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The purpose of this project is to investigate the clinical and brain MR characteristics of Epstein-Barr virus (EBV) encephalitis and encephalomyelitis. Clinical and 30 MR findings of 29 patients with EBV encephalitis or encephalomyelitis were retrospectively reviewed. Patients included 24 with encephalitis, 3 with encephalomyelitis, and 2 with brain-stem encephalitis. Altered consciousness, seizures, visual hallucination, and acute psychotic reaction were the common presentations. Eight patients had positive MR findings. These included T2 prolongation over gray and white matter, periventricular leukomalacia, and brain atrophy. Transient T2 prolongation over gray and white matter was found in one patient. Our results indicate that EBV encephalitis and encephalomyelitis have a wide range of both clinical and MR findings. The MR lesions may disappear in a short period, so the timing for the MR scan may be critical.

Lumbopritoneal shunt (L-P shunt) has been widely accepted as a treatment for communicating hydrocephalus. The technique offers many advantages such as easy insertion, a low infection rate, a reduced incidence of brain damage, et centera. Despite these advantages, there are also some associated complications, such as tonsillar herniation. This complication rarely occurs, but, when it does, it develops gradually. This report concerns an eight-year-old girl, who had received L-P shunt at the age of 20 days; she then developed tonsillar herniation, requiring operative decompression, eight years later. This case illustrates the necessity for carefully follow up patients who have undergone the L-P shunt procedure.

Immunocompromised children are potentially threatened by infections, among which, the highly contagious chickenpox infection is the most common. In the past six months, there has been a spate of five chickenpox infections in children with malignancy, all of whom were receiving chemotherapy at that time.The cases of 17 children with malignancies, who suffered from varicella-zoster infection during a period of chemotherapy at Taichung Veterans General Hospital were reviewed.The diagnoses of their neoplasms were 12 acute lymphoblastic leukemia (ALL), 2 lymphoma, 3 solid tumors. The mean age was 6.8 +/- 4.0 year-old (range 3 to 15 year-old). The average duration from chickenpox skin eruption to admission was 3.3 +/- 1.8 days. Four patients suffered from abdominal pain and three of them died soon; three of them suffered from back pain and one died later. Seven of these 11 patients had impaired liver function (GOT45 U/L), of whom 4 died later. There were seven patients with pneumonitis, of whom five died later. Among 12 patients with ALL, 3 had absolute lymphocyte counts (ALC)500/mm3, but only 1 died later; 9 had ALC500/mm3, of whom 4 had pneumonitis, and all died later. Four patients developed disseminated intravascular coagulopathy, and three of them died later. Seven patients were prescribed acyclovir within three days after first skin eruption, none of these died. Ten patients were prescribed acyclovir three days or more after first skin eruption and five of them died later. Five patients were prescribed intravenous immunoglobulin (IVIG) within three days after first skin eruption, and none of them died; of the seven patients prescribed IVIG three days or more after first skin eruption, three died later.Abdominal pain and disseminated intravascular coagulopathy (DIC) were signs of visceral dissemination. Severe liver function impairment, pneumonitis and DIC were the principal causes of death. Early administration of acyclovir and intravenous immunoglobulin (IVIG) can probably effectively prevent the dissemination of varicella-zoster virus (VZV). While varicella-zoster immunoglobulin (VZIG) was unavailable, IVIG was still valuable in treating VZV infection.

Hallervorden-Spatz syndrome is generally considered to be an autosomal recessively hereditary disorder of unknown etiology. Some reported cases have been known to be sporadic. We present a boy who suffered from regressive developmental milestones since he was 2 years and 6 months old. He began to manifest tremors of the upper extremities, followed by unsteady gait, choreoathetosis, dystonia, dysarthria, and dysphagia at 4 years old, and subsequently became completely bedridden at 6 years old. Neurologically, opisthotonus, rigidity of extremities, dystonia, hyperreflexia, profound emaciation, and bilaterally positive Babinski signs were present. The brain magnetic resonance imaging (MRI) done at the age of 8 years revealed symmetrical low signal intensity over the bilateral globus pallidi in the T2-weighted images coexistent with an area of high signal intensity over the anteromedial portion, known as "eye of the tiger" sign. Another MRI, followed up two years later, did not show marked difference in signal abnormalities over the globus pallidi in the T2-weighted images as compared with that of the previous one. However, progressive neurological deterioration existed.

