Spontaneous Bone Regeneration After Large Craniectomy in Pediatric Patient.

BACKGROUND: In children, decompressive craniectomy is commonly performed in cases of increased intracranial pressure that is not medically managed. Currently, it is standard practice to perform cranioplasty after decompressive craniectomy, although optimal timing for the procedure remains controversial. To date, few studies have reported spontaneous cranial bone regeneration in children without intervention. CASE DESCRIPTION: A 7-year-old female presented with frontotemporal bone fractures accompanied by dura mater lacerations and brain edema after a motor vehicle accident. She underwent a large decompressive craniectomy and repair of the lacerated dura with a collagen dural substitute. The patient was discharged from the hospital and did not present for follow-up until 10 months after surgery. At that time, computed tomography imaging revealed remarkable spontaneous bone regeneration. With conservative management, she developed enough bone regeneration in the calvarial defect area that cranioplasty surgery was deemed unnecessary. To this date, the patient has no aesthetic deformation of the skull bone and does not exhibit any residual cognitive impairment or motor deficits. CONCLUSIONS: This case report shows that cranial bone regeneration is possible in children older than 6 years old, bypassing the need for cranioplasty after decompressive craniectomy. On the basis of this observation, we recommend that more studies should be performed to identify the factors involved in spontaneous skull bone regeneration in the pediatric population.

CSF concentrations of adipsin and adiponectin in patients with amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is described as a neurodegenerative disorder. However, neuroinflammation and chemokine expression are prominent pathological finding at sites of injury. Adipsin and adiponectin are molecules that are implicated in the pathogenesis of neurodegenerative and neuroimmune disorders. Adipsin and adiponectin concentrations were determined in the CSF of ALS patients and controls and the relationship of these chemokines with clinical severity and disease duration in ALS was determined. Seventy-seven ALS patients (mean age 49.5 ± 10.4 years) (mean body mass index 23.5 ± 4.5) were included. Twenty patients had bulbar, 53 spinal, and four bulbospinal onset ALS. Median adipsin CSF level was 12,650.94 pg/ml in ALS patients and 3290.98 pg/ml in controls (p < 0.001). Median adiponectin CSF level was 4608 pg/ml in ALS patients and 3453 pg/ml in controls (p = 0.1). No differences were observed in disease duration, progression rate or disease severity. There was a significant positive correlation between adipsin and adiponectin concentrations (r = 0.379, p = 0.01). No correlation with age, body mass index or ALFRS-R score was found. Adipsin was significantly elevated in CSF, suggesting that this chemokine might have a role in ALS pathogenesis. Adiponectin showed a trend towards higher concentrations, but failed to reach statistical significance. Due to the clinical heterogeneity in our cohort, these chemokines do not appear to be associated with disease duration or severity.

Stem-cell transplantation into the frontal motor cortex in amyotrophic lateral sclerosis patients.

BACKGROUND AIMS: Amyotrophic lateral sclerosis (ALS) is characterized by the selective death of motor neurons. CD133(+) stem cells are known to have the capacity to differentiate into neural lineages. Stem cells may provide an alternative treatment for ALS and other neurodegenerative diseases. METHODS: Five men and five women (aged 38-62 years) with confirmed ALS were included in this study. Our institutional ethics and research committees approved the protocol. After informed consent was obtained, patients underwent Hidrogen-Magnetic Resonance Imaging (H-MRI) spectroscopy and were given scores according to an ALS functional rating scale, Medical Research Council power muscle scale and daily living activities. Bone marrow was stimulated with 300 microg filgrastim subcutaneously daily for 3 days. Peripheral blood mononuclear cells were obtained after admission by leukapheresis. The cell suspension was conjugated with anti-human CD133 superparamagnetic microbeads, and linked cells were isolated in a magnetic field. The isolated cells (2.5-7.5x10(5)) were resuspended in 300 microL of the patient's cerebrospinal fluid, and implanted in motor cortexes using a Hamilton syringe. Ten patients with confirmed ALS without transplantation were used as a control group. Patients were followed up for a period of 1 year. RESULTS: The autologous transplantation of CD133(+) stem cells into the frontal motor cortex is a safe and well-tolerated procedure in ALS patients. The survival of treated patients was statistically higher (P=0.01) than untreated control patients. CONCLUSIONS: Stem-cell transplantation in the motor cortex delays ALS progression and improves quality of life.