Managing predefined templates and macros for a departmental speech recognition system using common software.

The authors have developed a networked database system to create, store, and manage predefined radiology report definitions. This was prompted by complete departmental conversion to a computer speech recognition system (SRS) for clinical reporting. The software complements and extends the capabilities of the SRS, and 2 systems are integrated by means of a simple text file format and import/export functions within each program. This report describes the functional requirements, design considerations, and implementation details of the structured report management software. The database and its interface are designed to allow all radiologists and division managers to define and update template structures relevant to their practice areas. Two key conceptual extensions supported by the template management system are the addition of a template type construct and allowing individual radiologists to dynamically share common organ system or modality-specific templates. In addition, the template manager software enables specifying predefined report structures that can be triggered at the time of dictation from printed lists of barcodes. Initial experience using the program in a regional, multisite, academic radiology practice has been positive.

Quantitative assessment of brainstem development in Joubert syndrome and Dandy-Walker syndrome.

Key features of Joubert syndrome include developmental delay, hypotonia, hyperpnea and apnea, oculomotor apraxia, and the presence of the molar tooth sign on axial imaging through the brainstem isthmus--the junction of the pons and mesencephalon. Interestingly, 1 in 10 patients with Joubert syndrome has abnormal cerebrospinal fluid collections misdiagnosed as Dandy-Walker variants. Because of important differences in patient management, genetic counseling, and prognosis between these conditions, we undertook a study to determine if the brainstem isthmus is normal in Dandy-Walker syndrome. Using standard landmarks, we evaluated development of the isthmus in normal subjects and in subjects with Joubert syndrome and Dandy-Walker syndrome. Four of five brainstem measures increased with age in normal subjects. In subjects with Joubert syndrome, the depth and length of the interpeduncular fossa were increased, and the width of the isthmus was decreased. In subjects with Dandy-Walker syndrome, the width of the brainstem isthmus was normal, and the molar tooth sign was absent. Although the pons can be hypoplastic in Dandy-Walker syndrome, we conclude that the pontomesencephalic junction is normal. Thus, the molar tooth sign can effectively distinguish between Joubert and Dandy-Walker syndromes. Genetic heterogeneity or epigenetic factors may account for abnormal cerebrospinal fluid collections in some cases of Joubert syndrome.

Listeria spinal cord abscess responsive to trimethoprim-sulfamethoxazole monotherapy.

OBJECTIVE: To describe an alternative antibiotic regimen for the treatment of central nervous system Listeria monocytogenes infection. BACKGROUND: Classical treatment of listeria infections of the brain and spinal cord has included ampicillin in combination with gentamicin and chloramphenicol. Antibiotic resistance to L. monocytogenes is extraordinarily low, and the combined risks of nephrotoxicity, ototoxicity, and agranulocytosis in an already critically ill patient make the potential use of trimethoprim-sulfamethoxazole monotherapy for coverage or treatment of listeria an important alternative. METHODS: Case report. RESULTS: A 58-year-old woman presented with a two-week history of progressive quadriplegia. Gadolinium enhanced MRI showed diffuse edema of the cervical and thoracic spine with ring-enhancing lesions. Cerebrospinal fluid and blood cultures both grew L. monocytogenes. Spinal cord biopsy of the lesion revealed inflammation with necrosis and also grew listeria. Intravenous trimethoprim-sulfamethoxazole (8 mg/kg in four divided doses) was administered for six weeks with resultant arrest of neurological symptoms and stabilization of the clinical course. Although the patient was quadraparetic she was able to be discharged to a rehabilitation facility. CONCLUSIONS: Trimethoprim-sulfamethoxazole monotherapy may be a potential alternative option for critically ill patients with central nervous system L. monocytogenes infection.

A neuropsychological outcome study of a child's left pericallosal arteriovenous malformation with occult fornix lesion.

Pericallosal arteriovenous malformations are rarely reported, particularly in children. Moreover, few arteriovenous malformation studies report thorough neuropsychological outcome data for assessing post-surgical functioning. This case report provides a longitudinal study of a boy who initially presented for neuropsychological testing at the age of 8 years and 1 month, following polyvinyl alcohol embolization, two craniotomies and resections and stereotactic radiosurgery for a pericallosal arteriovenous malformation involving nearly all of the corpus callosum. Follow-up magnetic resonance imaging also indicated absence of the left fornix. Functioning has been assessed over 7 years. Neuropsychological measures identified a consistent pattern of verbally mediated cognitive and memory deficits, with relatively spared visual perceptual and visual motor functioning. No evidence of a split-brain syndrome was found. The findings are consistent with insult to dominant hemisphere language and memory systems, provide an interesting example of neurodevelopmental compensation for significant early brain insult, and may provide insight into functions subserved by the fornix.