Guillain-Barre syndrome (GBS) occurs at all ages, and population-based studies give it a crude average annual incidence rate ranging from 0.4 to 1.7 per 100,000 population. This retrospective study is performed for fifteen Chinese infants and children in light of the fact that GBS infrequently occurs during childhood years.Hospital records of fifteen Chinese infants and children, i.e. eleven males and four females, with GBS were included. Their ages ranged from one to sixteen years old, with a mean average of 8.5 years. Their performances were graded on a scale from 0 to 6 according to the GBS Study Group. Their performances were graded under categories of Grades 2 (2/15), 3 (2/15), 4 (8/15) and 5 (3/15).Seven patients (46.7%) had preceding viral illness, occurring from seven to fourteen days prior to admission, with a mean average of twelve days. In addition to weakness and areflexia, musculoskeletal pain and subjective paresthesia were the most common presentations upon admission. The later the cerebrospinal fluid (CSF) was examined, the more cases had CSF features of increased protein level with no or mild pleocytosis. Eleven patients underwent serological examinations, and seven of their tests yielded positive for Epstein-Barr virus in five and Mycoplasma pneumoniae in two. Seven patients underwent electromyography and nerve conduction velocity measurement, all exhibiting the characteristic features of demyelination. Twelve patients had supportive treatment, and three of them required mechanical ventilation. Intravenous gamma globulin was administered in two patients, and oral prednisolone was administered in one patient. It took one to six months with a mean average of 3.4 months for patients' performance returning to Grade 0. All of the patients survived without residual disability.Some infectious pathogens could play a role in the pathogenesis of GBS. Furthermore, complete recovery may be the rule for most infants and children with GBS.

PURPOSE: To assess the role of regional cerebral blood flow measured by technetium-99m hexamethyl propyleneamine oxime (HMPAO) brain single-photon emission CT (SPECT) in viral encephalitis of children. METHODS: Eighteen children diagnosed as having viral encephalitis (12 Epstein-Barr virus, 4 herpes simplex virus, and 2 Japanese B virus) underwent Tc-99m HMPAO brain SPECT and brain MR and/or CT. RESULTS: During the acute episode, 4 (22%) of the 18 patients had localized abnormality on brain MR and/or CT. Tc-99m HMPAO brain SPECT in the acute phase showed that 17 (94%) of the 18 patients had increased regional cerebral blood flow and 1 (6%) of the 18 children had a normal brain SPECT. Follow-up brain SPECT was performed at least 15 days after the acute episode. In 17 patients with abnormal first brain SPECT, 12 (71%) had normal second brain SPECT and 5 (29%) had decreased regional cerebral blood flow. The group of patients with normal regional cerebral blood flow on the follow-up brain SPECT had a better outcome than the group of patients with decreased regional cerebral blood flow. CONCLUSIONS: (a) The Tc-99m HMPAO brain SPECT was abnormal more often than CT or MR in children with acute viral encephalitis and provided better location. (b) In acute episodes of encephalitis, most patients showed locally increased regional cerebral blood flow. (c) After acute episodes, the regional cerebral blood flow returned to normal in most cases. (d) A normal Tc-99m HMPAO brain SPECT in the subacute phase usually indicates a good clinical outcome (no neurologic defect) 1 year after the acute illness in children with viral encephalitis.

