Epstein-Barr virus encephalitis and encephalomyelitis: MR findings.

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.

Neuroimage in infants and children with mitochondrial disorders.

Neuroimage studies of thirty-eight infants and children with mitochondrial disorders were reviewed: 24 ultrasound (US), 21 computed tomography (CT), and 27 magnetic resonance image (MRI) examinations were analyzed. Patients included seventeen with Leigh syndrome, two with Kearns-Sayre syndrome (KSS), one with myoclonus, epilepsy, and ragged red fibers (MERRF), one with Alpers disease, five with Menkes disease, two with fatty acid metabolic defect, two with Rett syndrome, and eight with unspecified mitochondrial disorders. KSS and MERRF tended to occur in older children, whereas Leigh syndrome, Menkes disease, and Alpers disease occurred in infants and young children. The deep cerebral nuclei and the cerebral white matter were commonly involved in Leigh syndrome and KSS. Subdural hematomas or effusions with profound cerebral atrophy was found in Alpers disease and Menkes disease. Tortuosities of basilar, Willis circle, and cerebral vessels were also noted in Menkes disease. MRI and CT examinations of Rett syndrome, fatty acid metabolic defect, and most of the unspecified mitochondrial disorders were normal. Our results indicate that neuroimage studies have characteristic findings for specific mitochondrial syndromes.

Magnetic resonance imaging of herpes simplex encephalitis.

Eight brain magnetic resonance imagings (MRIs) and one spinal MRI of 7 small infants and children with herpes simplex encephalitis (HSE) were retrospectively studied. Hypointense and hyperintense areas of gray and white matters on T1- and T2- weighted images, respectively, were commonly present, with temporal lobes being the most common lesion sites. Hemorrhagic lesions were found in 4 patients (57%). Early involvement of the white matter, as early as day 4, was a common MRI finding in these patients. One patient had relapsed encephalomyelitis, whose spinal MRI showed diffuse hyperintense T2 signals from the lumbar spinal cord to the conus medullaris. All patients but one survived with major neurological sequelae. Our results indicate that MRI is a sensitive diagnostic modality in cases of HSE, and early involvement of white matter is not an uncommon MRI finding of HSE. Spinal MRI may be helpful in the diagnosis of relapsed herpes encephalomyelitis.

Lumboperitoneal shunt complicated with chronic tonsillar herniation: a case report.

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.

Unilateral porencephalic cyst presenting as infantile spasms: a case report.

Multiple etiological factors have been associated with infantile spasms (IS), and are usually linked to diffuse or multifocal brain damage. It is generally believed that infants with symptomatic IS have a significantly higher incidence of mental retardation and epilepsy than those with cryptogenic IS. However, IS secondary to focal brain lesions in which surgical removal of the lesions has resulted in cessation of generalized spasms and subsequent normal development are rarely reported. A seven-month-old male infant who manifested head nodding and generalized flexor spasms one month prior to the admission is reported. Waking interictal electroencephalography revealed a pattern of burst-suppression variant of hypsarrhythmia. Brain magnetic resonance imaging disclosed a huge porencephalic cyst over the left temporo-parietal region. The infant underwent craniotomy with corpus callosotomy, causing marked improvement in seizures. He attained subsequent stable psychomotor development.

Varicella-zoster virus infection in children with malignancy.

BACKGROUND: 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. METHODS: 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. RESULTS: 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 (GOT > 45 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 ALC > 500/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. CONCLUSIONS: 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.

Lesch-Nyhan Syndrome: report on two brothers.

