Reversible leukoencephalopathy with acute neurological deterioration and permanent residua in classical homocystinuria: A case report.

We report a 24-year-old patient with underlying classical homocystinuria who developed acute neurological deterioration apparently induced by malnutrition secondary to poor compliance with treatment and pancreatitis. Neurological examination revealed stupor, tremor of the upper extremities, spasticity, and Babinski responses of the bilateral lower extremities. The biochemical profile, including marked hypermethioninaemia, hyperhomocysteinaemia, and decreased cystine in plasma, is consistent with cystathionine ß-synthase deficiency. Brain MRI showed reversible diffuse white-matter changes without evidence of thrombosis. The clinical features and neuroimaging including diffusion-weighted MRI suggest a demyelinating process similar to other inborn errors of the transsulfuration pathway such as methylenetetrahydrofolate reductase deficiency, disorders affecting methylcobalamin metabolism (cobalamin C, D, E, and G disorders), and methionine adenosyltransferase deficiency. She was left with residual spastic paraparesis despite normal follow-up MRI. Our observation suggests that reversible demyelination is another neurological manifestation of classical homocystinuria; however, the pathophysiology is unknown.

Posterior reversible encephalopathy syndrome: another manifestation of CNS SLE?

A variety of neuropsychiatric findings may complicate systemic lupus erythematosus (SLE) and pose diagnostic and therapeutic dilemmas. We describe the clinical and radiographic features of posterior reversible encephalopathy syndrome (PRES) and distinguish PRES from other conditions seen in SLE. Patient charts and magnetic resonance imaging (MRI) findings of four patients with SLE on immunosuppressive therapy with acute or subacute neurologic changes initially suggesting cerebritis or stroke were reviewed. The English language literature was reviewed using the Medline databases from 1996-2006 for other reports of PRES with SLE. Literature review yielded 26 other SLE cases reported with PRES. SLE patients with PRES were more commonly on immunosuppressive drugs, had episodes of relative hypertension, and had renal involvement. Characteristic findings are seen on MRI, which differentiate PRES from other CNS complications of SLE. Clinical and radiographic resolution of abnormalities within 1-4 weeks is typically seen. PRES has been increasingly recognized. Reversible changes are found on brain MRI accompanied by sometimes dramatic signs and symptoms. The therapeutic implications for separating PRES from stroke or cerebritis are important. We propose that PRES should be considered in the differential diagnosis in SLE patients with new-onset neurologic signs and symptoms.

A prospective multicenter study of cervical spine injury in children.

OBJECTIVE: Pediatric victims of blunt trauma have developmental and anatomic characteristics that can make it difficult to assess their risk of cervical spine injury (CSI). Previous reports, all retrospective in nature, have not identified any cases of CSI in either children or adults in the absence of neck pain, neurologic symptoms, distracting injury, or altered mental status. The objective of this study was to examine the incidence and spectrum of spine injury in patients who are younger than 18 years and to evaluate the efficacy of the National Emergency X-Radiography Utilization Study (NEXUS) decision instrument for obtaining cervical spine radiography in pediatric trauma victims. METHODS: We performed a prospective, multicenter study to evaluate pediatric blunt trauma victims. All patients who presented to participating emergency departments underwent clinical evaluation before radiographic imaging. The presence or absence of the following criteria was noted: midline cervical tenderness, altered level of alertness, evidence of intoxication, neurologic abnormality, and presence of painful distracting injury. Presence or absence of each individual criterion was documented for each patient before radiographic imaging, unless the patient was judged to be too unstable to complete the clinical evaluation before radiographs. The decision to radiograph a patient was entirely at the physician's discretion and not driven by the NEXUS questionnaire. The presence or absence of CSI was based on the final interpretation of all radiographic studies. Data on all patients who were younger than 18 years were sequestered from the main database for separate analysis. RESULTS: There were 3065 patients (9.0% of all NEXUS patients) who were younger than 18 years in this cohort, 30 of whom (0.98%) sustained a CSI. Included in the study were 88 children who were younger than 2, 817 who were between 2 and 8, and 2160 who were 8 to 17. Fractures of the lower cervical vertebrae (C5-C7) accounted for 45.9% of pediatric CSIs. No case of spinal cord injury without radiographic abnormality was reported in any child in this study, although 22 cases were reported in adults. Only 4 of the 30 injured children were younger than 9 years, and none was younger than 2 years. Tenderness and distracting injury were the 2 most common abnormalities noted in patients with and without CSI. The decision rule correctly identified all pediatric CSI victims (sensitivity: 100.0%; 95% confidence interval: 87.8%-100.0%) and correctly designated 603 patients as low risk for CSI (negative predictive value: 100.0%; 95% confidence interval: 99.4%-100.0%). CONCLUSIONS: The lower cervical spine is the most common site of CSI in children, and fractures are the most common type of injury. CSI is rare among patients aged 8 years or younger. The NEXUS decision instrument performed well in children, and its use could reduce pediatric cervical spine imaging by nearly 20%. However, the small number of infants and toddlers in the study suggests caution in applying the NEXUS criteria to this particular age group.

