular shape, subarachnoid space flattening, and an increase in ventricular size over time. In 2 patients with new headache and altered mental status, symptoms and ventriculomegaly were dismissed as long-standing and not reflective of current ICP elevation. In the first patient, ICP was considered normal because there were no corroborative imaging features of elevated ICP. In the second patient, ICP was considered normal because ventricular size was stable over a 1-year period. The diagnosis of ICP elevation was finally made by ICP monitoring after papilledema was recognized. Ventriculoperitoneal shunting rapidly resolved the papilledema and markedly improved mentation. Brain imaging may often be an unreliable guide to the presence of elevated ICP. In such patients, the finding of papilledema is a critical determinant of management.

A 50-year-old woman presented with subacute cognitive decline, impaired eye movements, and simultanagnosia, components of the Balint syndrome. She had relatively low blood pressure in the left arm and left finger clubbing. Brain imaging identified multiple acute infarcts. Transesophageal echocardiography showed no cardiac abnormalities but demonstrated a thickened aortic wall and an intraluminal aortic arch mass. The surgical specimen revealed angiosarcoma. Of the few reported angiosarcomas involving the aorta, most have been located in the abdominal segment. This is only the second reported case of aortic arch sarcoma presenting with stroke.

Diffuse malignant gliomas, the most common type of brain tumor, carry a dire prognosis and are poorly responsive to initial treatment. The response to treatment is typically evaluated by measurements obtained from radiographic images several months after the start of treatment; therefore, an early biomarker of tumor response would be useful for making early treatment decisions and for prognostic information. Thirty-four patients with malignant glioma were examined by diffusion MRI before treatment and 3 weeks later. These images were coregistered, and differences in tumor-water diffusion values were calculated as functional diffusion maps (fDM), which were correlated with the radiographic response, time-to-progression (TTP), and overall survival (OS). Changes in fDM at 3 weeks were closely associated with the radiographic response at 10 weeks. The percentage of the tumor undergoing a significant change in the diffusion of water ( V T ) was different between patients with progressive disease (PD) vs. stable disease (SD) ( P < 0.001). Patients classified as PD by fDM analysis at 3 weeks were found to have a shorter TTP compared with SD (median TTP, 4.3 vs. 7.3 months; P < 0.04). By using fDM, early patient stratification also was correlated with shorter OS in the PD group compared with SD patients (median survival, 8.0 vs. 18.2 months; P < 0.01). On the basis of fDM, tumor assessment provided an early biomarker for response, TTP, and OS in patients with malignant glioma. Further evaluation of this technique is warranted to determine whether it may be useful in the individualization of treatment or evaluation of the response in clinical protocols.

Assessment of radiation and chemotherapy efficacy for brain cancer patients is traditionally accomplished by measuring changes in tumor size several months after therapy has been administered. The ability to use noninvasive imaging during the early stages of fractionated therapy to determine whether a particular treatment will be effective would provide an opportunity to optimize individual patient management and avoid unnecessary systemic toxicity, expense, and treatment delays. We investigated whether changes in the Brownian motion of water within tumor tissue as quantified by using diffusion MRI could be used as a biomarker for early prediction of treatment response in brain cancer patients. Twenty brain tumor patients were examined by standard and diffusion MRI before initiation of treatment. Additional images were acquired 3 weeks after initiation of chemo- and/or radiotherapy. Images were coregistered to pretreatment scans, and changes in tumor water diffusion values were calculated and displayed as a functional diffusion map (fDM) for correlation with clinical response. Of the 20 patients imaged during the course of therapy, 6 were classified as having a partial response, 6 as stable disease, and 8 as progressive disease. The fDMs were found to predict patient response at 3 weeks from the start of treatment, revealing that early changes in tumor diffusion values could be used as a prognostic indicator of subsequent volumetric tumor response. Overall, fDM analysis provided an early biomarker for predicting treatment response in brain tumor patients.

Chronically elevated levels of cortisol have been associated with changes in cognitive functioning and brain morphology. Using Cushing's disease as a model to assess the effects of high levels of cortisol on cognitive functioning, 27 patients with Cushing's disease were examined at baseline and three successive follow-up periods up to 18 months after successful surgical treatment. At all follow-up periods, patients were administered cognitive tests as well as measures of plasma and urinary free cortisol. Structural MRIs and a depression measure were taken at baseline and one-year follow-up. Results showed that there is a specific pattern of significant cognitive and morphological improvement following successful treatment. Verbal fluency and recall showed recovery, although brief attention did not. Age of participants was a significant factor as to when recovery of function occurred; younger patients regained and sustained their improvement in cognitive functioning more quickly than older participants. Improvement in verbal recall also was associated with a decrease in cortisol levels as well as an increase in hippocampal formation volume one year after treatment. Overall, these findings suggest that at least some of the deleterious effects of prolonged hypercortisolemia on cognitive functioning are potentially reversible, up to at least 18 months post treatment.