Recognition of the significance of juvenile myoclonic epilepsy (JME) in the English-language neurological literature is relatively new. There are many factors responsible for delay in diagnosis of JME, including lack of familiarity with the syndrome, failure to elicit a history of myoclonic jerking and absence or generalized tonic-clonic seizures predating myoclonic jerks in some patients.The medical and electroencephalographic (EEG) records of seven Chinese children with JME, four males and three females, were reviewed.The age of onset ranged from 10 to 14 years with mean 11.8 +/- 1.6 years. The precipitating factors were sleep deprivation (5/7), photostimulation (3/7), hyperventilation (2/7) and menstruation (1/3). Childhood and juvenile absence epilepsies predated JME in three and one patients, respectively. All patients had concomitant grand mal on awakening. Generalized 3-4 Hz polyspikes-wave complexes occurred in all patients, and two patients had additional 3-4 Hz spike-wave complexes. These activities were provoked with photic stimulation (3/7) and hyperventilation (2/7). All patients were treated with valproate for more than four years, and relapse occurred six months to one year after discontinuation of valproate.JME is a benign generalized epilepsy with a strong genetic basis. Valproate is the drug of choice up to now, but long-term (life-long) medication may be needed.

Childhood absence epilepsy (CAE) is a relatively rare form of idiopathic generalized epilepsy, occurring in previously normal children with a strong genetic predisposition. The annual incidence of CAE has been estimated at 6.3/100,000 to 8/100,000 children aged 0-15 years.Medical and electroencephalographic records of 50 Chinese children with CAE were reviewed.There were 22 males and 28 females. The age of onset ranged from 3 to 12 years with a mean of 6.9 +/- 2.4 years. Six children (12%) had positive familial epileptic history and 11 patients (22%) had a past history of febrile convulsion. Electroencephalographically, there were posterior delta rhythm (6/50), frontocentral (1/50), and centrotemporal (1/50) spikes in addition to the characteristic 3 Hz spike and wave complex. All patients received regular or irregular treatment with valproate. Sixteen patients (32%) evolved to generalized tonic-clonic seizure (GTCS) with a mean of 2.1 +/- 1.6 years of lag from stopping valproate. The patients with GTCS had significant late onset of age, a longer active period, and longer treatment duration than those without GTCS (p0.01). There were significantly more patients with irregular treatment evolving to GTCS than those with regular treatment (p0.01). None of the patients with posterior delta rhythm evolved to GTCS.Regular treatment with valproate is the modality of choice in the management of CAE. Posterior delta rhythm seems to be a good prognostic factor electroencephalographically.

From October 1982 to June 1992, 94 neurologically normal Chinese Children (50 males44 females) with benign partial epilepsy of childhood with centrotemporal spikes were studied among 1847 epileptic infants and children. Their ages at onset were between 3.6 and 12 years (a mean of 7.3 years). All of them had normal birth and developmental histories. Seven patients (7.5%) had past history of febrile convulsion and 12 cases (12.8%) had family history of epilepsy. The depicted seizure patterns were protean, but most attacks (83%) occurred during sleep (nocturnaldiurnal). The duration of active period and seizure frequency had no significant difference in terms of sex and treatment, but early-onset patients (7.3 years) had statistically significant longer duration of active period than that of late-onset group (p0.01). We recommend that antiepileptic drug treatment is not essential for this disease. Even if antiepileptic drug is used, the low-dosed carbamazepine is suggested.

Seventy-one Chinese children with idiopathic generalized epilepsy, from among 1931 pediatric epileptics, were reviewed clinically and electroencephalographically. They were classified under childhood absence epilepsy (CAE, 50/71), juvenile myoclonic epilepsy (JME, 7/71) and epilepsy with grand mal on awakening (GMA, 14/71). All the patients' family members with epilepsy were classified under idiopathic generalized epilepsy. Hyperventilation was the most common precipitating factor for CAE, and sleep deprivation was the most common one for JME and GMA. Electroencephalographically, fast generalized spike or polyspike-wave complex (or = 3Hz) was the most common feature. Three JME (42%) and 4 GMA patients (28%) had been diagnosed as CAE about 5 to 7 years preceding JME and GMA, respectively. Sixteen CAE cases (32%) later evolved to be GMA. All JME patients had concomitant GMA and three GMA cases (21%) had concomitant JME at diagnosis. There is strong evolutional correlation among CAE, JME and GMA in this series.

