Alexander disease (AD) is a rare leukodystrophy of the central nervous system of unknown etiology. AD is characterized by progressive failure of central myelination and the accumulation of Rosenthal fibers in astrocytes, and is inevitably lethal in nature. Symptomatically, AD is associated with leukoencephalopathy with macrocephaly, seizures, and psychomotor retardation in infants, and usually leads to death within the first decade. Its characteristic magnetic resonance imaging (MRI) findings have been described as demyelination predominantly in the frontal lobe. Moreover, dominant mutations in the GFAP gene, coding for glial fibrillary acidic protein (GFAP), a principal astrocytic intermediate filament protein, have been shown to lead to AD. The disease can now be detected by genetic diagnosis. We report the Korean case of an 8-month-old male patient with AD. He was clinically characterized due to the presence of psychomotor retardation, megalencephaly, spasticity, and recurrent seizures including infantile spasms which is a remarkable presentation. Demyelination in the frontal lobe and in a portion of the temporal lobe was demonstrated by brain MRI. Moreover, DNA analysis of peripheral blood showed the presence of a R239L mutation in the GFAP gene, involving the replacement of guanine with thymine.
Alexander Disease; Spasms, Infantile; Mutation; Glial Fibrillary Acidic Protein; Magnetic Resonance Imaging
Electrospray process was developed for organic layer deposition onto polymer organic light-emitting diode [PLED] devices in this work. An electrospray can be used to produce nanometer-scale thin films by electric repulsion of microscale fine droplets. PLED devices made by an electrospray process were compared with spin-coated ones. The PLED device fabricated by the electrospray process showed maximum current efficiency of 24 cd/A, which was comparable with that of the spin-coating process. The electrospray process required a higher concentration of hole and electron transport materials in the inks than spin-coating processes to achieve PLED maximum performance. Photoluminescence [PL] at 407 nm was observed using electrosprayed poly(N-vinyl carbazole) films, whereas a peak at 410 nm was observed with the spin-coated ones. Similar difference in peak position was observed between aromatic and nonaromatic solvents in the spin-coating process. PLED devices made by the electrospray process showed lower current density than that of spin-coated ones. The PL peak shift and reduced current of electrosprayed films can therefore be attributed to the conformation of the polymer.
organic light-emitting diodes; electrospray, polymer, conformation.
A single balloon extrapedicular kyphoplasty has been introduced as one of the unilateral approaches for thoracic compression fractures; however, the unilateral extrapedicular technique in the lumbar area needs a further understanding of structures in the lumbar area. The purpose of the present study is to describe methods and pitfalls of this procedure based on the anatomy of the lumbar area and to analyze clinical outcome and complications.
Anatomical evaluation was performed with 2 human cadavers. A retrospective review of unilateral extrapedicular approaches yielded 74 vertebral levels in 55 patients that were treated with unilateral extrapedicular vertebroplasty and kyphoplasty. Radiographic assessment included the restoration rate of vertebral height and correction of kyphosis.
Anatomical evaluation indicates that the safe needle entry zone of bone for the extrapedicular approach was located in the supero-lateral aspect of the junction between the pedicle and vertebral body. The unilateral extrapedicular procedure achieved adequate pain relief with a mean decreases in pain severity of 7.25±1.5 and 2.0±1.4, respectively. Complications were 1 retroperitoneal hematoma, 6 unilateral fillings and 3 epidural leak of the polymethylmethacrylate.
The method of a unilateral extrapedicular approach in kyphoplasty and vertebroplasty in the lumbar area might be similar to that in thoracic approach using a route via the extrapedicular space. However, different anatomical characteristics of the lumbar area should be considered.
Kyphoplasty; Vertebroplasty; Anatomy; Lumbar vertebra; Complications; Outcome
Degenerated conditions such as herniated disc or spinal stenosis are common etiologies of lumbar radiculopathy. Less common etiologies include spinal extradural cyst such as synovial cysts and ganglion cysts. Ganglion cyst of the posterior longitudinal ligament (PLL) of the spine is a rare entity that can result in classical sciatica. Posterior longitudinal ligament cyst has no continuity with the facet joint and has no epithelial lining. Two young male patients presented with unilateral sciatica and were found to have intraspinal cystic lesions causing lumbar radiculopathy. Magnetic resonance imaging demonstrated rounded, cystic lesions (i.e., hypointense on T1- but hyperintense on T2-weighted images) adjacent to minimally dehydrated, nonherniated disc spaces in both cases. These patients underwent posterior decompression and cysts were excised, and their sciatic symptoms were completely resolved. Histological examination showed typical features of ganglion cysts in these cases.
