11 research outputs found
A Brazilian family with Brown-Vialetto-van Laere syndrome with autosomal recessive inheritance
Infantile spasms in an infant with cytomegalovirus infection treated with ganciclovir
A 3-month-old male infant with cytomegalovirus infection and intractable
partial seizures was treated with ganciclovir for 6 weeks. The drug was
well tolerated, and virus shedding in the cerebrospinal fluid and urine
was eliminated, although infantile spasms at the age of 6 months
appeared. At the age of 12 months, intractable seizures persisted, and
the psychomotor development of the infant was markedly delayed. To our
knowledge, no previous similar case has been reported. These findings
suggest that treatment with ganciclovir of infants with cytomegalovirus
infection results only in cessation of virus shedding in the
cerebrospinal fluid and urine without having a preventive effect on the
future appearance of infantile spasms. This may be due to the
irreversibility of previous brain damage from the cytomegalovirus
infection and the virostatic nature of the drug
Long-term findings on brain magnetic resonance imaging in acute encephalopathy with bilateral striatal necrosis associated with measles
The long-term findings on brain magnetic resonance imaging (MRI) in a 7
10/12-year-old boy with a history of acute encephalopathy with bilateral
striatal necrosis following measles at the age of 22 months are
described. At the early stage of illness, brain MRI studies revealed
bilateral, symmetric basal ganglia lesions, predominant on the globi
pallidi, appearing as hyperintense signals on T-1- and T-2 weighted
images. Six years later, follow-up brain MRI studies showed that the
bilateral, symmetric lesions on the globi pallidi persisted with low
signal on T-1- and high signal on T-2 weighted images. At present, the
patient has some persistent neurologic signs. These findings suggest
that both clinical and neuroradiologic findings may persist in children
with acute encephalopathy with bilateral striatal necrosis following
measles
Acute Necrotizing Encephalopathy Associated With Parainfluenza Virus in a Caucasian Child
Acute necrotizing encephalopathy associated with parainfluenza virus in a Caucasian child
Acute necrotizing encephalopathy is a severe parainfectious disorder
with a clear racial predilection for Oriental children living in the Far
East. The prognosis was originally reported as grave; however, a mild
form of the disease has recently been described. A case of parainfluenza
virus-associated acute necrotizing encephalopathy in a Caucasian child
with a mild clinical course and excellent prognosis is presented. In
this patient, the initial clinical picture was not very impressive, and
the diagnosis was delayed until the third week of the illness, when
neuroimaging was performed. Two months later, clinical and neuroimaging
findings had almost completely resolved. Suggested criteria for a benign
prognosis, such as normal liver function and cerebrospinal fluid protein
levels, asymmetric thalamic lesions, and no brainstem involvement, were
relevant in the present case. An extended diagnostic work-up for
metabolic, vascular, coagulation, and infectious diseases was negative
apart from a seroconversion for parainfluenza virus. To our knowledge,
this is the first reported case of acute necrotizing encephalopathy
associated with parainfluenza virus infection. Acute necrotizing
encephalopathy, especially in the mild form, might not be fully
recognized and could be underdiagnosed in Europe, where the reported
incidence of the syndrome is very low
Serum amylase, pancreatic amylase and lipase concentrations in epileptic children treated with carbamazepine monotherapy
Background: Serum total amylase and lipase activities have been
determined in epileptic patients treated with polytherapy using
enzyme-inducing anticonvulsant drugs; however, to our knowledge, serum
total amylase, pancreatic amylase and lipase activities have not
previously been determined in patients receiving carbamazepine
monotherapy. The purpose of this study was to investigate by a
prospective, self-controlled method, whether early treatment with
carbamazepine monotherapy may alter serum total amylase, pancreatic
amylase and lipase concentrations of epileptic children.
Methods: Serum total amylase, pancreatic amylase and lipase activities
have been determined in 18 epileptic children before and at 6 and 12
months of treatment with carbamazepine monotherapy. Serum
gamma-glutamyltransferase activities were also determined.
Results: Serum total amylase concentrations were significantly increased
at 6 months of treatment (p=0.034), and serum nonpancreatic amylase
concentrations were significantly increased at 6 (p=0.016) and 12 months
of treatment (p=0.039), whereas serum pancreatic amylase and lipase
concentrations did not significantly change at 6 or 12 months of
treatment with carbamazepine monotherapy. Furthermore, serum
gamma-glutamyltransferase concentrations were significantly increased at
6 (p=0.000) and 12 months of treatment (p=0.000) with carbamazepine
monotherapy. There was no significant correlation between serum
nonpancreatic amylase concentrations and serum gamma-glutamyltransferase
or carbamazepine concentrations at 6 and 12 months of treatment with
carbamazepine monotherapy.
Conclusions: These findings indicate that nonpancreatic amylase
concentrations may be increased in patients treated with carbamazepine
monotherapy. Therefore, measurement of serum pancreatic amylase and
lipase concentrations is suggested in epileptic patients receiving
carbamazepine monotherapy with symptoms suggesting pancreatic
dysfunction, so that unnecessary discontinuing of treatment with
carbamazepine should be avoided. (C) 2004 Elsevier B.V. All rights
reserved