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Biomedical subjects

Meng-Fai Kuo

Publications and source records attributed to Meng-Fai Kuo.

14 recordsLinked to original sources
PubMed2006

Miller-Dieker syndrome associated with tight filum terminale.

An 8-year-old female was diagnosed with Miller-Dieker syndrome with typical facial presentation. Brain magnetic resonance imaging disclosed lissencephaly, and chromosome study revealed 17p13.3 deletion. She developed infantile spasms at an early age, and her seizures were poorly controlled by multiple antiepileptics. Recurrent urinary tract infections were diagnosed during routine out-patient department follow-up. Urodynamic study disclosed a neurogenic bladder. Spinal magnetic resonance imaging revealed a tethered cord resulting from tight filum terminale, and untethering surgery was performed. Four months after the surgery, repeated urine cultures indicated that she was free from the urinary tract infection. Urodynamic study after untethering surgery demonstrated improved compliance of the urinary bladder.

Brain↗
PubMed2005

Diagnostic approach to recurrent bacterial meningitis in children.

Recurrence of bacterial meningitis in children is not only potentially life-threatening, but also involves or induces psychological trauma to the patients through repeated hospitalization and multiple invasive investigations if the underlying cause remains undetected. Bacteria migration, along congenital or acquired pathways from the skull or spinal dural defects, gains entrance into the central nervous system (CNS) and should be taken into consideration when children face recurrent bacterial meningitis, however, symptoms and signs of cerebrospinal fluid (CSF) rhinorrhea or otorrhea are rare in such patients. Without evidence of CSF leakage, a cranial symptom/sign or coccygeal cutaneous stigmata may suggest the approximate lesion site, diagnosis and detection remains difficult. To detect an occult dural lesion along the craniospinal axis, such as basal encephalocele, dermal sinus tract, or neurenteric cyst, a detailed clinical evaluation and the use of the modern diagnostic imaging methods is necessary. Because of the possibility of concomitant occurrence of more than one malformation, both the frontal and the lateral skull base should be carefully evaluated. Precise localization of the dural lesion is a prerequisite for successful surgical repair. In addition, the bacteria specificity could leave significant clues: Pneumoccocus or Hemophilus suggests cranial dural defects, E. coli or other gram negative bacilli suggests spinal dural defects, and meningococci suggest immunologic deficiency. Asplenia or immunodeficiency such as complement or immunoglobulin deficiency rarely causes recurrent meningitis without a history of frequent infection of non-CNS areas. Salmonella meningitis or brain abscess should not be treated incompletely or inadequately and could lead to recrudescence, relapse or recurrence of bacterial meningitis. Antibiotic (penicillin or trimethoprim-sulfamethoxazole) induced meningitis may repetitively occur on occasion.

Cerebrospinal Fluid Rhinorrhea↗
PubMed2004

Auditory agnosia caused by a tectal germinoma.

The authors describe a patient with auditory agnosia caused by a tectal germinoma. Despite having normal audiometric tests, the patient failed to recognize words and musical characters. On head MRI, the inferior colliculi were infiltrated by tumor. Neuropsychological tests revealed severe impairment in recognition of environmental sounds and words, defective musical perception, and stop consonant-vowel discrimination. Inferior colliculus may play a role in the analysis of sound properties.

Acoustic Stimulation↗
PubMed2004

Sinus pericranii: from gross and neuroimaging findings to different pathophysiological changes.

CASE REPORTS: We report two girls with a typical presentation of sinus pericranii. Both patients received total excision of the subgaleal masses with complete obliteration of the communications to the intracranial portion. However, different gross appearances, neuroimaging findings and intraoperative findings were found. It took more time and attention to control the bleeding due to the large communication between the extracranial and intracranial components in the second case. DISCUSSION: Sinus pericranii is composed of abnormal extracranial vascular tissues that communicate directly with intracranial dural sinuses or veins. A review of the literature showed that 13 out of 31 patients with sinus pericranii had a high-flow pattern. Detailed physical examination and imaging studies to detect dilated scalp veins and a large bony defect should remind surgeons of the presence of large venous communications. Meticulous treatment of the large communication during surgery can avoid massive bleeding.

Child↗
PubMed2004

Acute hydrocephalus upregulates monoamine oxidase mRNA in neonatal rat brain.

