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Three Cases of Symptomatic Huge Arachnoid Cysts.

Ki Hyun JEON ; Hyo Geun JU ; Tae Hyung CHO ; Keun Mo KIM ; Young Jong WOO ; Jung Kil LEE ; Jae Hyu KIM

Journal of the Korean Child Neurology Society.1998;6(1):142-148.

Congenital arachnoid cysts are commonly located at sylvian cistern or middle cranial fossa which are usually asymptomatic and incidentally found. Posterior fossa cysts, however, are usually large when diagnosed, and symptomatic. Three cases of large posterior fossa cysts were recognized on the diagnostic MRI investigation for infantile spasm, developmental delay, and the precocious puberty. Surgical decompression of the cysts by craniectomy, cyst excision and fenestration were performed successfully in two patients with arachnoid cysts in the cerebellopontine cistern and the suprasellar, right cerebellopontine, and prepontine cisterns, but an additional cystoperitoneal shunt was needed in a case with the cyst in the quadrigemial cistern with obstructive hydrocephalus. Infantile spasm was treated with vigabatrin and pyridoxine, and the true precocious puberty was managed with LHRH analogue(Decapeptyl ).
Arachnoid Cysts* ; Arachnoid* ; Cranial Fossa, Middle ; Decompression, Surgical ; Gonadotropin-Releasing Hormone ; Humans ; Hydrocephalus ; Infant ; Infant, Newborn ; Magnetic Resonance Imaging ; Puberty, Precocious ; Pyridoxine ; Spasms, Infantile ; Vigabatrin

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A Case of Enterobacter sakazakii Epidural Abscess in Neonate.

Jung A SEO ; Se Wook OH ; Yong Won PARK ; Sang Woo KIM ; Ki Jae LEE

Journal of the Korean Child Neurology Society.1998;6(1):138-141.

Enterobacter sakazakii previously known as a 'yellow-pigmented Enterobacter cloacae', was designated as a unique species in 1980. This reclassification was based on differences from E. cloacae in DNA relatedness, pigment production and biochemical reactions. In 1961, Urmenyi and Franklin reported the first two known cases of neonatal meningitis and septicemia caused by E. sakazakii, although at that time it was still considered as an E. cloacae strain. Since 1961, a number of cases of neonatal infection caused by E. sakazakii have been reported worldwide. Usually newborn with E. sakazakii meningitis have a poor prognosis, many of them dying within days of infection. The reported case-fatality rate vary from 40-80%. We diagnosed and experienced a case of epidural abscess caused by E. sakazakii in a 1 month old male patient, who was asymptomatic with brain mass detected on brain ultrasound examination. We presented this case with a brief review of literatures.
Brain ; Cloaca ; Cronobacter sakazakii* ; DNA ; Enterobacter* ; Epidural Abscess* ; Humans ; Infant, Newborn* ; Male ; Meningitis ; Prognosis ; Sepsis ; Ultrasonography

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A Case of Spontaneous Discitis in Child.

Eun Jung LEE ; Joon Soo LEE ; Chang Jun COE

Journal of the Korean Child Neurology Society.1998;6(1):133-137.

The authors present a case of spontaneous discitis in a 1-year-old female who presented with fever and limping gait. The erythrocyte sediment rate was increased. The roentgenograms revealed normal appearnce, and radioactive bne scan showed increased uptake at the affected level. Discitis should be considered in any child with limping gait or leg pain, refusal to walk, or abdominal pain. Early recognition may avoid unnecessary diagnotic and treatment procedures.
Abdominal Pain ; Child* ; Discitis* ; Disulfiram ; Erythrocytes ; Female ; Fever ; Gait ; Humans ; Leg

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A Case of Moyamoya Syndrome Associated with Hereditary Spherocytosis.

Joon Sung KIM ; Won Sang YOON ; Geun Mo KIM ; In Seok KIM ; Young Jong WOO ; Je Hyuk LEE

Journal of the Korean Child Neurology Society.1998;6(1):125-132.

Moyamoya is a chronic cerebrovascular disease characterized by progressive stenosis or occlusion of the terminal parts of both intermal carotid arteries with telangiectatic vascular network of collateral circulation at the base of the brain and leptomeningeal arteries. The etiology and pathophysiology of this disease are still unknown. Although the idiopathic presentattion is the commonest, moyamoya disease has also been reported in several hereditary or acquired clinical conditions including neurofibromatosis, sickle cell anemia, tuberculous meningitis, atherosclerosis, and following radiation therapy to the head. The term moyamoya disease should be reserved for those cases in which the characteristic angiogrphic pattern is idiopathic; moyamoya syndrome is used when the underlying condition is known. We have experienced a case of coexistence of moyamoya syndrome and hereditary spherocytosis in a 6-year-8-month-old girl who presented with right-sided hemiparesis and pallor. A cerebral angiogram revealed occlusion of proximal portion of left middle cerebral artery and abnormal collateral network. The peripheral blood smear and osmotic fragility test disclosed hereditary spherocytosis. To our knowledge, the coexistence of moyamoya syndrome and hereditary spherocytosis has not been documented. We report here the case and the brief review of related literatures. Further studies are needed to clarify the intimate relationship between the two diseases.
Anemia, Sickle Cell ; Arteries ; Atherosclerosis ; Brain ; Carotid Arteries ; Collateral Circulation ; Constriction, Pathologic ; Female ; Head ; Humans ; Middle Cerebral Artery ; Moyamoya Disease* ; Neurofibromatoses ; Osmotic Fragility ; Pallor ; Paresis ; Tuberculosis, Meningeal

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A Case of Cockayne Syndrome.