Resolution of pachymeningeal enhancement following dural puncture and blood patch.

We describe thickening and contrast enhancement of the intracranial pachymeninges, revealed by MRI in a patient with presumed low-pressure headache following dural puncture and a blood patch. The clinical and radiological abnormalities resolved within 2 weeks.

MR imaging of temporomandibular joint abnormalities associated with cervical hyperextension/hyperflexion (whiplash) injuries.

Patients often have temporomandibular joint (TMJ) dysfunction-related symptoms after cervical hyperextension/hyperflexion injuries ("whiplash") caused by rear-end motor-vehicle collisions. To determine abnormalities of the TMJ associated with these injuries, 33 consecutive symptomatic patients (66 joints) with no direct trauma to the jaw, mouth, head, or face due to the accident and no prior history of TMJ dysfunction underwent magnetic resonance (MR) imaging, and the images were retrospectively analyzed. Overall, 29 (88%) patients had some type of TMJ abnormality related to whiplash injury. Displacement of the disk was seen in 37 (56%) of the TMJs as follows: 21 (32%) had anterior displacement with reduction, nine (14%) had anterior displacement without reduction, six (9%) had lateral or medial displacement, and one (2%) had posterior displacement. On T2-weighted images, 43 (65%) TMJs had abnormal joint fluid or edema, predominantly affecting the joint capsule and/or lateral pterygoid muscles. The finding that many of the patients had joint fluid and/or soft-tissue edema indicates that T2-weighted images are especially useful for assessment of patients with a history of whiplash injury.

Kinematic magnetic resonance imaging of the joints: techniques and clinical applications.

Recently, kinematic magnetic resonance imaging (MRI) techniques have been developed to provide diagnostic information related to the functional aspects of the joints. The kinematic MRI evaluation of the joint is used to assess the various interactions of the soft tissues and bony anatomic structures that comprise the joint, and to evaluate the relative alignment of these structures through a specific range of motion. The use of kinematic MRI techniques for examination of the joints provides the radiologist and the clinician with augmented data of anatomic and movement-related information.

An early CT sign of ischemic infarction: increased density in a cerebral artery.

The diagnosis of ischemic cerebral infarction by CT usually is not possible for 12-24 hr after the event. A sign that allows earlier diagnosis is increased density of a cerebral vessel secondary to a thrombus or embolus. We report 19 cases of increased density in a middle cerebral artery or one of its major branches visible on the initial CT scan of patients subsequently clinically proven to have suffered a cerebrovascular accident. In three cases angiography confirmed the presence of embolus or thrombus as seen on the CT scans. In several cases subsequent CT scans showed the density to dissipate, thereby confirming its nature as thrombus or embolus. Recognition of this finding will allow earlier diagnosis of ischemic infarction, which may be important in instituting an appropriate therapeutic regimen.

Low-dose metrizamide spinal computed tomography in outpatients.

Delineation of the thecal sac in CT can be improved by the presence of intrathecal metrizamide. This may be especially helpful in postoperative patients in whom the landmarks are often obscured by epidural scarring. Metrizamide-enhanced CT was performed on outpatients using 2 1/2 ml metrizamide (170 mg I/ml). Follow-up is available on 40 patients. The procedure was well tolerated with only three instances of severe headache, one case of vomiting, and no reported seizures. Adequate opacification was obtained at 92% of the levels examined with fair opacification at the remainder of the levels. This technique is considered a safe and useful outpatient procedure.

CT-guided stereotactic placement of ventricular cannula.

Cannulation of a small lateral ventricle for the injection of medication was achieved by using a CT-guided stereotactic technique. Methodology is discussed.

Laryngeal granular cell myoblastoma two case reports with radiographic demonstration.

Laryngeal granular cell myoblastoma is a rare, benign lesion. Two cases were studied by contrast laryngography and demonstrated similar, non-specific findings of a smooth, sessile mass.

Posterior fossa hemorrhage. Localization by computerized tomography.

The differentiation between pontine and cerebellar hemorrhage is important both prognostically and therapeutically but is often very difficult. Computerized axial tomography of the brain is a new, rapid, nonivasive method of studying intracranial disease. This technique proved useful in making this important differential diagnosis in the case reported here.

Histiocytosis-X of the hypothalamus.

Hypothalamic masses associated with histiocytosis-X may be mistaken for neoplasms. As a result radiation therapy will often be administered rather than chemotherapy, which appears to be the treatment of choice for histiocytosis-X of the hypothalamus. Particularly in young people, the possibility of granulomatous disease should be entertained and biopsy should be considered in the presence of a hypothalamic mass.