Computer analysis of magnetic resonance imaging of the brain in children and adolescents after treatment of diabetic ketoacidosis.

Cerebral vascular accidents are one of the causes of morbidity and mortality in children with diabetic ketoacidosis. We investigated the possible occurrence of asymptomatic cerebrovascular infarcts and the course of subclinical brain edema in six patients. Neurologic examinations and computer analysis of magnetic resonance imaging were performed immediately after, and again at 14 days after, correction of DKA. None of the patients had clinical evidence of a neurologic deficit. Neither radiologic evaluation nor computer analysis of MRI identified changes indicating asymptomatic ischemic events. However, a computer analysis of the MRI identified a significant increase of the total ventricle area between Day one and Day 14. Our study does not establish whether this change is a return to the baseline prior to DKA or a new baseline, representing an early manifestation of diabetic encephalopathy.

Magnetic resonance imaging of the forearm as a diagnostic aid in patients with sporadic inclusion body myositis.

Because weakness of finger flexors and atrophy of the forearms are frequent findings in inclusion body myositis (IBM) patients, we examined the forearm muscles by MRI to determine if involvement of the distal musculature has a characteristic diagnostic pattern. We performed MRI of the forearms in 21 randomly selected patients with histologically confirmed IBM and in 9 patients with other, age-matched, neuromuscular diseases who served as controls. In addition, we analyzed axial images of 10 individual forearm muscles blindly without knowledge of the clinical status or diagnosis of the patients. T1-weighted MR images showed marbled brightness of the flexor digitorum profundus (FDP) in 20 of 21 IBM patients, of the flexor carpi ulnaris in 7, the flexor digitorum superficialis (FDS) in 6, the flexor carpi radialis in 4, the supinator in 3, and the brachioradialis in 1. The extensors were normal. The abnormalities of the FDP correlated with the severity but not the duration of the disease and in some patients preceded overt clinical signs of FDP weakness. In contrast, the FDS was spared even late in the disease. We conclude that selective involvement of the FDP may occur early in the course of IBM and can be easily demonstrated by MRI in up to 95% of patients. Because selective FDP involvement appears to be a very frequent and characteristic finding in patients with IBM, MRI of the forearm is a useful noninvasive test in supporting the diagnosis of sporadic IBM.

A controlled study of intravenous immunoglobulin in demyelinating neuropathy with IgM gammopathy.

Eleven patients with demyelinating polyneuropathy associated with monoclonal IgM antibodies were randomized to receive IVIg or placebo, monthly, for 3 months in a double-blind study. After a washout period, they crossed over to the alternate therapy. Response was gauged by evaluating muscle strength, sensation, and neuromuscular symptoms at baseline, after 3 months, and at treatment's end. After IVIg therapy, the strength improved in only 2 of 11 patients, by 28 and 38.5 points from baseline, and declined after placebo. In 1 other patient, the sensory score improved by 13 points. Antibody titers to MAG/SGPG or gangliosides did not appreciably change. We conclude that IVIg has only a modest benefit to not more than 18% of patients with IgM paraproteinemic demyelinating neuropathy.

Aseptic meningitis associated with high-dose intravenous immunoglobulin therapy: frequency and risk factors.

OBJECTIVE: Intravenous immunoglobulin is widely used to treat various autoimmune disorders. After observing instances of aseptic meningitis in treated patients, we studied the frequency and associated risk factors for aseptic meningitis in patients treated with high-dose intravenous immunoglobulin. DESIGN: Retrospective analysis of a prospective cohort study. SETTING: Tertiary research referral center. PATIENTS: 54 consecutive patients with various immune-related neuromuscular diseases participating in ongoing therapeutic trials of high-dose (2 g/kg) intravenous immunoglobulin. MEASUREMENTS: Analysis of patient records for evidence of aseptic meningitis, associated risk factors, penetration of serum IgG into the cerebrospinal fluid, and clearance of cerebrospinal fluid IgG. RESULTS: Of 54 patients, 6 (11%; 95% CI, 4% to 23%) developed aseptic meningitis within 24 hours after completion of the infusions. Symptoms, lasting 3 to 5 days, included severe headache, meningismus, photophobia, and fever. Cerebrospinal fluid showed pleocytosis in 4 patients (leukocyte count as high as 1169 x 10(6)/L in one patient), eosinophilia in 3 patients, and IgG elevation in all patients (as great as 7 times the upper limit of normal in one patient). Repeat cerebrospinal fluid and serum studies after 24 hours showed a 46% cerebrospinal fluid IgG clearance compared with an 11% clearance of serum IgG in one patient. Cerebrospinal fluid cultures were negative. Aseptic meningitis developed in 4 of 8 patients (50%; CI, 16% to 84%) with a history of migraine but in only 2 of 46 (4%; CI, 0.5% to 15%) patients without such a history (P = 0.003). Aseptic meningitis recurred in patients who had migraine despite the use of different commercial intravenous immunoglobulin preparations and slower rates of infusion. CONCLUSION: Aseptic meningitis develops in patients receiving high-dose intravenous immunoglobulin therapy. Patients with a history of migraine are more likely to develop aseptic meningitis while receiving intravenous immunoglobulin therapy, regardless of the type of commercial preparation or the infusion rate. Possible inciting factors include the IgG itself, various stabilizing products within each of the preparations, cytokine release triggered by the therapy, or cerebrovascular sensitivity in migraineurs.

Electrocardiographic findings in Rett syndrome: an explanation for sudden death?

Girls with Rett syndrome had significantly longer corrected QT intervals (p < 0.001) and more T-wave abnormalities (p < 0.001) than were found in age-matched healthy girls. With advancing stages of the syndrome, the proportion of corrected QT interval prolongations and T-wave changes increased. The findings suggest a possible cardiac basis for sudden, unexpected death in Rett syndrome.

D-2-hydroxyglutaric aciduria in neonate with seizures and CNS dysfunction.

D-2-Hydroxyglutaric aciduria was documented in a newborn who presented with seizures, hypotonia, cortical blindness, a movement disorder, and developmental delay. Her clinical presentation differs from that of patients with L-2-hydroxyglutaric aciduria and a single previously reported patient with D-2-hydroxyglutaric aciduria. Cerebrospinal fluid levels of gamma-aminobutyric acid were elevated, while biogenic amine metabolites were normal. The movement disorder in our patient and in those with L-2-hydroxyglutaric aciduria suggests involvement of the basal ganglia in the disease process. Prenatal diagnosis of an affected fetus was accomplished during a subsequent pregnancy.