Jae Yoon KIM ; Jae Hyun PARK ; Joon Soo LEE ; Chang Jun COE

Journal of the Korean Child Neurology Society.1998;6(1):118-124.

Cockayne syndrome is a rare autosomal recessive disorder that results in postnatal growth failure and progressive neurological dysfunction. Associated clinical features are gait disturbance, progressive pigmentary retinopathy and other ocular anomalies such as cataracts and optic disk atrophy, sensorineural hearing loss, dental caries and cutaneous photosensitivity. The disease is clinically heterogeneous with a wide range in the type and severity of symptoms. We experienced a case of Cockayne syndrome in 13 year-old male, who had delayed development, hypophasia, characteristic physical appearance, cutaneous photosensitivity, dental caries. We reported the case with review of literatures.
Adolescent ; Atrophy ; Cataract ; Cockayne Syndrome* ; Dental Caries ; Gait ; Hearing Loss, Sensorineural ; Humans ; Male ; Optic Disk ; Retinitis Pigmentosa

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A Case of Aase-Smith Syndrome.

Dong Jun KIM ; In Joon SEOL

Journal of the Korean Child Neurology Society.1998;6(1):113-117.

The Aase-Smith syndrome is characterized by Dandy-walker malformation, hand abnormalities, joint contractures of the hands, and cleft palate. It is known to be inherited as autosomal dominant trait and to have variable patterns of expression. We experienced a case of a 3 day old male neonate who had cleft palate, joint contractures, long fingers, and Dandy-Walker malformation with a agenesis of cerebellar vermis, partial agenesis of corpus callosum, cerebellar atrophy, lissencephaly, and microcephaly. We reported a case with a brief review of literatures.
Agenesis of Corpus Callosum ; Atrophy ; Cleft Palate ; Contracture ; Dandy-Walker Syndrome ; Fingers ; Hand ; Humans ; Infant, Newborn ; Joints ; Lissencephaly ; Male ; Microcephaly

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Neuroradiological Findings in a Case of Menke's Disease.

So Hee CHUNG ; Mun Hyang LEE ; Hye Kyung YOON ; Bo Kyung KIM ; Hee Jung CHUNG

Journal of the Korean Child Neurology Society.1998;6(1):106-112.

Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.
Angiography ; Atrophy ; Brain ; Central Nervous System ; Ceruloplasmin ; Copper ; Diagnosis ; Early Diagnosis ; Hair ; Hematoma, Subdural ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Menkes Kinky Hair Syndrome ; Metabolism ; Muscle Hypotonia ; Neurodegenerative Diseases ; Rupture ; Seizures ; Thrombosis

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Precipitating Factors of the Initial Seizure in Childhood Epilepsy.

Sun Young KIM ; Ik Jin SONG ; Sang Ook NAM

Journal of the Korean Child Neurology Society.1998;6(1):98-105.

BACKGROUND: Control of seizure inducing factors (SIFs) is essential in the management of epilepsy. We suggest that avoiding of SIFs may prevent first attak of seizure in patients with risk factors or family history of epilepsy. We observed SIFs, clinical features associated with SIFs on their first seizure attack in patients diagnosed as epilepsy to help the prevention or management of the childhood epilepsy. SUBJECTS AND METHODS: A retrospective study was undertaken the analysis of 339 epileptic children visited to the pediatric Department of Pusan National University hospital from November, 1992 to July, 1998. RESULTS: 1) The 168 of 339 patients (49.5%) are associated with SIFs on first seizure attack. 2) Mean Age on their first seizure attack in the group with SIFs is 4.08+/-3.63 year-old and without SIFs is 2.80+/-3.34 year-old.3) The factors were febrile state (32.7%), sleepy state (29.0%), acute illness (8.3%), emotional stress (5.8%), fatigue (5.3%), hypoxia (4.7%), during watching TV or playing game (3.5%), head trauma (3.5%), bathing (2.8%), infection of central nervous system (2.3%), sleep deprivation (1.1%), vaccination (0.5%), hyperventilation(0.5%). 4) The types of seizure in patients with SIFs were as following : generalized tonic, clonic, tonic-clonic seiuzre was 140(90.9%), partial seiuzre 14(9.1%). 5) The family history of seizure disorders were not associated with SIFs. And 14(8.3%) of patients with SIFs had developmental delay, 24 (14.0%) of patients without SIFs. 6. Mean duration of control seizure was 1.52+/-2.65 years in the group with SIFs, 0.84+/-1.30 years in the group without SIFs. CONCLUSION: Early detection and education of the possible SIFs in each epileptic patients is considered as an important therapeutic modality in managing the epileptic patients in childhood.
Anoxia ; Baths ; Busan ; Central Nervous System ; Child ; Craniocerebral Trauma ; Education ; Epilepsy* ; Fatigue ; Humans ; Precipitating Factors* ; Retrospective Studies ; Risk Factors ; Seizures* ; Sleep Deprivation ; Stress, Psychological ; Vaccination

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Recurrences of Febrile Seizures Related to the Pyrexia.