Lesch-Nyhan syndrome is a rare X-linked disease characterized by over-production of uric acid and a central nervous system (CNS) disorder consisting of mental retardation, spasticity, choreoathetosis, and a compulsive form of self-mutilation. A deficiency in hypoxanthine-guanine phosphoribosyl transferase (HPRT) provides the underlying metabolic basis for this disease. A 12 month-old male baby who had orange crystals over the diapers since he was 3 months old was brought to our hospital due to developmental delay. Mental retardation and athetosis were also noted. Chemical analysis revealed hyperuricemia (uric acid 8.6 mg/dl). Urine routine showed microscopic hematuria and uric acid crystals. The activity of HPRT in erythrocyte lysates of parents were both within normal limits, but that of the patient was very low (0.0547 nm/min/mg protein, < 0.05% of control). His younger brother was born 2 months after this disorder diagnosed in this patient. The younger brother was noted to have uric acid crystals over the diapers when he was 40 days old and hyperuricemia (10.6 mg/dl) showed up later. He was also a case of Lesch-Nyhan syndrome since the activity of HPRT in erythrocyte lysates was also low (0.0327 nmol/min/mg protein, < 0.05% of control). Further studies, including carrier detection and deoxyribonucleic acid (DNA) analysis, could be helpful for genetic counseling. This syndrome is rare among Chinese, and this may be due to underdiagnosis.

Langerhans cell histiocytosis: a 10-year review.

Hospital records of seventeen patients (11 males & 6 females) with Langerhans cell histiocytosis, confirmed by electron microscopic demonstration of Birbeck granules, were studied retrospectively from October 1982 to October 1992 at Taichung Veterans General Hospital. The ages at presentation ranged from 5 months to 17 years (a median of 6 years). The clinical features were protean and included fever, pain, bony lesions, lung lesions, abnormal dentition, diabetes insipidus, oral ulcer, otorrhea, dermatitis, anemia, thrombocytopenia, lymphadenopathy, and hepatosplenomegaly. Skull and femur were the most common sites of bony lesions. The main therapeutic modalities were excision, radiotherapy, and chemotherapy with vincristine and prednisolone. The young age at presentation, several involved organ systems, presence of organ dysfunction, and clinical diagnosis with Letterer-Siwe disease were poor prognostic factors. Although Langerhans cell histiocytosis is not a rare disease, the cause is still unknown. It needs further research to disclose the mystery.

Clinical and MRI study of the Hallervorden-Spatz syndrome: long-term follow-up of one case.

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.

Clinical study of epileptic children with history of febrile convulsion.

BACKGROUND: Clinically, some epileptic syndromes have been linked to febrile convulsions (FC), but the exact relationship between FC and later epilepsy remains a mystery. METHODS: Detailed histories of 81 Chinese children among 1950 pediatric epileptics with a history of FC were obtained retrospectively. The clinical courses and risk factors were analyzed. RESULTS: According to their epileptic patterns, the children were divided into a generalized group (Group G, 45/81), a partial group (Group P, 30/81) and those with severe myoclonic epilepsy in infancy (6/81). Patients of Group P had an earlier age of onset of FC with a larger number of risk factors than those of Group G (p < 0.01), and patients of Group G had a shorter interval from the last FC to later epilepsy than those of Group P (p < 0.01). More risk factors of FC were present in children of Group P; these included especially focal seizures, prolonged duration and retarded psychomotor development (p < 0.01). CONCLUSIONS: For group of epileptics in this study, genetic factors might predispose for the expression of FC and later epilepsy, and anticonvulsant prophylaxis for FC seemed to be unnecessary.

Dural arteriovenous malformation with symmetrical calcification of the basal ganglia: a case report.

Dural arteriovenous malformations associated with symmetrical calcification of the basal ganglia are rare in children. This report concerns a 22-month-old female infant who was admitted with the problem of acute onset of status epilepticus. Physical examination revealed a grade II/VI heart systolic murmur over the left sternal border, and engorged scalp veins. Neurologically, left side hemiparesis, brisk deep tendon reflexes, bilateral presence of Babinski sign and ankle clonus were present. Brain computed tomography without contrast medium showed cortical atrophy and symmetrical calcification of the basal ganglia. Brain magnetic resonance imaging showed a signal-voided tortuous structure over the right parietal region. Cerebral angiography disclosed a dural arteriovenous malformation, located over the right parietal region, which had two major feeding arteries from the branch of the anterior cerebral artery and the parietal branch of the middle cerebral artery, and had direct venous drainage into the superior sagittal sinus. The patient received craniotomy with ligation of feeding arteries. She became seizure-free, and her neurological deficits improved gradually.

Viral encephalitis in children: detection with technetium-99m HMPAO brain single-photon emission CT and its value in prediction of outcome.

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.

Guillain-Barre syndrome in infants and children.