Magnetic resonance imaging features and classification of central nervous system malformations in Joubert syndrome.

This article examines the magnetic resonance imaging features that typify the Joubert malformation. Specific morphologic features include: (1) dysgenesis of the isthmic portion of the brain stem at the pontomesencephalic junction, (2) abnormally thick superior cerebellar peduncles perpendicular to the brain stem, (3) hypoplasia of the cerebellar vermis with consequent enlargement of the 4th ventricle and rostral shift of the fastigium, and (4) sagittal vermic clefting. At least two of these features were present in every patient and all were present in some. The only cerebral anomaly identified was mild prominence of the ventricles and subarachnoid spaces. The "Joubert-plus anomaly" has been defined as the Joubert malformation plus additional anomalies of either the mesencephalon or the caudal 4th ventricle; this likely represents a similar but more extensive embryologic defect. By placing a relative numeric value on each morphologic feature, a classification scheme has been created that can quantitate the extent of the Joubert malformation in any individual case.

Molar tooth sign in Joubert syndrome: clinical, radiologic, and pathologic significance.

Joubert syndrome is a rare autosomal-recessive condition characterized by early hyperpnea and apnea, developmental delay, and truncal ataxia. We previously described key ocular motor signs in Joubert syndrome and the molar tooth sign resulting from dysplasia of the isthmic segment of the brain stem, superior cerebellar peduncles, and vermis. In this study, we obtained clinical and developmental data in 61 cases, and radiologic data in 46 of these, to determine the prevalence of the molar tooth sign in a large sample, and to ensure that magnetic resonance images obtained for study were representative of the Joubert syndrome population at large. We studied the morphology of the isthmic segment of the pontomesencephalic junction, the segment of the brain stem derived from the primitive isthmus. Portions of the cerebellum analyzed included the superior cerebellar peduncles, the anterior and posterior lobes of the vermis, and the flocculonodular lobe. In one case, autopsy of the brain was performed. The average age at diagnosis was 33 months. All patients were hypotonic and developmentally delayed. The molar tooth sign was present in 85% of cases with 13% of these showing additional malformations. All patients without the molar tooth sign had other mimicking conditions such as neocerebellar dysgenesis, isolated vermian atrophy, cerebellar aplasia, and cystic dilation of the cisterna magna. Autopsy showed aplasia of the cerebellar vermis with dysplasia of the dentate nucleus, elongated locus coeruleus, and marked dysplasia of the caudal medulla. A better understanding of the clinical, radiologic, and pathologic features of Joubert syndrome should help uncover the genetic basis for the syndrome.

The importance of cerebrospinal fluid lactate in the evaluation of congenital lactic acidosis.

In 27 of 28 children with congenital lactic acidosis, cerebrospinal fluid lactate was higher than venous blood lactate. The mean +/- SEM difference between these variables was 2.4 +/- 0.3 mmol/L (P =.0001). Girls or patients with pyruvate dehydrogenase deficiency had higher cerebrospinal fluid lactate concentrations than boys or patients with respiratory chain defects or mitochondrial DNA mutations.

Neurobehavioral development in Joubert syndrome.

Research on children with Joubert syndrome has focused on brain structural abnormalities and associated clinical symptoms. The degree of developmental delay has not been objectively reported. We investigated the neurobehavioral development of children with Joubert syndrome through neurobehavioral assessment in the largest sample to date. Thirty-two parents of children with Joubert syndrome completed the Child Development Inventory and magnetic resonance imaging (MRI) data was gathered on 17 of these children. Results indicate that 94% were severely impaired according to the Child Development Inventory, with age being positively correlated with degree of neurobehavioral impairment. The average developmental age of our sample was 19 months (63% below chronological age). Severity of illness as measured by the General Development scale of the Child Development Inventory and severity of illness as measured by MRI (overall severity rating) did not yield consistent data regarding severity of the midbrain and cerebellar malformations. Similarly, markers of abnormal cerebral development such as cortical atrophy and delayed myelination were independent of severity of illness ratings on the Child Development Inventory. The degree of developmental delay in Joubert syndrome and the severity of gross central nervous system malformations appear independent.

Retrorectal cyst-hamartomas and sacral dysplasia: MR appearance.

Retrorectal cyst-hamartoma, an uncommon lesion, arises from hindgut embryonic remnants and may be associated with sacral anomalies. Such a lesion is presacral, multicystic, and lined with glandular or transitional epithelium. Malignant transformation of these lesions has been reported. We describe the clinical, pathologic, and imaging findings in an infant.

Calcified cystic lesions in a patient with epilepsy and progressive dementia.