Byung Joon CHOI ; In Goo LEE ; Kyung Tai WHANG

Journal of the Korean Child Neurology Society.1998;6(1):91-97.

PURPOSE: Febrile convulsion is a common clinical problem affecting 2-4 percent of children. The recurrence rate of FC is a approximately 33-50%. To identify factors influencing the recurrences of febrile convulsions, we investigated the risk factors, especially the relationship between the degree of pyrexia at the initial febrile seizure and the likelihood of subsequent febrile fits. METHODS: During the 24 months, we reviewed the children who presented their first febrile convulsion. All were admitted to the hospital for investigation and treatment. The study group compromised 136 children. A febrile convulsion was defined as a generalized convulsion that occurred within 15 minutes in a child aged 6 months to 5 years who had no pre-existing evidence of neurological abnormalities. The children were divided into three groups according to the severity of the fever recorded on presentation to hospital. Group I had temperatures higher than 39.5degrees C, group II, 38.5-39.4degrees C, and group III lower than 38.4degrees C. RESULTS: 1) 17 (40.5%) children had recurrences with a positive family history in first-degree relatives, 12 (60.0%) of febrile convulsions, 3 (42.9%) of epilepsy and 2 (66.7%) of febrile convulsions and epilepsy.2) In group I, three (15.8%) infants aged 6-18 months and two (13.3%) aged 19-30 months had recurrences of febrile convulsions. In group II, 13 (36.1%) infants aged 6-18 months and 5 (23.8%) aged 19-30 months had recurrences of febrile convulsions. In group III, 10 (41.7%) infants aged 6-18 months and 6 (46.2%) aged 19-30 months had recurrences of febrile convulsions.3) The overall recurrence after the initial febrile convulsion was 22 (52.4%) children by 6 months, 34 (81.0%) children by 12 months, 39 (92.9%) children by 18 months, and 42(100%) by 24 months.4) Fifteen (42.9%) had the recurrences between 6 and 12 months of age at initial febrile convulsion, 21(27.6%) between 13 and 24 months, and 4 (18.2%) between 25 and 36 months. CONCLUSION: Early age at onset of first febrile convulsion, a history of febrile convulsions in first-degree relatives were associated with an increased risk of recurrent febrile convulsions. Especially children with the lower degree of pyrexia at the time of the initial convulsion were more susceptible to recurrent convulsions than those with higher levels of pyrexia.
Child ; Epilepsy ; Fever* ; Humans ; Infant ; Recurrence* ; Risk Factors ; Seizures ; Seizures, Febrile*

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Clinical Investigation of Benign Infantile Convulsion with Diarrhea.

Il Ran KIM ; Heung Dong KIM

Journal of the Korean Child Neurology Society.1998;6(1):83-92.

PURPOSE: "Benign Infantile Convulsion with Diarrhea" was first described by Morooka in 1982. Even without fever, dehydration, electrolyte imbalance and intracranial infection, seizures can occur several times in a day after the onset of diarrhea. Except for the seizures during the patient's acute illness, neurological outcome is excellent and no patient suffers epilepsy. Because seizures can occur repeatedly without accompanying fever, and with only mild diarrhea, these patients can be misdiagnosed with infantile epilepsy and treated with unnecessary anticonvulsants. METHODS: This study was carried out with 61 patients with benign infantile convulsion with diarrhea who visited at Inje University Sang-gye Paik Hospital, from September 1989 to June 1997. RESULTS: There were 61 patients with benign infantile convulsion with diarrhea, 32 boys and 29 girls. Sixty out of 61 patients had generalized tonic-clonic seizures, and one patient showed partial seizure with secondary generalization. Sixteen(45.7%) out of 35 patients showed positive rotazyme tests. Cerebrospinal fluid examination showed normal in 14 patients, EEG's were normal in 38(84.4%) out of 45 patients, brain CT's were negative in 24 out of 25 patients, and brain MRI's were negative in 4 patients. We could follow up 38 patients, and 3 patients had recurring seizures due to benign infantile convulsion with diarrhea. Psychomotor developments were normal in all the follow-up patients. CONCLUSION: Benign infantile convulsion with diarrhea is a benign seizure disorder in infancy without relation to later neurological disability or epilepsy.
Anticonvulsants ; Brain ; Cerebrospinal Fluid ; Dehydration ; Diarrhea* ; Epilepsy ; Female ; Fever ; Follow-Up Studies ; Generalization (Psychology) ; Humans ; Seizures*

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