Charactersitics and issues of guideline to withdrawal of a life-sustaining therapy

Agenerally accepted consensus of end-of-life (EOL) care decision-making did not appear in Korean medical society until the year 2009. To enhance physician's ethical perception of EOL care, consensus guidelines to withdrawing life-sustaining therapies endorsed by Korean Medical Association, Korean Academy of Medical Science, and Korean Hospital Association, were published on October 13, 2009. In this article, the characteristics and issues with the guidelines are presented to improve understanding by physicians who interact with EOL patients. According to the guidelines, physicians should identify, document, respect, and act on hospitals inpatients' needs, priorities, and preferences for EOL care. The guidelines advocate that competent patients express their right of self-determination in EOL care decisions through advance directives. However, there are barriers to adopting advance directives as a legitimate tool of EOL decision-making in our current society. The guidelines stressed the importance of open communication between care-givers and patients or their surrogates. Through communication, physicians can create a plan regarding how to manage EOL until the patients' last day of life. Concerted actions among the general public, professionals, other stake-holders for EOL care, and governmental organizations to improve EOL care in our society are also stipulated. Physicians, who know the clinical meaning of the treatments available to EOL patients, should play a central role based on the consensus guidelines to help patients and their families make informed decisions about EOL care.

Wernicke's Encephalopathy Caused by Dysphagia in Down Syndrome: A case report

A subset of aged individuals with Down syndrome (DS) exhibits eating, drinking, and swallowing (EDS) difficulties, which have the potential to lead to life-threatening conditions such as malnutrition. We report a case of 37-year-old woman with DS, who developed Wernicke's encephalopathy related to malnutrition that was caused by EDS problems. The patient presented with a history of EDS problems for 2 years without treatment and had developed mental-status changes, unsteadiness of stance and gait with associated MRI findings, corresponding to Wernicke's encephalopathy. The patient was treated with nutritional support including thiamine replacement and dysphagia rehabilitation therapy. Our case highlights the importance of early and proper management of malnutrition associated with EDS problems in adult DS. The suspicion of Wernicke's encephalopathy must be considered when adult DS manifests as prolonged malnutrition, altered mentality and decreased mobility.

Hip Subluxation according to Neuromotor Type and Motor Quotient in Patients with Cerebral Palsy

OBJECTIVE: To find the incidence of hip subluxation according to corrected chronologic age (CCA), neuromotor type, severity and motor quotient in cerebral palsy (CP). METHOD: We reviewed clinical records and radiological findings of CP visited our hospital between June 1999 and June 2003. One hundred ninety-six children with CP (mean CCA: 19.0+/-13.9 months) were evaluated by measurement of the migration index, acetabular index, central edge angle and neck shaft angle from pelvic X-ray. The hip subluxation was defined as above 33% on the migration index. RESULTS: Among the 311 hips, 35 hips (11.3%) were subluxated and none was dislocated. The incidence of hip subluxation in CP was not significantly different in neuromotor type, severity, type by involvement of extremity and motor quotient, but statistically different in 25~36 month old group in comparison with the other age groups (p<0.05). CONCLUSION: The hip subluxation in CP may occur irrespective of neuromotor type, severity, type by involvement of extremities and motor quotient. Therefore, it is recommended to perform routine screening and follow-up examination of pelvic x-ray in CP to find out hip subluxation.

Magnetic Resonance Imaging Findings Based on Clinical Subtypes of Cerebral Palsy

OBJECTIVE: To consider the relation between MRI findings and clinical subtypes of cerebral palsy (CP). METHOD: The subjects comprised 83 patients with CP. We analyzed the brain MRI findings such as periventricular leukomalacia (PVL), brain atrophy, infarction or hemorrhage, basal ganglia lesion, migration anomaly and delayed myelination with consideration of clinical subtypes of CP. RESULTS: Of the 83 MRI findings, 69 abnormalities (83.1%) were the followings; PVL in 47 cases{17 spastic diplegics (SD), 17 spastic quadriplegics (SQ), 5 spastic hemiplegics (SH), 4 atonic or hypotonic quadriplegics, 2 ataxic quadriplegics and2 mixed quadriplegics (MQ)}, brain atrophy in 6 cases (3 SQ, 1 SD, 1 SH and 1 MQ), infarction or hemorrhage in 7 cases (5 SH and 2 SQ), migration anomaly in 2 cases (1 SQ and 1 SH), delayed myelination in 3 cases (2 SQ and 1 SH) and basal ganglia lesion in 4 cases (3 MQ and 1 atonic quadriplegic). 33 cases of 47 PVL and 2 cases of 6 brain atrophy were preterm CPs. There was no difference in severity of CP between preterm and fullterm CPs. CONCLUSION: The results of this study would be helpful in estimating the brain lesions in various clinical subtypes of CP.