We report a case of hairy polyp of the pharynx diagnosed on brain MRI in order to stress the need to examine carefully all tissues included on an imaging study, even those outside the clinically stated region of interest, and to remind practitioners to consider unusual as well as common etiologies for neonatal respiratory distress. Our case is unique in that thorough examination of a brain MRI, ordered in the evaluation of presumed central apnea, led to the correct diagnosis.

MRI and 2D-CSI spectroscopy were performed in eight patients with systemic lupus erythematosus who presented with acute onset of neuropsychiatric lupus (NP-SLE), and in seven normal controls to evaluate for differences in metabolic peaks and metabolic ratios between the two groups. Also, the interval change of the metabolic peaks and their ratios during treatment in the NP-SLE patient group was evaluated. Metabolic peaks for N-acetyl-aspartate (NAA), choline (Cho), creatine (Cr), and lactate/lipids (LL) and their ratios (NAA/Cr, NAA/Cho, Cho/Cr, LL/Cr) were determined at initial presentation and 3 and 6 months later. In the eight lupus patients compared to the seven normal controls, NAA/Cho ratios were lower at presentation (1.05 vs 1.25; p = 0.004) and decreased even further at the three month follow-up (0.92 vs 1.05; p = 0.008). In contrast, both Cho/Cr (1.42 vs 1.26; p = 0.026) and LL/Cr ratios (0.26 vs 0.19; p = 0.002) were higher in the lupus patients at presentation compared to the controls and did not significantly change at three and six months follow-up. The NAA/Cr ratios were lower in the lupus patients compared to the controls at presentation but the difference was not statistically significant. However, the mean NAA/Cr significantly decreased from the initial examination to the three month follow-up (1.42 vs 1.32; p = 0.049) but did not significantly change from the three to the six month follow-up examinations. The NAA/Cr, Cho/Cr, and NAA/Cho ratios varied significantly (p < 0.05, p < 0.05, p < 0.05, respectively) between the 17 different locations measured in the brain in all eight patients and seven controls. Both the NAA/Cr ratios and the Cho/Cr ratios were also significantly lower in the gray matter than in the white matter (p < 0.0001) in both patients and controls, whereas the LL/Cr and NAA/Cho ratios were not significantly different. In conclusion, 2D-CSI MR spectroscopy may be useful in the early detection of metabolic CNS changes in NP-SLE patients with acute onset of new neurological symptoms as well as in the follow-up after treatment to assess presence and changes in metabolic brain injury. However, although there are detectable differences between normal individuals and lupus patients it is currently unclear whether these relate to the acute episode. Future studies are needed comparing NP-SLE patients with active CNS involvement with those inactive disease.

Summary Background: Chloral hydrate (CH) sedation for magnetic resonance imaging (MRI) is associated with significant failure rates, adverse events and delayed recovery. Pentobarbital (PB), reportedly produces successful sedation in 98% of children undergoing diagnostic imaging. This study compared the efficacy, adverse events and recovery characteristics of CH vs PB in children undergoing MRI. Methods: With Institutional Review Board approval and written consent, children were randomly assigned to receive intravenous (i.v.) PB (maximum 5 mgAEkg )1 in incremental doses) or oral CH (75 mgAEkg )1 ) prior to MRI. Sedation was augmented with 0.05 mgAEkg )1 doses of i.v. midazolam (maximum 0.1 mgAEkg )1 ) as necessary. Adverse effects, including hypoxaemia, failed sedation, paradoxical reactions and behavioural changes, the return of baseline activity, and parental satisfaction were documented. The quality of MRI scans was evaluated by a radiologist blinded to the sedation technique. Results: PB facilitated an earlier onset of sedation (P ¼ 0.001), higher sedation scores (P ¼ 0.01), and less need for supplemental midazolam compared with CH. Severe hypoxaemia occurred in two children (6%) in the PB group. Fourteen per cent of the PB group experienced a paradoxical reaction, 9% sedation failure and 11% major motion artefact, compared with 0% (P ¼ 0.05), 3 and 2% (P ¼ NS), respectively, in the CH group. CH and PB were both associated with a high incidence of motor imbalance, and agitation. However, children who received PB had a slower return to baseline activity (P ¼ 0.04). Conclusions: Although PB facilitated a quicker sedation onset and reduced the requirement for supplemental sedation, it produced a higher incidence of paradoxical reaction and prolonged recovery with a similar failure rate compared with CH.