Progress in early surgery and intensive care have enhanced survival for more individuals with serious cyanotic congenital heart disease (CHD). Parents are understandably concerned about patients' brain function. Therefore, a study was undertaken of brain magnetic resonance images (MRI) in infants and children with cyanotic CHD, stressing the correlation between clinical neurological examination and brain MRI findings.Sixteen infants and children with cyanotic CHD, 6 males and 10 females, were selected randomly from June 1991 to June 1993. Their ages ranged from 1 to 10 years, with a mean of 4.4 years. All patients underwent brain MRI and thorough clinical neurological examinations.Prominent and tortuous vessels over the Willis circle and basal ganglia were the most common brain MRI findings among patients with normal neurological examination (7/9), while ventriculomegaly (5/7) was the common findings for patients with abnormal neurological examination. Two patients who presented with low-grade fever and Babinski sign were found incidentally to have brain abscesses.It is not possible to make a conclusion from this preliminary report about the role which cyanotic CHD may play in the pathogenesis of brain insult, but the clinical neurological findings may provide guidance for the arrangement of brain MRI in infants and children with cyanotic CHD. Further larger scale, case-controlled study is needed to answer the controversial question whether cyanotic CHD per se causes brain insult.

Epilepsy with grand mal (GMA) on awakening is a benign generalized idiopathic epilepsy, occurring around the time of puberty. There have been few reports regarding Chinese children with GMA, thus this analysis of fourteen Chinese children with GMA who have been encountered in the past 10 years.The medical and electroencephalographic (EEC) records of 14 Chinese children with GMA, 7 males and 7 females, were investigated retrospectively.The age of onset ranged from 9 to 15 years with a mean of 12 +/- 1.6 years. Six patients had previous history of febrile convulsion; four and three patients had positive family history for epilepsy and febrile convulsion, respectively. All patients had generalized tonic-clonic seizures on awakening and three of them had concomitant myoclonic jerks. The precipitating factors were sleep withdrawal (9), photostimulation (4), hyperventilation (3), fevers (2), and menstruation (2). The first EEG findings included 3-4 Hz generalized spike-wave complex (8/14), photostimulation activated (4/14), and hyperventilation activated (3/14). Valproate had been regularly administered orally for a duration of 1 to 8 years with a mean of 3.8 +/- 2.0 years up to time of study. Six (75%) out of eight patients relapsed about 6 months to 1 years after discontinuation of anticonvulsant.GMA may have some clinical relationship with other idiopathic generalized epilepsies, such as childhood absence epilepsy and juvenile myoclonic epilepsy. High-percentage relapse after drug discontinuation may be the rule for GMA patients, so long-term use of anticonvulsants may be needed.

The estimated incidence of herpes simplex encephalitis (HSE) is 2 to 4 per million of the population per year, of whom one-third are neonates or older children and two-thirds, adults. As effective antiviral therapy improves the high mortality and morbidity for younger patients with HSE, early diagnosis has become important, especially in children.Hospital records, neuroimages and electroencephalographic (EEG) findings of 10 Chinese infants and children with HSE were reviewed thoroughly over a 7-year period. Diagnosis of HSE was made via clinical setting of acute encephalitis with serological examinations of serum and cerebrospinal fluid (CSF).Fever, consciousness disturbance and seizures were the most common clinical presentations on admission. Most of the CSF findings were mild pleocytosis (200/cumm), red cells, slightly elevated protein level and normal ratio of CSF/serum glucose concentration. All patients had abnormal features, by brain computerized tomography, and most had low densities over the temporal and frontal regions. Five patients underwent follow-up brain magnetic resonance imaging and most showed features of brain atrophy and demyelinating changes over the temporal and frontal regions. Generalized slow background activity was the most common EEG finding. Four patients' EEGs showed periodic laterizing epileptiform discharge. Intravenous administration of acyclovir (30 mg/kg/day) for 10 days was the drug-of-choice in these patients. All patients but the three deaths had psychomotor or mental retardation and spasticity. Six patients had epileptic sequelae, and one patient had the sequela of amnesia.HSE remains a serious illness with high mortality and morbidity despite appropriate antiviral therapy. Adequate intravenous acyclovir therapy should be started as soon as possible. CSF examinations, EEG, serology, brain computerized tomography and magnetic resonance imaging may provide some clues of HSE, but negative findings should not deter or delay treatment in suspicious cases.