A 67-year-old woman had intractable epilepsy and developed a progressive dementia with upper motor neuron signs over the last 6 years. Magnetic resonance imaging (MRI) revealed multiple areas of large calcified cysts, which increased in number and size over the last 3 years. Discussion includes the appearance of these lesions radiologically and pathologically, as well as their differential diagnosis and clinical significance, focusing on the increasing detection of these lesions with current imaging techniques.

High prevalence of bihemispheric structural and functional defects in Sturge-Weber syndrome.

Abnormal cerebral venous drainage is associated with hypoxia and glucose deprivation, which can account for progressive neurologic deterioration in Sturge-Weber syndrome. Although developmental delay is common in Sturge-Weber syndrome, bihemispheric calcification is uncommon. Computed tomography (CT) and magnetic resonance imaging (MRI) were used to study the neuroanatomy, while single photon emission computed tomography (SPECT) was used concurrently to evaluate perfusion and glucose metabolism using 99mTc hexamethylpropyleneamine oxime (HMPAO) and [18F] fluorodeoxyglucose (FDG), respectively. Ten patients (10 to 22 years of age) with previously diagnosed Sturge-Weber syndrome, port-wine nevi, and clinical evidence of seizures or stroke-like episodes were studied. Five children with onset of seizures in the first year of life had overall clinical severity comparable to that of children with later-onset seizures. Calcification was present in both hemispheres in one patient; six additional patients had other radiologic evidence of bihemispheric disease; SPECT studies detected bihemispheric disease in four cases. Our study is the first to concurrently evaluate structure, perfusion, and glucose metabolism in Sturge-Weber syndrome and to show a mismatch between functional and structural brain imaging in both cerebral hemispheres. Widespread abnormalities of cerebral perfusion and glucose metabolism might explain the high prevalence of developmental delay associated with Sturge-Weber syndrome. Longitudinal studies are needed to define better the natural history of neurologic deterioration and radiologic progression that relates to central nervous system circulatory dysfunction in Sturge-Weber syndrome.

Central nervous system structure and function in Sturge-Weber syndrome: evidence of neurologic and radiologic progression.

Sturge-Weber syndrome is characterized by the presence of a port-wine nevus, epilepsy, stroke-like episodes, headache, and developmental delay. We studied 20 cases to test the hypothesis that decreased cerebral blood flow alters neurologic function by affecting cellular glucose metabolism. Group A consisted of 10 patients with a mean age of 1.75 years and early seizure onset (6.8 months), whereas group B was composed of older patients (mean age, 15.3 years) with later onset of seizures (3.7 years). Neurologic disease was more severe in group A, but group B had more widespread structural brain defects - shown on computed tomographic scans and magnetic resonance imaging - and metabolic brain defects shown on hexamethylpropyleneamine oxime and [18F] fluorodeoxyglucose single photon emission computed tomographic scans. Six group A cases had hypoperfusion at baseline and five of nine had worsening of perfusion and glucose metabolism 1 year later. A total of 119 stroke-like episodes occurred in six group A cases and eight group B cases; there were 65% fewer strokes in children treated with aspirin. The data suggest that progressive hypoperfusion and glucose hypometabolism are associated with neurologic deterioration in Sturge-Weber syndrome. Longitudinal studies are needed to better define the natural history of disease and to evaluate the safety and efficacy of aspirin therapy.

"Joubert syndrome" revisited: key ocular motor signs with magnetic resonance imaging correlation.

Joubert syndrome is characterized by episodic hyperpnea and apnea, developmental delay, hypotonia, truncal ataxia, ophthalmologic abnormalities, and vermian dysgenesis. We studied 15 patients with the diagnosis of Joubert syndrome to (1) more fully define the syndrome's clinical features, and (2) correlate the clinical features with magnetic resonance imaging (MRI) findings. Eight of 15 patients had a history of episodic hyperpnea and apnea. All patients had developmental delay and hypotonia. Of the 13 patients receiving detailed neuro-ophthalmologic evaluations, three had optic nerve dysplasia, pendular nystagmus, and gaze-holding nystagmus. All 13 patients had a normal vestibulo-ocular reflex based on head thrust, but had absent to poor ability to cancel the vestibulo-ocular reflex horizontally and vertically. Twelve of 13 patients had impaired smooth pursuit. Twelve of 13 patients had defects in initiation of saccades and quick phases. Two of the most consistent radiologic features were absent or hypoplastic posterior cerebellar vermis, and deformed midbrain and pontomesencephalic junction, which based on ocular motor physiology correlate with the vestibulo-ocular reflex cancellation/ pursuit defect and saccade initiation defect, respectively. As a result of midbrain, vermian, and superior cerebellar peduncle abnormalities, axial neuroimaging showed a unique "molar tooth" appearance of these structures. These results indicate that Joubert syndrome results from maldevelopment of the midbrain and cerebellar vermis, producing a pathognomonic sign on MRI.