Tc-99m-ECD Brain Single Photon Emission Computed Tomography Findings in Cerebral Palsy: Comparison with Magnetic Resonance Imaging Findings

OBJECTIVE: To investigate the correlation between magnetic resonance imaging (MRI) findings and single photon emission computed tomograpy (SPECT) in cerebral palsy (CP). METHOD: Fourty-one patients with CP underwent MRI and SPECT of the brain. The patients were divided into 5 groups. Group 1 was for the cases with normal findings on MRI and SPECT, group 2 for abnormal on MRI but normal on SPECT, group 3 for normal on MRI but abnormal on SPECT, group 4 for abnormal findings on both MRI and SPECT with same abnormal lesion and group 5 for abnormal findings on both MRI and SPECT but with different abnormal lesion. RESULTS: In group 2, periventricular leukomalacia (PVL) and cortical atrophy were shown on MRI. In group 3, decreased blood flow at cerebellum was shown on SPECT. In group 4, brain atrophy on MRI and the decreased blood flow at the same site on SPECT were shown. In group 5, 15 of 22 cases with PVL on MRI and decreased blood flow at cerebellum, thalamus, basal ganglia and the cortical areas were shown. CONCLUSION: Brain SPECT was more sensitive in the detection of cerebellum, thalamus and cortical blood flow abnormality. MRI was more sensitive in demonstration of white matter lesion.

Paraplegia Due to Spinal Hematoma in a Patient with Acute Lymphocytic Leukemia: A case report

Intrathecal administration of methotrexate is one of the standard therapies in the acute lymphocytic leukemia (ALL). Spinal puncture and tapping for intrathecal administration of methotrexate is considered as a routine procedure but this procedure carries risks of spinal hematoma in ALL patients. Spinal hematoma after spinal puncture is an uncommon condition, but it can occur more often in patients with thrombocytopenic or coagulation disorder. We report 4 year-4 month-old boy of ALL with spinal hematoma leading to paraplegia following lumbar puncture for intrathecal methotrexate treatment.

Pelizaeus-Merzbacher Disease: A case report

Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive disorder characterized by dysmyelination of the central nervous system (CNS) caused by mutations in the proteolipid protein (PLP) gene. PLP is located at Xq22 and its mutation result in abnormal expression or production of PLP, the most abundant protein in CNS myelin. We present a case of PMD in the 7-year-old boy with nystagmus, ataxia, spastic quadriplegia and severe psychomotor delay. His brain MRI revealed totally dysmyelinated white matter involving entire supratentorial region, atrophic change, and overaccumulation of the iron in both basal ganglia. He also showed soft-tissue contractures of the hip adductors, associated hip dislocations and equinovarus foot deformities due to severe spasticity of lower extremities. Orthopaedic surgery was performed on both hips. Antispastic medication and physical therapy were maintained for reduction of spasticity. We report this case with the review of literatures.

The Estimation of the Probable Cause and the Time of Cerebral Insult in Cerebral Palsy

OBJECTIVE: To estimate the probable cause and the time of cerebral insult in cerebral palsy (CP) based on MRI findings and risk factors. METHOD: The subjects comprised all sixty-seven patients with CP showing abnormal MRI findings between March 1999 and September 2001 at the Catholic University of Korea, St. Mary's Hospital. A detailed medical history was available for all patients including those not born in our hospital. They ranged in age from two months to five years. We analyzed the brain magnetic resonance (MR) findings of patients with CP to correlate the probable cause and the time of cerebral insult through the consideration of medical histories including prenatal, perinatal and postnatal histories. RESULTS: Of the 67 MRIs, abnormalities were the followings; periventricular leukomalacias (PVLs) in 49 cases, cortical or subcortical infarction in 4 cases, brain atrophy in 7 cases, neuronal migration disorder in 4 cases, and delayed myelination in 3 cases. Among the patients with PVL, perinatal risk factors were responsible for cerebral insult in preterm, but pre- and perinatal contribution were similar in patients born at full term. Among the patients with cerebral infarction, only one case with meningitis at 11 months was suspected for cerebral insult. These patients had no risk factor as a peri- or post-natal etiology. Four patients with neuronal migration disorder had no risk factor for peri- or postnatal etiology except for the one who was a twin. CONCLUSION: Review of brain MRI findings such as PVL, infarct, neuronal migration disorder and a detailed medical history including prenatal and perinatal etiology would be a useful method to estimate the probable cause and the time of cerebral insult in CP.