Hospital records of 16 infants and children (9 males7 females) with tuberculous meningitis or tuberculoma were reviewed retrospectively over a 10-year period. Patients were aged from 5 months to 16 years, with a mean age of 7.2 years. The diagnoses were confirmed via a positive culture for Mycobacterium tuberculosis from cerebrospinal fluid (CSF) in 14 patients, and from brain tissue in 2. Most patients presented with fever (100%), conscious change (80%), vomiting (80%) and headaches (75%). Neurologically, meningeal and pyramidal signs were the most common findings. Lymphocytic CSF pleocytosis with hypoglycorrhachia, increased protein, and decreased CSF/serum glucose ratio were the major CSF findings in our patients. Hyponatremia was present in 70%. Brain computerized tomography showed hydrocephalus (87%), basal exudate (50%), ischemic infarction (37%) and tuberculoma (12%). Two patients (12%) expired and 10 (71%) of the surviving patients had neurological sequelae. In summary, the characteristic CSF findings and hydrocephalus (87%) seemed to be sensitive clues supporting early initiation of antituberculous drug therapy and thorough investigation as reflected in this series.

Reviewed 49 cases of Pseudomonas bacteremia which occurred in pediatric patients during an 8-year period showed the annual rate, per 1000 discharges, was 1.7. In most of the patients (73.5%), the disease was hospital-acquired. Male to female distribution was about 3 to 2. The average age of the 49 patients was 1.29 years, and 55.1% were infected at less than 2 months old. Overall mortality was 44.9%; among the mortalities, 63.6% were under two months of age. Clinical features were not characteristic, but the most common sign associated with this infection was fever (42.9%). Ecthyma gangrenosum occurred in only one patient. Respiratory tract and skin were the most frequent sources of the bacteremia. Polymicrobial bacteremia occurred in 18.4%. Patients with shock, pneumonia, inadequate antibiotic therapy or persistent neutropenia had a substantially poorer prognosis. Administration of combination therapy to patients with Pseudomonas bacteremia seemed to be superior to monotherapy for positive outcome.

Between 1983 and 1992, 11 children and 38 adults were seen at Taichung Veterans General Hospital with a definite diagnosis of dermatomyositis. Their clinical pictures, laboratory findings, courses and outcomes were compared. The mean age at diagnosis was 12 years and 50 years, respectively. Children had a higher female-to-male ratio (2.7:1 vs. 1.2:1) and a more acute onset, while adults had a higher incidence of malignancy and other connective tissue disease associations. Clinically, shawl sign was more common in the adults, whereas myalgia was more frequently seen in the children (0.05p0.1). Hemograms, serological parameters and immunological investigations showed no significant differences between the two groups. Elevated erythrocyte sedimentation rate and circulating immune complexes were found in over half of both groups of patients; however, their values bore no relationship to either disease activity or future outcome. Although no significant differences were demonstrated between the two groups of patients with regard to either clinical manifestations or laboratory findings, the disease entity seemed not to be the same because evidence of vasculopathy in muscle pathology was more prominent in the children, with endothelial swelling and necrosis the most common findings. Steroids had been used as the first choice of therapy in both groups of patients, followed by cytotoxic drugs and immunosuppressants. Children had a more favorable outcome compared to a 24% mortality rate in the adult group. Experience here recommends a complete cancer work-up in adults, especially for nasopharyngeal carcinoma among the Chinese. Various autoantibodies screenings may also be helpful for early detection of other associated connective tissue diseases.