Correlation between gadolinium-diethylenetriaminepentaacetic acid contrast enhancement and thallium-201 chloride uptake in pediatric brainstem glioma.

We previously showed that thallium-201 (201Tl) chloride is accumulated in over 75% of brain tumors, including brainstem gliomas. The imaging of 201Tl with single photon emission computed tomography (SPECT) may require an abnormal increase in permeability of tumor vessels to allow penetration of the blood-brain barrier. To test this hypothesis, we evaluated the correlation between gadolinium enhancement and the degree of 201Tl uptake on SPECT and the contributions of either gadolinium enhancement or 201Tl uptake to the prognosis in children with brainstem gliomas. Forty-two sets of paired SPECT scans and magnetic resonance imaging (MRI) scans were obtained longitudinally in 13 cases. Altogether, 31 of 42 pairs (74%) of scans showed concordance between the presence of gadolinium enhancement and 201Tl uptake. There were no cases that demonstrated 201Tl uptake but lacked gadolinium enhancement. The results indicate that 201Tl SPECT is of value primarily when brainstem tumors have vessels that are demonstrably permeable to gadolinium, prior to or as a result of radiotherapy.

Primary intracranial germ cell tumors: clinicopathologic review of 32 cases.

Primary intracranial germ cell neoplasms are rare tumors and constitute a heterogeneous group. We have reviewed 32 cases, over a 21-year period, from the University of Florida. The cases include 22 germinomas, 6 mixed germ cell tumors, and 4 teratomas. The clinical presentations in these cases were more closely related to the location of the tumor, that is, pineal or suprasellar, rather than the histologic subtype. Neuroimaging evaluation was useful in distinguishing between germinomas, teratomas, and other mixed germ cell tumors (MGCTs), primarily by evaluation of cystic versus solid lesions (teratoma versus germinoma), contents of cysts (teratoma versus MGCT), and infiltrative nature of the tumors (MGCT), although cytologic-histopathologic confirmation remains necessary. Germinomas responded favorably to radiation therapy with survival periods of over 16 years; MGCTs were treated with combination chemotherapy and radiation, with a markedly poorer prognosis. This study underlines the critical significance of histopathologic evaluation of the tumor in determining therapeutic interventions as well as prognosis.

Imaging for pediatric brain tumors.

Radiological imaging plays an important role in the care of pediatric and adolescent patients undergoing evaluation and treatment of brain tumors. The primary objective for any diagnostic magnetic resonance or computed tomography study is to distinguish between normality and abnormality. Establishing normality is typically more difficult than recognizing obvious pathology. To declare that an imaging procedure reveals either no apparent or no significant disease requires the examination to have been performed appropriately with the most sensitive imaging modality, scanned in the right location, using parameters capable of providing the optimum spatial and contrast resolution. Once tumor is observed, the role of imaging then shifts toward more specialized tasks including predicting the type of tumor, establishing the spatial relationship between the mass and eloquent areas, and staging the furthest extent of disease. These tasks require sophisticated imaging and experienced interpretation to adequately plan treatment.

Intractable temporal lobe epilepsy: comparison of positron emission tomography with qualitative and quantitative MR.

PURPOSE: To compare the ability of qualitative fludeoxyglucose F 18 positron emission tomography (QPET), qualitative MR imaging (QMR), and quantitative MR imaging with hippocampal formation volumetric assessment (HV MR) to lateralize the seizure focus in patients with temporal lobe epilepsy. METHODS: Sixteen consecutive patients undergoing presurgical examination for temporal lobe seizures had QPET, QMR, and HV MR. The presence of temporal lobe epilepsy was confirmed by Engel class I or II outcomes at 1-year postoperative follow-up examinations. A QPET, QMR, or HV MR study was considered to be lateralizing if it matched the side of the seizure focus, nonlateralizing if it did not lateralize the seizure focus to either temporal lobe, or incorrectly lateralizing if it lateralized the seizure focus to the incorrect side. RESULTS: Of 16 patients with proved temporal lobe seizures, QPET was correctly lateralizing in nine (56%), nonlateralizing in six (37.5%), and incorrectly lateralizing in one (6%). QMR was correctly lateralizing in six (37.5%), nonlateralizing in six (37.5%), and incorrectly lateralizing in four (25%). HV MR was correctly lateralizing in all 16 patients (100%). Age at onset, seizure duration, and total number of seizures did not correlate with QPET, QMR, and HV MR lateralization. CONCLUSIONS: Our results show that each imaging technique yields useful information for seizure lateralization in temporal lobe epilepsy and that HV MR yields considerably more information that QPET or QMR.