We have had an opportunity to study a patient with acute sensory neuronopathy. The patient was a 32-yearold housewife; the rapidly spreaded tingling sensation along both arms and legs developed, rendering her severely ataxic. There was no history of antecedent illness, familial neurological disease, or exposure to toxins and special drugs. On examinations, there was no abnormality in her mental and cranial nerve function. There was no motor weakness. She showed the profound loss of kinesthetic sense which was acutely progressive and associated with severe sensory ataxia and pseudoathetosis. All tendon reflexes were absent. However, cutaneous senses were preserved. There was no significant abnormal laboratory finding except elevated CSF protein content. On electrophysiologic findings, the decrease in the amplitude of action potentials with only mild slowing of conduction velocities of sensory nerves were found even though motor nerve conduction studies were normal. Median and tibial somatosensory evoked potentials could be elicited, although the median N19 scalp response and tibial N45 waveforms were prolonged in latency. Plasmapheresis were provided; clinical features improved. However, the electrophy-siological abnormalites remained. Thus we wish to report an additional case of woman suffering from the acute sensory neuronopathy, complementing the cases described by Stemm, Schaumburg and Asbury.
Action Potentials ; Arm ; Ataxia ; Complement System Proteins ; Cranial Nerves ; Evoked Potentials, Somatosensory ; Female ; Humans ; Kinesthesis ; Leg ; Neural Conduction ; Plasmapheresis ; Reflex, Stretch ; Scalp ; Sensation

Accurate diagnosis and management of a femoral vascular injury is important as it is a life-threatening injury with high morbidity and mortality. This is the case of a 75-year-old man admitted to the emergency room with trauma to the right groin due to a horse fall. Computed tomography showed active bleeding of the femoral artery without pelvic or femoral fracture. We inserted a stent-graft, but hypotension persisted. Exploration of the groin was completed, and the bleeding from the external iliac vein was identified and repaired. In conclusion, vascular injury is rare in groin trauma without associated fracture, however, arterial and venous injury should not be completely ruled out. Endovascular therapy is worth recommending as a quicker and safer management than surgery in patients with active bleeding in the femoral artery. However, the possibility of combined injury of the femoral vein should be suspected in case of ongoing hemodynamic instability.

PURPOSE: Tumor necrosis factor-alpha(TNF-alpha) is a pro-inflammatory cytokine that has been implicated in the pathogenesis of cardiovascular disease. Serum levels of TNF-alpha are elevated in many human cardiac related pathogenic conditions, including heart failure. It is well known that TNF-alpha inhibits myocardial contractility and induces apoptosis of adult rat cardiomyocytes via stimulation of TNF receptor 1. But pathophysiologically relevant low levels of TNF-alpha can not induce apoptosis of neonatal cardiomyocytes. So, we evaluated the effects of different concentrations of TNF-alpha in cultured rat neonatal cardiomyocytes : apoptosis or necrosis. METHODS: Neonatal ventricular myocytes were isolated from 3-day-old rats by stepwise collagenase dissociation, and the cells were cultured for 3 days. After that, cardiomyocytes were treated with low(25 ng/mL) and high(250 ng/mL) concentration of TNF-alpha for 48 hours. Apoptosis was determined by terminal deoxynucleotidyl transfer-mediated end labelling(TUNEL) staining, and cell viability was evaluated by lactate dehydrogenase(LDH) measurements using cell culture supernatants. RESULTS: Low dose TNF-alpha did not induce apoptosis compared with controls(10.5 +/- 3.5% : 10.4 +/- 4.3%). And high dose TNF-alpha also did not induce significant apoptosis(10.2 +/- 3.6% : 10.4 +/- 4.3%). There was no detectable morphological changes of cardiomyocytes after low and high concentration of TNF-alpha treatment. LDH levels after TNF-alpha treatment was not significant compared with control(control : low : high, 3.2 +/- 0.1% : 3.1 +/- 0.2% : 3.3 +/- 0.3%). CONCLUSIONS: Our results suggest that high concentration of TNF-alpha alone can not induce apoptosis and significant cytotoxicity in neonatal rat cardiomyocytes.
Adult ; Animals ; Apoptosis ; Cardiovascular Diseases ; Cell Culture Techniques ; Cell Survival ; Collagenases ; Heart Failure ; Humans ; Lactic Acid ; Muscle Cells ; Myocytes, Cardiac* ; Necrosis ; Rats* ; Receptors, Tumor Necrosis Factor ; Tumor Necrosis Factor-alpha*