Mixed Quadriplegia with Lissencephaly and Dysmyelination

Lissencephaly results from a neuromigrational arrest during first and second trimester of pregnancy and shows hypotonia, marked mental retardation and seizure as predominant features. Myelination is a perinatal process and co-occurence of migrational disorder with myelination disorder is rare. We report a 17-month-old male with mixed quadriplegia and mental retardation with type 1 lissencephaly and dysmyelination of cerebral white matter diagnosed by magnetic resonance imaging.

Aicardi Syndrome: A case report

Aicardi syndrome is defined by the clinical triad infantile spasms, agenesis of the corpus callosum, and pathognomonic chorioretinal lacunae. Infantile spasm begins at early infancy and tends to be controlled poorly. The prognosis is poor in the patient with severe developmental delay and intractable seizures being common. We present a case of Aicardi syndrome in the 9-month-old female infant with infantile spasm, spastic tetraplegia and microcephaly. Her brain MRI revealed corpus callosum agenesis, atrophy of left hemisphere and periventricular heterotopia. She showed bilateral choroidal and optic disc coloboma. We report this case with the review of literatures.

Clinical Features and Electrodiagnostic Findings of Ulnar Neuropathy at the Elbow

OBJECTIVE: To evaluate the clinical and electrodiagnostic findings of ulnar neuropathy at the elbow. METHOD: Sixty-two patients with ulnar neuropathy at the elbow were reviewed retrospectively to establish causes, severity and type of neuropathy, symptom, sign, operation name and operative findings. RESULTS: 1) Of total 62 cases, 41 were male and 21 were female and the most often were in their forties and fifties. 2) The main cause of the neuropathy is bone deformity caused by previous fracture or dislocation (43.6%). 3) The symptoms observed were motor weakness (66.1%), sensory change (79%) and muscle atrophy (35.5%). 4) Forty-nine cases showed abnormality in nerve conduction study and needle electromyography study, and 9 cases showed abnormality only in the needle electromyography study. 5) On needle electromyography, sparing of flexor carpi ulnaris was shown in 50 cases (80.6%). 6) Operative treatment was performed in 15 cases. Among them, electrodiagnostic and operative diagnosis coincided in only 12 cases (80%). CONCLUSION: We conclude that above clinical and electrodiagnostic findings are useful for the diagonosis ulnar neuropathy at the elbow with consideration of etiology, localization and for the selection of operative treatment.

Clinical Significance of the R3 Response of the Blink Reflex in Medullar and Cervical Spinal Cord Lesions

OBJECTIVE: To compare the R3 response of the blink reflex in medullar and spinal cord lesion and to investigate whether the reflex arc of the R3 response descend to the cervical spinal cord or not. METHOD: We have studied 3 patients with medullar lesion and 5 patients with cervical spinal cord or vertebral lesion. Normal ranges of the R3 response refer to the results suggested by Moon et al. RESULTS: In 3 patients with medullar lesion, two patients with lateral medullar lesion showed delayed R3 latency or no evoked potential. Four patients with cervial spinal cord lesion showed no R3 response. In one patient with disc protrusion R3 was normal. CONCLUSION: Our results support the hypothesis that the reflex arc of the R3 response descend to the cervical spinal cord.

Normal Values of R3 Response of Blink Reflex

OBJECTIVE: To determine the normal data of R3 component of blink reflex in normal adults. METHOD: Subjects included 17 healthy males and 16 healthy females. Five trials of blink reflex were recorded from each side. The shortest latency of R3 response was the shortest among the 5 consecutive trials was selected. RESULTS: Mean onset latency of R3 was 79.8 ms, mean duration 31.0 ms, mean amplitude 399.5 V and a side-to-side difference of latency was 2.3 ms. The amplitude of R3 decreased with age. The latencies and durations of R1 and R2 were not related to those of R3. The amplitude of R2 was correlated with that of R3. CONCLUSION: This data will be useful for the localization of brainstem and cervical spinal cord lesions.