A 2-year-old boy with blindness as an isolated symptom was found to have no light perception binocularly because of compression of both optic nerves by a neuroblastoma infiltrating the walls of the optic canals and medial sphenoid bone. Imaging disclosed a primary tumor near the kidney and multiple osseous metastases. Although neuroblastoma commonly causes blindness by metastasis to the orbit, it rarely causes bilateral blindness from intracranial compression of the optic nerves. This is the first report of bilateral blindness as the presenting feature.

Assessment of the effectiveness of cancer therapy traditionally relies on comparison of tumor images acquired before and after therapeutic intervention by inspection of gross anatomical images to evaluate changes in tumor size. The potential for imaging to provide additional insights related to the therapeutic impact would be enhanced if a specific parameter or combination of parameters could be identified that reflect tissue changes at the cellular or physiological level. This information could also provide a more sensitive and earlier indicator of treatment response in an individual animal or patient. Diffusion magnetic resonance imaging can detect relatively small changes in tissue structure at the cellular level and thus provides an opportunity to quantitatively and serially follow therapeutic-induced changes in solid tumors. This article provides an overview of the use of diffusion magnetic resonance imaging as a surrogate marker for quantitating treatment responsiveness in both preclinical and clinical studies.

Background Brain magnetic resonance imaging (MRI) findings during acute cerebellar ataxia in cases of postinfectious cerebellitis are frequently normal. This has resulted in the use of other imaging modalities, such as single-photon emission computed tomography, to aid diagnosis. Objective To illustrate the chronologic occurrence of cerebellar ataxia, abnormal findings on MRI, and cerebral spinal fluid pleocytosis in an adult case of postinfectious cerebellitis. Methods Case report. Results A patient with a 6-week history of occipital headaches and only mild tandem gait difficulty had abnormal MRI findings that were consistent with cerebellar inflammation. As cerebellar ataxia progressed in parallel with cerebral spinal fluid pleocytosis, MRI findings indicative of cerebellar inflammation resolved, while single-photon emission computed tomography showed cerebellar hyperperfusion. Recovery of neurologic function was accompanied by clearing of the pleocytosis and residual MRI-detected cerebellar atrophy. Conclusion This case demonstrates that transient abnormalities can be detected by MRI before clinical manifestations of cerebellitis appear, while hyperperfusion detected by single-photon emission computed tomography is prolonged.

Objective: To characterize the otologic phenotype in a family with autosomal dominant stapes ankylosis, hyperopia, and skeletal abnormalities caused by a mutation in the noggin gene (NOG). Study Design: Case series. Setting: Academic tertiary care center. Patients: Eight affected and 3 unaffected family members. Main Outcome Measures: History, physical and radiologic examination, and surgical outcomes. Results: Although affected members were initially presumed to have typical nonsyndromic otosclerosis, the clinical data were most consistent with an autosomal dominant congenital stapes ankylosis syndrome. Eight of eight affected family members had bilateral low-frequency conductive hearing loss. Six of eight underwent fenestration procedures and/or stapedectomies. All members with initial postoperative closure of the air-bone gap returned to their baseline conductive loss within 2 years. Two affected family members had documented maximal conductive hearing loss by age 4, and two members without previous otologic surgery have not experienced sensorineural hearing loss. High-resolution temporal bone computed tomography showed stapes ankylosis and indistinction of the incudomalleal junction bilaterally and bony regrowth over the stapedotomy for those with stapedectomies. Detailed physical and radiologic examination identified multiple other skeletal abnormalities. Conclusions: Although this phenotype may present as classic otosclerosis to the otolaryngologist, detailed investigation revealed a congenital stapes ankylosis syndrome. Because NOG is essential in regulating normal bone development and maturation, mutations in this gene may be associated with excessive bony overgrowth and refixation of the stapes footplate after initial successful surgery. Patients with hereditary conductive hearing loss should be assessed to rule out subtle features of a skeletal syndrome.

PURPOSE: To display the characteristic magnetic resonance imaging (MRI) abnormalities associated with hemifacial spasm caused by compression of the seventh cranial nerve by a dolichoectatic vertebral artery. DESIGN: Interventional case report. METHODS: Correlation of imaging and clinical findings. RESULTS: In a 36-year-old man with mild left hemifacial spasm, MRI showed an enlarged (dolichoectatic) vertebral artery that compressed the seventh cranial nerve at its exit from the caudal pons. Manifestations of hemifacial spasm were relieved with periocular botulinum toxin injections. CONCLUSION: In hemifacial spasm, MRI may show compression of the ipsilateral seventh cranial nerve or other structural abnormalities. Relief of hemifacial spasm may occur with botulinum toxin injection or with neurosurgical intervention designed to relieve the compression.