Metabolic derangement of the dopamine system in hydrocephalic brain has been observed, but the change of monoamine oxidase (MAO), the major enzyme to metabolize dopamine, is not known. The metabolic changes of dopamine and MAO mRNA in the striatum were examined in acute hydrocephalic rats whose ventricular size and intracranial pressure were controlled to a similar degree. The tissue levels of dopamine and its metabolites as well as MAO-A and MAO-B mRNA elevated significantly in hydrocephalus. Cerebrospinal fluid (CSF) diversion reversed these changes and induced an initial decline, followed by an elevation of these substances in extracellular fluid. In summary, the metabolism of dopamine system in the striatum was up-regulated in acute hydrocephalus and CSF diversion reversed this metabolic derangement.

3,4-Dihydroxyphenylacetic Acid↗
PubMed2004

Arachnoid cyst with GnRH-dependent sexual precocity and growth hormone deficiency.

The coexistence of gonadotropin-releasing hormone (GnRH)-dependent sexual precocity and growth hormone deficiency in patients with arachnoid cysts is rarely reported, and its pathogenesis is not well recognized. This report describes an 11-year-old female who had a huge intracranial arachnoid cyst with initial symptoms and signs of sexual precocity. Her brain magnetic resonance imaging revealed distorted hypothalamus with a thin and stretched pituitary stalk. After treatment with cysto-peritoneal shunting and gonadotropin-releasing hormone analogue, her puberty was arrested and subnormal growth rate was observed. Catch-up growth was detected after growth hormone therapy. Hence, coexistence of gonadotropin-releasing hormone-dependent sexual precocity and growth hormone deficiency in this patient was confirmed.

Arachnoid Cysts↗
PubMed2003

Tourette's syndrome in Taiwan: an epidemiological study of tic disorders in an elementary school at Taipei County.

We have done an epidemiological study in an elementary school with 2000 Taiwanese children aged from 6 to 12 years, and found 11 of them with Tourette's syndrome. The prevalence rate of Tourette's syndrome was around 0.56%. The ratio of male to female was 9 to 2. The comorbid rate of attention-deficit/hyperactivity disorder was 36%, self-injurious behaviors 27%, and obsessive-compulsive behaviors 18%. The familial rate of Tourette's syndrome was 27%. We also found that another 98 children had transient tic disorders. For 73% of patients with mild tics, understanding and acceptance from family, teachers, and friends are the most important things. When tics are so severe that medication is necessary, haloperidol is no longer the first or only choice. We tried clonidine, atypical neuroleptics such as risperidone or olanzapine, or pergolide. The first support group was established in 1999 for children with tics in Taiwan and transformed to Taiwan Tourette Family association in June 2001 to provide further service for Tourette's syndrome.

Antipsychotic Agents↗
PubMed2003

Sonographic lenticulostriate vasculopathy in infancy with tic and other neuropsychiatric disorders developed after 7 to 9 years of follow-up.

On gray-scale transfontanel sonography, the small arteries supplying the basal ganglia are indistinct from the brain parenchyma in normal infants. Bright linear 'branched candlestick' stripes in these regions, suggesting sonographic lenticulostriate vasculopathy, were reported in more than 200 infants in the English literature; including 34 our own patients. To identify its long-term outcome, a prospective study was accomplished on our 34 infants with sonographic lenticulostriate vasculopathy which included 13 cryptogenic cases and 21 with distinct etiologies. At the age of 7 to 9 years in the cryptogenic group, 7 in 13 patients developed tics, attention deficits, hyperactivity, and/or obsession/compulsion; while in the symptomatic group only 2 of 21 patients had tics. The rate of mortality (33% vs. 0%), developmental delay (24% vs. 8%), mental retardation (24% vs. 0%), and neurologic deficits (29% vs. 0%) were significantly higher in the symptomatic group than the cryptogenic group. Comparatively, the occurrence rate of attention deficit-hyperactivity disorder (10% vs. 54%), tics (10% vs. 38%), and obsessive-compulsive disorder (5% vs. 13%) were significantly lower in the symptomatic group than the cryptogenic group. The rates of these neuropsychiatric disorders were 10% in the symptomatic group and 54% in the cryptogenic group. We concluded that idiopathic sonographic lenticulostriate vasculopathy in infancy may predict development of neuropsychiatric disorders later in childhood.

Age of Onset↗
PubMed2003

Periodic lateralized epileptiform discharges of pediatric patients in Taiwan.