BACKGROUND: 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. METHODS: 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). RESULTS: 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. CONCLUSIONS: 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.

Acute transverse myelitis in children: clinical analysis of seven cases.

BACKGROUND: The reported annual incidence of acute transverse myelitis (ATM) is 1 per 3.4 million. Because of its rarity, we performed this retrospective clinical investigation. METHODS: Hospital records of seven Chinese children with ATM were reviewed thoroughly. The diagnosis of ATM were made clinically with or without neuroimages. RESULTS: Five males and two females were included, of age from three to sixteen years with a mean of 8.6 years. The common presentations included weakness of lower extremities, acute flaccid paralysis, urinary distention, constipation, hyporeflexia, sensory impairment and paresthesia. Slightly elevated protein level in cerebrospinal fluid was noted. Mycoplasma pneumoniae, varicella/zoster virus and Epstein-Barr virus infections were confirmed serologically in one, one, and two patients, respectively. All patients received supportive treatment. The duration required to reach peak severity from onset of illness ranged from six to forty-eight hours. All but one patient recovered, and one to six months were required to achieve maximum improvement. CONCLUSIONS: Some infectious pathogens may play a role in the pathogenesis of ATM. In general, the prognosis of childhood ATM is good.

Tc-99m HMPAO brain SPECT findings in pediatric viral encephalitis.

Two children were diagnosed with viral encephalitis, due to Epstein-Barr virus infection in one case and to herpes simplex virus infection in the other. Tc-99m HMPAO brain SPECT was arranged to detect changes in regional cerebral blood flow (rCBF) secondary to viral encephalitis. During the acute episode, Tc-99m HMPAO brain SPECT showed that the two cases had increased rCBF. After the acute episode, follow-up brain SPECT was arranged 6 months later. The rCBF in one case was restored to normal on the second brain SPECT, and that in the other case was decreased. The child with normal rCBF in the follow-up brain SPECT had better learning ability and intelligence than the other child with decreased rCBF.

Juvenile myoclonic epilepsy.

BACKGROUND: 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. METHODS: The medical and electroencephalographic (EEG) records of seven Chinese children with JME, four males and three females, were reviewed. RESULTS: 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. CONCLUSIONS: 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.

BACKGROUND: 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. METHODS: Medical and electroencephalographic records of 50 Chinese children with CAE were reviewed. RESULTS: 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 (p < 0.01). There were significantly more patients with irregular treatment evolving to GTCS than those with regular treatment (p < 0.01). None of the patients with posterior delta rhythm evolved to GTCS. CONCLUSIONS: 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.

Leukemoid reaction in a child with appendiceal abscess: a case report.

A case of appendiceal abscess presenting leukemoid reaction is reported. A seven-month-old male infant was admitted because of fever and diarrhea. Anemic conjunctiva and mild abdominal distension were the only physical findings on arrival. Progressive leukocytosis with left shift maturation was noted during hospitalization. The maximal white blood cell count was 178,000/cumm, with a high leukocyte alkaline phosphatase score. On the fourth day of admission, a palpable right lower quadrant abdominal mass with local tenderness was found. Appendiceal abscess was diagnosed by both ultrasonography and computed tomography. The infant received triple antibiotic therapy consisting of ampicillin, gentamicin, and clindamycin for 10 days; the mass disappeared two weeks later. As appendiceal abscess is a rare occurrence in infants and its production of a leukemoid reaction is even rarer, the case described here may cause a diagnostic challenge, and deserves attention from clinicians.

Leigh syndrome with progressive ventriculomegaly.

A 6-month-old female infant was diagnosed with Leigh syndrome after an abnormal muscle specimen was obtained and after magnetic resonance imaging demonstrated characteristic cranial lesions. She presented with episodic hyperventilation, myoclonus, ophthalmoplegia, hypotonia, and elevation of lactate in the cerebrospinal fluid and blood. A series of cranial ultrasounds revealed progressive ventricular enlargement before the typical lesions were detected by magnetic resonance imaging. Myelin destruction is believed to play an important role in the early stage of Leigh syndrome. Ultrasonography may provide a convenient way to document changes in brain that provide early suspicion of Leigh syndrome.