Nine Chinese children (four males, five females) in whom serology showed Epstein-Barr virus infection were studied retrospectively. They were from 1.5 to 14 years of age. Various symptoms and signs, including disturbance of consciousness, visual hallucination, cranial neuropathies and sphincter dysfunction, led to initial clinical impressions of encephalitis, encephalomyelitis and neuro-degenerative disease. Electroencephalography showed focal spikes over the frontal, occipital, temporal and parietal areas. The patients underwent brain computed tomography and/or magnetic resonance imaging, and three of them underwent HMPAO SPECT. Eight patients appeared normal neurologically during follow-up, and the single patient who had spastic quadriparesis improved gradually. Though visual hallucination is not a specific presentation, it is a peculiar picture of Epstein-Barr virus encephalitis. Epstein-Barr virus encephalitis seemed to be self-limiting without specific treatment in this group.

A 6-year-9-month-old girl with the characteristic features of Rett syndrome is reported. Clinically, she had microcephaly, psychomotor arrest, deterioration of communication, autistic behaviour, loss of language development, gait apraxia and stereotyped hand washing movement. Amino acid and organic acid analysis were normal. An abnormal rise in serum lactate was noted 120 minutes after oral glucose loading. Muscle biopsy was performed and there was no specific finding noted under light microscope. Electron microscopic evaluation revealed mild accumulation of mitochondria at subsarcolemmal area with abnormal tubular cristae. The cause of Rett syndrome remains obscure. Several articles concerning abnormal mitochondrial morphology or respiratory enzymes have been reported. The exact pathogenesis requires further investigation.

Fourteen patients (10 boys, 4 girls) aged from 4 months to 14 years old were diagnosed with mitochondrial disease based on the clinical manifestations together with abnormal muscle mitochondrial morphologies. Their clinical diagnoses included Leigh syndrome, three; Menkes' syndrome, three; Kearns-Sayre syndrome, two; myoclonic epilepsy with ragged fibres, one; and infant-onset progressive myoclonic epilepsy, one; fatal infantile mitochondrial myopathy, one; fatty acid oxidation defect, two; and myopathy with cardiopathy, one. Organs involved other than muscles included central nervous system, ten; heart, six; eye, two; liver, two; and kidney, two. Clinical manifestations varied to include hypotonia, seizures, myoclonus, mental retardation, nystagmus, ataxia, ptosis, ophthalmoplegia, retinal degeneration, muscle atrophy, spasticity etc. Nine had an abnormal rise in lactate after glucose loading. Ragged-red fibres were found in four patients. Abnormal mitochondrial morphology included abnormal accumulation, abnormal cristae pattern of tubular, concentric, or parallel form, some contained osmiophilic inclusion bodies. One patient of Leigh syndrome had had brain necropsy which showed intramyelin splitting of myelinated axons.

Over a 9-year period from 1983 to 1992, 50 patients with Henoch-Schönlein purpura were admitted to the Department of Pediatrics, Taichung Veterans General Hospital. There were 33 boys and 17 girls, with a male to female ratio of 2:1. Their ages ranged from 2 to 14 years with a mean of 6.3 years. Seventy percent of the patients were over the age of five. The months of peak incidence were October to December (48%). Fifty-four percent of the patients had evidence of preceding infections. The main clinical features were skin rashes (100%) followed by gastrointestinal symptoms (66%), joint symptoms (58%), soft tissue edema (30%) and renal involvement (30%). Hemograms showed leukocytosis, thrombocytosis, and elevated erythrocyte sedimentation rate in about half of these patients. Serological examinations were nonspecific; anti-streptolysin O titers were elevated in 40% of the patients. Forty-two percent of our patients had elevated serum IgA levels which bore no relationship to the symptomatology and duration of the illness. One patient had a complication of intussusception requiring surgical reduction. Another patient who had renal involvement progressed to end-stage renal disease. Eighty-two percent of our patients received prednisolone therapy at a dose of 1-2 mg/kg/day, with good response. Seventy-two percent of the patients recovered completely within one month after the onset of the malady. The overall prognosis remains good.