The Time-course of Neurologic Recovery in Traumatic Spinal Cord Injury

Possible mechanisms of neurologic recovery in spinal cord injury were postulated by Ditunno Jr. JF in 1987. The first window encompasses recovery from neurapraxia within 6 to 8 weeks. The second window covers the period from 2 to 8 months after the injury. Recovery during this period might be due to peripheral sprouting of intact nerves to denervated muscle and hypertrophy of functioning muscles. The third window of recovery happens usually beyond 8 to 12 months when axonal regeneration may play a role in further increases in strength. On the basis of these possible mechanisms, we measured the neurological and functional recovery rate according to the periods of these possible mechanisms of motor recovery through 12 months following injury in 21 traumatic spinal cord injury patients. The results were as follows: 1) Neurologically, the most rapid recovery was shown within 6 to 8 weeks after injury, during the phase of recovery from neurapraxia. 2) Most of functional recovery occured in the period between 2 month and 8 month of the compensatory phase. 3) Statistically significant correlation between motor and functional recovery was shown among the incomplete spinal cord injury group. These data would be helpful in planning a timely appropriate rehabilitation program by understanding the time-course of neurologic recovery and prognostication of neurologic and functional recovery in the spinal-cord injured.

Glycogen Storage Disease Type II: A Case Report

Glycogen Storage Disease Type II is caused by the deficiency of acid maltase resulting in lysosomal accumulation of glycogen. There are two major clinical syndromes, a severe generalized and invariable fatal disease of infancy, and a myopathy starting in juvenile or adult life. The clinical and laboratory findings of a patient with Glycogen Storage Disease Type II are presented. The patient, a 17-year-old male, experienced slowly progressive weakness of muscle of the pelvis shoulder girdles and trunk. Muscle biopsy showed vacuolar myopathy and electromyograph showed features of myopathy with fibrillation potentials, positive sharp waves, myotonic discharges, without clinical myotonia at rest, and polyphasic potentials on volition. Clinical features, histopathologic and electrophysiologic findings of this disease and differential diagnosis were reviewed.

Brachial Plexopathy Associated with Thoracoscopic Sympathectomy for Treatment of Primary Hyperhidrosis: A Case Report

Brachial plexus is vulnerable to injury because it is close to shoulder joint of which the range of motion is very large and it is surrounded by many complex structures. Iatrogenic injuries are commonly associated with surgeries such as procedures in the posterior neck or in the shoulder and open thoracotomy, during regional anesthetic blocks, and during other procedures that use needles and cannulas. Radiation therapy can cause brachial plexus injury. A case of brachial plexus injury after thoracoscopic sympathectomy for treatment of primary hyperhidrosis is discussed in this report. Twenty eight years old male patient developed motor and sensory paralysis of Rt. upper extremity shortly after thoracoscopic sympathectomy performed with hyperabduction position of Rt. upper extremity for 2 hours and 30 minutes. Electrodiagno stic study was performed on the 11th day after paralysis. Electrodiagnostic findings from the which was compatible with showed abnormal spontaneous activities at rest and decreased numbers of MUAPs on volition sampled muscles innervated by brachial plexus. The motor power of these muscles was trace or fair grade. On the 14th day after paralysis the patient recoverd the motor power to near nomal on muscle testing. The reason of this complication is not clear, but it might have occurred most likely in association with tension and traction forces applied to patient's brachial plexus while hyperabducting the arm for a long period of time during surgery. In this study we have reviewed perioperative brachial plexus injuries and their possible mechanisms, of injury hoping to decrease the occurrence of perioperative brachial plexus injuries.

A Case of Trigeminal Neuropathy Associated with Mixed Connective Tissue Disease / 대한류마티스학회지

The occurrence of a trigeminal neuropathy associated with connective tissue disease is rare. Trigeminal neuropathy in connective tissue disease is predominantly sensory and it is characterized by numbness, hypesthesia, slurred speech, and touch pain. Although the pathogenesis of trigeminal neuropathy associated with connective tissue disease remains obscure, the main pathologic findings are vasculitis and neuritis. A case of trigeminal neuropathy associated with mixed connective tissue disease is described, and the incidence, symptoms, pathophysiology, treatment of the disease is discussed.

Brain MRI findings in children with developmental motor delay

No abstract available.

Twin embolization syndrome as a cause of cerebral palsy: case report

No abstract available.

A case of polyneuropathy associated with folic acid deficiency

No abstract available.