Huntington's disease(HD) is an autosomal dominantly inherited neurodegenerative disease, which is characterized by choreic movement and progressive dementia. HD is a disease caused by CAG repeat expansion of huntintin gene and definitely diagnosed or is excluded by molecular genetic analysis. Juvenile HD, of which onset is in children or young adult, is the most severely disabled type and shows several distinct clinical and genetic features in contrast to usual late-onset type. We report a 10 year-old girl who presented with involuntary movement, seizure and moderate dysarthria confirmed by molecular genetic analysis.
Child ; Chorea ; Dementia ; Dysarthria ; Dyskinesias ; Female ; Humans ; Molecular Biology ; Neurodegenerative Diseases ; Seizures ; Young Adult

Glanzmann's thrombasthenia is a rare autosomal recessive hemorrhagic disorder of platelet function with missing or abnormal platelet plasma membrane glycoprotein IIb-IIIa, which functions as a receptor for fibrinogen. We have experienced a case of thrombasthenia in a 6-year-old female whose chief complaints were easy bruising, frequent epistaxis, arthralgia and swelling of the right ankle joint. Bleeding time was prolonged in the presence of normal platelet levels and the platelet aggregation test showed lack of aggregation after exposure to ADP, epinephrine and collagen, but showed an aggregation response to ristocetin. Platelet analysis by flow cytometry is a successful alternative rapid diagnostic technique for Glanzmann's thrombasthenia patients as well as for carriers of this disease. Flow cytometry technique provides an effective tool for investigating platelet function defects caused by altered expression or deficiency of platelet surface proteins.
Adenosine Diphosphate ; Ankle Joint ; Arthralgia ; Bleeding Time ; Blood Platelets ; Cell Membrane ; Child ; Collagen ; Epinephrine ; Epistaxis ; Female ; Fibrinogen ; Flow Cytometry* ; Glycoproteins ; Hemorrhagic Disorders ; Humans ; Membrane Proteins ; Platelet Aggregation ; Ristocetin ; Thrombasthenia*

Glanzmann's thrombasthenia is a rare autosomal recessive hemorrhagic disorder of platelet function with missing or abnormal platelet plasma membrane glycoprotein IIb-IIIa, which functions as a receptor for fibrinogen. We have experienced a case of thrombasthenia in a 6-year-old female whose chief complaints were easy bruising, frequent epistaxis, arthralgia and swelling of the right ankle joint. Bleeding time was prolonged in the presence of normal platelet levels and the platelet aggregation test showed lack of aggregation after exposure to ADP, epinephrine and collagen, but showed an aggregation response to ristocetin. Platelet analysis by flow cytometry is a successful alternative rapid diagnostic technique for Glanzmann's thrombasthenia patients as well as for carriers of this disease. Flow cytometry technique provides an effective tool for investigating platelet function defects caused by altered expression or deficiency of platelet surface proteins.
Adenosine Diphosphate ; Ankle Joint ; Arthralgia ; Bleeding Time ; Blood Platelets ; Cell Membrane ; Child ; Collagen ; Epinephrine ; Epistaxis ; Female ; Fibrinogen ; Flow Cytometry* ; Glycoproteins ; Hemorrhagic Disorders ; Humans ; Membrane Proteins ; Platelet Aggregation ; Ristocetin ; Thrombasthenia*

Repetitive nerve stimulation test(RNS) was performed on 32 patients with myasthenia gravis who showed positive response to the tensilon test. The result of the repetitive stimulation test before and after the tensilon injection was compared with the point of improvement of the CMAP and decremental response. The results were as follows: 1. All patients with myasthenis gravis showed decremental responses to the low rate of stimulation in RNS. 2. The results after tensilon injections showed significant improvements of the CMAP in ocularis oculi(OOC), flexor carpi ulnaris(FCU)(P <0.0005), deltoid and abductor digiti quinti muscles(ADQ)(P < 0.005). 3. There were improvements of decremental responses after tensilon injection. 1) At 2/sec, there were significant improvements in the muscles of ADQ(P <0.005), deltoidl OOC and FCU(P < 0.0005). 2) At 3/sec, there were significant improvements in the muscles of OOC(P <0.005), deltoid, FCU and ADQ(P < 0.0005). 3) At 5/sec, there were also significant improvements in all the muscles of OOC, deltoid, ADQ, FCU(P <0.0005). These results showed that tensilon improves the RNS abnorrnalities in myasthenic patients with positive tensilon test. We think these findings before and after the tensilon injection are helpful to diagnose and differentiate a patient with suspected myasthenia gravis who has a negative tensilon test or insignificant RNS findings.
Edrophonium* ; Humans ; Muscles ; Myasthenia Gravis*