Periodic lateralized epileptiform discharges are special electroencephalographic abnormalities present in adults with stroke, brain tumor, intracranial hemorrhage, or other rare etiologies. Few reports focused on the etiologies in pediatric patients. We retrospectively reviewed 8002 of our pediatric electroencephalographic records for the past 12 years and listed all associated illness and their outcomes. Forty-four children with periodic lateralized epileptiform discharges were collected. We found that there was an obvious difference in etiologies of our pediatric patients from those reported in the literature. Nearly two thirds of our patients (28 children) were associated with central nervous system infections. The other etiologies included head injury, encephalopathy, epilepsy, and others. Herpes simplex virus was responsible for two thirds (12) of the 18 children with identified pathogens causing a central nervous system infection. Ten patients failed to have a defined pathogen. Periodic lateralized epileptiform discharges have a different clinical significance in pediatric patients than in adults. In Taiwan, central nervous system infection is the most common etiology of periodic lateralized epileptiform discharges in pediatric patients. Herpes simplex virus, although the most common pathogen, should not be considered to be the only cause of encephalitis in children with periodic lateralized epileptiform discharges.

Adolescent↗
PubMed2002

Pyridoxal phosphate-responsive epilepsy with resistance to pyridoxine.

We present a female infant with seizures responsive to pyridoxal phosphate but that are resistant to pyridoxine. The mechanism by which pyridoxal phosphate controls seizures in this patient is unknown. Her seizures are perhaps not solely caused by pyridoxal phosphate deficiency. It is suggested that in addition to glutamic acid decarboxylase abnormality, the path from the absorption, transportation, phosphorylation, and oxidation of pyridoxine to pyridoxal phosphate in this patient might be defective. It should be considered whether pyridoxal phosphate can be the drug of choice instead of pyridoxine in treating patients suspected of pyridoxine-dependent epilepsy to reduce failure rate and further delay in seizure control.

Administration, Oral↗
PubMed2002

Transcranial ultrasound diagnosis of intracranial lesions in children with headaches.

The diagnostic value of transcranial ultrasound for intracranial lesions in children with headaches was evaluated. From January 1995 to December 1998, 444 children with headaches for more than 2 weeks visited our Pediatric Neurologic and Neurosurgical Outpatient Clinics. A 2-MHz-sector sonographic transducer was used to perform transcranial ultrasound. Nineteen patients were lost to follow-up. Two patients, both 15 years of age, failed to have a successful examination. Thirteen of the enrolled 423 patients presented with headaches and other symptoms and positive neurologic signs and had positive magnetic resonance imaging findings of intracranial lesions correlating with their transcranial ultrasound findings. Twelve of the remaining 410 children complaining of isolated headache had abnormal transcranial ultrasound findings. Nine of the 12 patients were confirmed to have intracranial lesions by magnetic resonance imaging studies. Among the nine patients, five had brain tumors, two patients had hydrocephalus, one patient had intracerebral hematoma, and one patient had temporal arachnoid cyst. The other three patients were misinterpreted to have a suprasellar lesion by transcranial ultrasound. These three patients and the remaining 398 children were regularly followed for at least 12 months. All revealed no evidence of an intracranial lesion. The sensitivity and specificity of transcranial ultrasound to detect intracranial lesions for children with isolated headaches were 75% and 99.7%, respectively. The average saving from utilizing this technique was $222 U.S. dollars per person. We suggest that transcranial ultrasound study is a reliable, convenient, time-saving, and economic diagnostic tool in detecting intracranial lesions in children with headaches.

Adolescent↗
PubMed2002

Sincipital encephalocele mismanaged as a facial hemangioma.

A 3-month-old male infant was found to have a protruding mass with irregular contour at the root of his nose since birth. The mass protruded more when he cried. It was diagnosed and treated as a hemangioma by laser surgery in another hospital. However, the lesion recurred 1 week later. Reviewing the previous computed tomographic study of the brain, it revealed that the protruding mass communicated with the brain. Sincipital encephalocele was diagnosed, and craniotomy for excising the encephalocele and repair of the dura were undertaken successfully. We report this patient with sincipital encephalocele that was misdiagnosed and treated as a vascular malformation on the face. Fortunately, there was no complication. The authors stress the importance of accurate diagnosis of a lesion near the craniofacial junction to avoid unnecessary surgery and possible catastrophes.

Diagnosis, Differential↗
PubMed2002

Chronological changes on magnetic resonance images in a case of desmoplastic infantile ganglioglioma.

Desmoplastic infantile gangliogliomas (DIGs) are rare tumors during infancy. They often occur as huge cystic tumors in the frontal and parietal lobes, with their solid component being adjacent to the leptomeningeal membrane. This report presents a patient with DIG and intractable epilepsy. Due to hesitation by the patient's family in allowing surgery, this is the first time that the chronological changes in DIG have been observed on imaging studies. During the follow-up, the tumor changed from a pure solid tumor to a cystic one, which is a typical picture of DIG. Surgical pathology confirmed the diagnosis of DIG. However, for a patient with epilepsy, it is recommended that this condition should be treated immediately with surgery.

Brain Neoplasms↗