Ganglion cyst; Intervertebral disc; Posterior longitudinal ligament
The lumbar intraspinal epidural ganglion cyst has been a rare cause of the low back pain or leg pain. Ganglion cysts and synovial cysts compose the juxtafacet cysts. Extensive studies have been performed about the synovial cysts, however, very little has been known about the ganglion cyst. Current report is about two ganglion cysts associated with implicative findings in young male patients. We discuss about the underlying pathology of the ganglion cyst based on intraoperative evidences, associated disc herniation at the same location or severe degeneration of the ligament flavum that the cyst originated from in young patients.
Ganglion cyst; Synovial cyst
The authors reviewed clinical and radiological outcomes in patients with three column injury of the cervical spine who had undergone posterior cervical fixation using Nitinol shape memory alloy loop in the anterior-posterior combined approach.
Nine patients were surgically treated with anterior cervical fusion using an iliac bone graft and dynamic plate-screw system, and the posterior cervical fixation using Nitinol shape memory loop (Davydov™) at the same time. A retrospective review was performed. Clinical outcomes were assessed using the Frankel grading method. We reviewed the radiological parameters such as bony fusion rate, height of iliac bone graft strut, graft subsidence, cervical lordotic angle, and instrument related complication.
Single-level fusion was performed in five patients, and two-level fusion in four. Solid bone fusion was presented in all cases after surgery. The mean height of graft strut was significantly decreased from 20.46±9.97 mm at immediate postoperative state to 18.87±8.60 mm at the final follow-up period (p<0.05). The mean cervical lordotic angle decreased from 13.83±11.84° to 11.37±6.03° at the immediate postoperative state but then, increased to 24.39±9.83° at the final follow-up period (p<0.05). There were no instrument related complications.
We suggest that the posterior cervical fixation using Nitinol shape memory alloy loop may be a simple and useful method, and be one of treatment options in anterior-posterior combined approach for the patients with the three column injury of the cervical spine.
Nitinol; Shape memory loop; Posterior cervical surgery; fracture fixation
We report a very rare case of a rapidly calcified chronic epidural hematoma (EDH) in a neonate. A 26-day-old female infant was referred to us from a regional hospital because of drowsy mentality and a seizure attack. She was delivered through caesarian section because normal spontaneous vaginal delivery was prolonged and failed. At birth, mild scalp swelling was found on the right frontal area. Scalp swelling was spontaneously resolved and she was discharged without any problems. On the 25th day after her birth, the baby presented with drowsiness and hypotonia following a generalized tonic-clonic seizure. Magnetic resonance imaging (MRI) and a computed tomography (CT) scan revealed a chronic EDH that had a thick layer of calcification. A small burr-hole trephination was performed and a single silastic drainage catheter was inserted. After the operation, a total of 12 ml of liquefied hematoma was drained, and the patient's mentality improved from drowsiness to alertness. The patient was asymptomatic when discharged.
Ossification; Neonate; Epidural hematoma
Cubital tunnel syndrome is the second most common entrapment neuropathy of the upper extremity. Although many different operative techniques have been introduced, none of them have been proven superior to others. Simple cubital tunnel decompression has numerous advantages, including simplicity and safety. We present our experience of treating cubital tunnel syndrome with simple decompression in 15 patients.
According to Dellon's criteria, one patient was classified as grade 1, eight as grade 2, and six as grade 3. Preoperative electrodiagnostic studies were performed in all patients and 7 of them were rechecked postoperatively. Five patients of 15 underwent simple decompression using a small skin incision (2 cm or less).
Preoperative mean value of motor conduction velocity (MCV) within the segment (above the elbow-below the elbow) was 41.8±15.2 m/s and this result showed a decrease compared to the result of MCV in the below the elbow-wrist segment (57.8±6.9 m/s) with statistical significance (p<0.05). Postoperative mean values of MCV were improved in 6 of 7 patients from 39.8±12.1 m/s to 47.8±12.1 m/s (p<0.05). After an average follow-up of 4.8±5.3 months, 14 patients of 15 (93%) reported good or excellent clinical outcomes according to a modified Bishop scoring system. Five patients who had been treated using a small skin incision achieved good or excellent outcomes. There were no complications, recurrences, or subluxation of the ulnar nerve.
Simple decompression of the ulnar nerve is an effective and successful minimally invasive technique for patients with cubital tunnel syndrome.
Cubital tunnel syndrome; Ulnar nerve; Simple decompression