Repetitive nerve stimulation test(RNS) was performed on 32 patients with myasthenia gravis who showed positive response to the tensilon test. The result of the repetitive stimulation test before and after the tensilon injection was compared with the point of improvement of the CMAP and decremental response. The results were as follows: 1. All patients with myasthenis gravis showed decremental responses to the low rate of stimulation in RNS. 2. The results after tensilon injections showed significant improvements of the CMAP in ocularis oculi(OOC), flexor carpi ulnaris(FCU)(P <0.0005), deltoid and abductor digiti quinti muscles(ADQ)(P < 0.005). 3. There were improvements of decremental responses after tensilon injection. 1) At 2/sec, there were significant improvements in the muscles of ADQ(P <0.005), deltoidl OOC and FCU(P < 0.0005). 2) At 3/sec, there were significant improvements in the muscles of OOC(P <0.005), deltoid, FCU and ADQ(P < 0.0005). 3) At 5/sec, there were also significant improvements in all the muscles of OOC, deltoid, ADQ, FCU(P <0.0005). These results showed that tensilon improves the RNS abnorrnalities in myasthenic patients with positive tensilon test. We think these findings before and after the tensilon injection are helpful to diagnose and differentiate a patient with suspected myasthenia gravis who has a negative tensilon test or insignificant RNS findings.
Edrophonium* ; Humans ; Muscles ; Myasthenia Gravis*

Pure sensory stroke (PSS), first described by Fisher in 1965, is a clinical condition characterized by numbness and paresthesia of the face, arm and trunk on one side, in absence of other neurologic deficit. PSS could arise anywhere along the sensory system from the cerebral cortex to the medulla. The authors experienced 5 patients with PSS: one patient had a hemorrhage on the thalamocortical pathway including the internal capsule and the corona radiata. Two another had thalarnic lesions. The fourth had a pontine hemorrhage with perioral onionpeel distributed face sensor- involvement. And the last had a pontine lacune involving crossed trigeminothalamic tract and lateral spinothalamic tract.
Arm ; Cerebral Cortex ; Hemorrhage ; Humans ; Hypesthesia ; Internal Capsule ; Neurologic Manifestations ; Paresthesia ; Spinothalamic Tracts ; Stroke*

PURPOSE: To evaluate the refractive outcomes of small incision lenticule extraction (SMILE) in high myopia patients compared with mild to moderate myopia patients. METHODS: This study included 332 eyes of 166 myopic patients treated with SMILE using Visumax 500 kHz femtosecond laser. Treated eyes were divided into 2 groups according to preoperative spherical equivalent (SE): mild to moderate myopia (A group, <-6.0 D) and high myopia (B group, > or =-6.0 D). Follow-up visits were at 1 day, 1 week, 1 month, 3 months, and 6 months. The outcome measures included uncorrected distance visual acuity (UDVA), best corrected distance visual acuity (BDVA), postoperative SE, efficacy index, safety index and predictability. RESULTS: Preoperative SE was -4.85 +/- 0.86 D in the A group and -7.70 +/- 1.0 D in the B group. No differences were observed between -0.04 +/- 0.29 D in the A group and -0.30 +/- 0.37 D in the B group at 6 months postoperatively (p = 0.062). At 6 months postoperatively, 98.3% and 97.3% had UDVA of 20/25 or better in the A group and B group, respectively. In the A group, 97.3% and 100% were within +/-0.5 D and +/-1.0 D of intended correction and in the B group, 91.7% and 96.9% were within +/-0.5 D and +/-1.0 D, respectively. Efficacy indices were 1.02 +/- 0.19 in the A group and 0.99 +/- 0.18 in the B group. Safety indices were 1.16 +/- 0.16 in the A group and 1.14 +/- 0.16 in the B group. The efficacy and safety indices were not significantly different between the A and B groups at 6 months postoperatively (p = 0.09, p = 0.695, respectively). CONCLUSIONS: This study showed that SMILE is effective and safe for correcting high myopia as well as mild to moderate myopia.
Follow-Up Studies ; Humans ; Myopia* ; Outcome Assessment (Health Care) ; Visual Acuity