Head trauma is the most frequent cause of morbidity and death in abused children. The shaken impact syndrome results from both types of injury-impact and severe acceleration-deceleration effects associated with impact causing shearing forces on the bridging veins and parechyma. To make the diagnosis of shaken impact syndrome, the physician should be suspicious of every child under 1 year of age with non-accidental brain injury and altered consciousness. Certain clinical features such as retinal hemorrhage , complex, depressed, or diastatic fractures, and associated findings such as metaphyseal fracture and failure to thrive make the diagnosis of abuse more likely. Yet in the absence of these findings, the diagnosis of abuse rests upon discrepancies between the history given and the injury incurred. We experienced a case of shaken impact syndrome in a 13 month-old female who had subdural hemorrhage, fractures of skull and rib, and encephalomalacia, so we report and review the related literatures.
Brain Injuries ; Child ; Child Abuse ; Consciousness ; Craniocerebral Trauma ; Diagnosis ; Encephalomalacia* ; Failure to Thrive ; Female ; Hematoma, Subdural ; Humans ; Infant ; Retinal Hemorrhage ; Ribs ; Skull ; Veins

"Idiopathic long QT syndrome" is characterized by prolongation of the QT interval due to unusual electrocardiographic repolarization abnormality and associated with variable clinical manifestations from no specific symptoms in lifetime to syncope or even sudden death. The prognosis of this syndrome is very grave and motality is approximately 50% within 10 years among untreated symptomatic patients after the initial syncope. But this sudden onset syncope may be misdiagnosed as epilepsy, being treated with antiepileptic drug for many years. However, this high mortality has been significantly reduced to less than 5% by the effective therapy. Therefore, it is crucial to make an early and accurate dianosis. We exprienced a case of 34 months old male who presented with recurrent syncopal attacks. He had no specific neurological abnomal finding except congenital deafness. He had normal EEG and brain MRI findings but ECG showed prolonged QT interval (QTc= 0.5), findings of which were compatible with long QT syndrome. He is currently being followed at OPD, but the pateint is still experiencing syncopal attack despite of treatment with beta-blocker, atenolol. Therefore, we are considering an insertion of pacemaker or performing thoracic sympathectomy.
Atenolol ; Brain ; Child, Preschool ; Deafness ; Death, Sudden ; Electrocardiography ; Electroencephalography ; Epilepsy* ; Humans ; Long QT Syndrome* ; Magnetic Resonance Imaging ; Male ; Mortality ; Prognosis ; Sympathectomy ; Syncope

Acute motor axonal Guillain-Barr syndrome is a paralytic disorder of abrupt onset, characterized electrophysiologically by near-normal terminal latencies, preserved nerve conduction velocity with low CAMP amplitude without a conduction block, and early appearing nerve inexcitability and by sparing sensory fibers. Most cases have antecedental infection with Campylobacter jejuni and have antibodies directed toward GM1 ganglioside-like epitopes. We have experienced a case of primary axonal type of Guillain-Barr syndrome in a 14-year-old female patient, who has symptoms of difficulty in swallowing and progressive flaccid paralysis associated with anti-GM1 antibody and anti-GD1 antibody.
Adolescent ; Antibodies ; Axons* ; Campylobacter jejuni ; Deglutition ; Epitopes ; Female ; Humans ; Neural Conduction ; Paralysis

Multiple sclerosis and acute disseminated encephalomyelitis are demyelinating diseases of the central nervous system those can present initially as an acute focal demyelinating syndrome. Multiple sclerosis is characterized by a multiphasic disease with stepwise or progressive deterioration in neurologic function, whereas acute disseminated encephalomyelitis is a monophasic disease with good prognosis. So the differentiation of the two diseases in a patient with single clinical episode attributable to central nervous system demyelination is of prognostic importance, but the differentiation is not easy. We report a case of multiple sclerosis with spinal cord involvement initially misdiagnosed as acute disseminated encephalomyelitis in the brain stem.
Brain Stem ; Central Nervous System ; Demyelinating Diseases ; Encephalomyelitis, Acute Disseminated* ; Humans ; Multiple Sclerosis* ; Prognosis ; Spinal Cord

Acute encephalitis with thalamotegmental involvement in infants and children was reported in Japan, 1995. This encephalitis was preceded for several days by fever and symptoms of upper respiratory infection. These symptoms were followed by the rapid evolution of stupor and coma, associated with generalized seizures and decorticated and decerebrate rigidity without focal neurologic or meningeal signs. The prognosis was generally poor. We have experienced a case of acute encephalitis; the child had three days of fever before the hospitalization and convulsion and deteriorated mental change into a semicomatous state had developed. The patient was diagnosed as acute encephalitis with thalamotegmental involvement by magnetic resonance imaging.
Child ; Coma ; Decerebrate State ; Encephalitis* ; Fever ; Hospitalization ; Humans ; Infant ; Japan ; Magnetic Resonance Imaging ; Prognosis ; Seizures ; Stupor

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disease of the central nervous system (CNS) caused by a persistent, aberrant measles virus infection. The outcome is usually fatal. After a latent period of 6 to 7 years, there is subtle and slow cognitive decline and visuospatial disorientation develops followed by myoclonic jerks, extrapyramidal symptoms, ataxia, and seizures, progressing to coma or vegetative state. The diagnosis is based on at least three of the following criteria 1) clinical manifestations 2) abnormal EEG 3) hyperglobulinorrachia, elevated serum or spinal fluid measles antibody 4) histologic features. No therapeutic maneuver has been proven conclusively to be of value. We have diagnosed and experienced a case of subacute sclerosing panencephalitis (SSPE) in a 5-year-old child with the chief complaint of myoclonic seizure and mental deterioration. We report a case and the brief review of related literature.
Ataxia ; Central Nervous System ; Child ; Child, Preschool ; Coma ; Diagnosis ; Electroencephalography ; Humans ; Measles ; Measles virus ; Myoclonus ; Persistent Vegetative State ; Seizures ; Subacute Sclerosing Panencephalitis*

Angelman syndrome is a rare genetic disorder characterized by developmental delay, speech impairment, ataxic gait, paroxysmal laughter, and seizures. The diagnosis is suspected in infants who have the characteristic clinical features and electroencephalographic (EEG) abnormalities, and is confirmed by the genetic identification of a maternally derived 15q11-13 deletion. We report a case of genetically confirmed Angelman syndrome who had the characteristic clinical and EEG features.
Angelman Syndrome* ; Chromosomes, Human, Pair 15 ; Diagnosis ; Electroencephalography ; Gait ; Humans ; Infant ; Language Development Disorders ; Laughter ; Seizures

Hemimegalencephaly is a rare brain malformation characterized by congenital hypertrophy of one cerebral hemisphere, ipsilateral ventriculomegaly, hemiparesis, intractable epilepsy, and mental retardation, which often results in early death. We reported a case of hemimegalencephaly in a 1-month-old male with the chief complaint of intractable focal seizure.
Brain ; Cerebrum ; Epilepsy ; Humans ; Hypertrophy ; Infant, Newborn ; Intellectual Disability ; Male ; Malformations of Cortical Development* ; Paresis ; Seizures*

Band heterotopia is a rare neuronal migration disorder, resulting in epilepsy and mental retardation. Epilepsy in band heteropopia, of which Lennox-Gastaut syndrome constituted about 20%, varied in nature and degree of severity. Band heterotopia can be diagnosed by brain magnetic resonance imaging (MRI), showing another diffuse layer of gray matter underlying the normal-looking cortex with intervening thin rim of white matter. While positron emission tomography (PET) with [18F]-fluorodeoxyglucose revealed glucose uptake similar to the overlying cortex, single photon emission computerized tomography (SPECT) findings of band heterotopia have not been reported. We report a 8-year-old girl who presented with variable types of generalized seizures and mild mental retardation. She was diagnosed as having band heterotopia with Lennox-Gastaut syndrome by MRI and interictal electroencephalogram (EEG) showing immature background and generalized 2 Hz slow spike and wave complexes. Interictal SPECT, using Tc 99m hexamethyl propylenamine oxime (Tc 99m-HMPAO), revealed the same degree of perfusion in both the areas of band heterotopia and the overlying cortex. By using valproate and lamotrigine, she is now in stable condition with a significant decrease in seizure frequency.
Brain ; Child ; Electroencephalography ; Epilepsy ; Female ; Glucose ; Humans ; Intellectual Disability ; Magnetic Resonance Imaging ; Neuronal Migration Disorders ; Perfusion ; Positron-Emission Tomography ; Seizures ; Tomography, Emission-Computed, Single-Photon ; Valproic Acid

PURPOSE: We performed this study to evaluate the changes of total leukocytes and leukocyte types in CSF and peripheral blood (PB) in the early course of aseptic meningitis. METHODS: One hundred and eighty-nine children with aseptic meningitis, who were admitted to the Pediatric Department of Dong-A University Hospital during the period from June 1996 to October 1997 were included. Patients were divided into 4 groups by 12-hour intervals according to the time between the onset of illness and initial diagnostic lumbar puncture. We analyzed clinical pictures, total leukocytes and leukocyte types in CSF and peripheral blood (PB) in each group. RESULTS: 1) There was no significant difference in average total leukocyte counts in CSF between each group, and all groups were considered to be acute stage of illness. 2) The PMNL proportion of CSF leukocytes was 57.0+/-31.6% in group I, 44.1+/-32.3% in group II, 39.4+/-33.1% in group III and 26.9+/-27.9% in group IV. The PMNL percentage was significantly higher in group I than group III and IV and the proportion of patients with a predominance of PMNL was higher in group I than group III and IV (p<0.05) 3) The proportion of neutrophils in PB was highest in group I (76.5+/-15.7%) and significantly higher in group I and II than group III and IV (p<0.05). 4) Significant correlations were found between the proportion of PMNL in CSF and PB of patients (r=0.62, p<0.001) CONCLUSION: The change from a predominance of PMNL to a predominance of mononucler leukocytes was occurred 12-24 hours after onset, and there was a strong correlation between the proportion of neutrophils in CSF and PB.
Cerebrospinal Fluid* ; Child ; Humans ; Leukocyte Count ; Leukocytes* ; Meningitis, Aseptic* ; Neutrophils ; Spinal Puncture