Cutaneous granulomas with primary immunodeficiency in children: a report of 17 new patients and a review of the literature.

BACKGROUND: Paediatric cutaneous granuloma with primary immunodeficiency (PID) is a rare condition. The physiopathology is unclear, and treatment is challenging. We report on 17 paediatric cases and review the literature. OBJECTIVES: To make dermatologists and dermatopathologists aware of the diagnostic value of skin granulomas in paediatric PID. METHODS: We collected data on 17 patients with cutaneous granulomas and PID registered with us and also reviewed 33 cases from the literature. RESULTS: Cutaneous granuloma was the presenting feature of the PID in 15 of the 50 collated cases. The lesions presented as red-brownish nodules and infiltrated ulcerative plaques, predominantly on the face and limbs. Scleroderma-like infiltration on a single limb was observed in 10% of the cases. The associated PID was ataxia-telangiectasia (52%), combined immunodeficiency (24%), cartilage-hair hypoplasia (6%) and other subtypes (18%). The granulomas were mostly sarcoidal, tuberculoid, palisaded or undefined subtypes. In some patients, several different histopathologic granulomatous patterns were found in the same biopsy. Some granulomas were associated with the presence of a vaccine strain of rubella virus. CONCLUSION: Cutaneous granulomas associated with a PID have a variable clinical presentation. A PID can be suspected when crusty, brownish lesions are found on the face or limbs. The concomitant presence of several histological subtypes in a single patient is suggestive of a PID.

Next-Generation Sequencing for Diagnosis and Tailored Therapy: A Case Report of Astrovirus-Associated Progressive Encephalitis.

A boy with X-linked agammaglobulinemia experienced progressive global motor decline, cerebellar syndrome, and epilepsy. All standard polymerase chain reactions for neurotropic viruses were negative on cerebrospinal fluid and brain biopsy. Next-generation sequencing allowed fast identification of a new astrovirus strain (HAstV-VA1/HMO-C-PA), which led to tailor the patient's treatment, with encouraging clinical monitoring over 1 year.

Methylprednisolone or tirilazad mesylate administration after acute spinal cord injury: 1-year follow up. Results of the third National Acute Spinal Cord Injury randomized controlled trial.

OBJECT: A randomized double-blind clinical trial was conducted to compare neurological and functional recovery and morbidity and mortality rates 1 year after acute spinal cord injury in patients who had received a standard 24-hour methylprednisolone regimen (24MP) with those in whom an identical MP regimen had been delivered for 48 hours (48MP) or those who had received a 48-hour tirilazad mesylate (48TM) regimen. METHODS: Patients for whom treatment was initiated within 3 hours of injury showed equal neurological and functional recovery in all three treatment groups. Patients for whom treatment was delayed more than 3 hours experienced diminished motor function recovery in the 24MP group, but those in the 48MP group showed greater 1-year motor recovery (recovery scores of 13.7 and 19, respectively, p=0.053). A greater percentage of patients improving three or more neurological grades was also observed in the 48MP group (p=0.073). In general, patients treated with 48TM recovered equally when compared with those who received 24MP treatments. A corresponding recovery in self care and sphincter control was seen but was not statistically significant. Mortality and morbidity rates at 1 year were similar in all groups. CONCLUSIONS: For patients in whom MP therapy is initiated within 3 hours of injury, 24-hour maintenance is appropriate. Patients starting therapy 3 to 8 hours after injury should be maintained on the regimen for 48 hours unless there are complicating medical factors.

Anhedonia, alexithymia and locus of control in unipolar major depressive disorders.

The relationship between anhedonia, alexithymia and locus of control was studied in a group of healthy subjects (n = 56) and in a group of unipolar major depressives (n = 59). These dimensions were rated using the Physical Anhedonia Scale (PAS), the Toronto Alexithymia Scale (TAS) and the Internal Powerful others and Chance Scale. The unipolar major depressives were divided into two subgroups using the endogenomorphic subscale of the Hamilton Depression Rating Scale. There are respectively 25 and 17 depressives in the nonendogenomorphic (NENDO) and endogenomorphic (ENDO) subgroups. The unipolar major depressives were significantly more anhedonic, alexithymic and externalized than the controls. In the ENDO group the PAS and TAS Feelings scores were positively correlated suggesting that a third variable of emotional deficit could characterize endogenomorphic depression. In the NENDO group the PAS, TAS Daydreaming, Powerful others and Chance scores were positively correlated. In nonendogenomorphic depression anhedonia could be related to a poverty of imagery and could be interpreted as a consequence of external causes.

[Validation of the French version of the revised Personal Style Inventory (revised Personal Style Inventory, PSI-II, Robins et al., 1994)]. / Etude de validation de la version française de l'inventaire révisé de style personnel (revised Personal Style Inventory, PSI-II, Robins et al., 1994).

The aim of the present study is to determine the validity and reliability of the French version of the PSI-II. The PSI-II is a self-rating scale divided into two subscales containing each 24 items and rating sociotropy and autonomy. 202 university students (170 females, 32 males) with a mean age of 21.54 years (sd = 4.16) were included. They filled out the PSI-II and the Interpersonal Dependency Inventory (IDI). The construct validity was explored using an exploratory principal components analysis followed by an orthogonal varimax rotation. The results had shown a three-factor solution with a "sociotropy" factor and two "autonomy" factors. The Cronbach alpha coefficients were respectively 0.83 and 0.72 for the sociotropy and autonomy subscales. The Pearson correlations between the PSI-II and IDI subscales showed significant correlation first between the sociotropy subscale and the two subscales of the IDI rating dependency, the values were respectively 0.67 (p < 0.001) and 0.44 (p < 0.001), and secondly between the autonomy subscales of the PSI-II and IDI, the value was 0.39 (p < 0.001). The sociotropy subscale items correlated significantly with the total score of the subscale with a mean of 0.46. 23 items of the autonomy subscale correlated with the total score with a mean of 0.36. The sociotropy and autonomy scores were respectively 93.98 (sd = 14.22) and 84.55 (sd = 11.69). The French version of the PSI-II had satisfactory metrological parameters and allows to study sociotropy and autonomy in French population.

Administration of methylprednisolone for 24 or 48 hours or tirilazad mesylate for 48 hours in the treatment of acute spinal cord injury. Results of the Third National Acute Spinal Cord Injury Randomized Controlled Trial. National Acute Spinal Cord Injury Study.

OBJECTIVE: To compare the efficacy of methylprednisolone administered for 24 hours with methyprednisolone administered for 48 hours or tirilazad mesylate administered for 48 hours in patients with acute spinal cord injury. DESIGN: Double-blind, randomized clinical trial. SETTING: Sixteen acute spinal cord injury centers in North America. PATIENTS: A total of 499 patients with acute spinal cord injury diagnosed in National Acute Spinal Cord Injury Study (NASCIS) centers within 8 hours of injury. INTERVENTION: All patients received an intravenous bolus of methylprednisolone (30 mg/kg) before randomization. Patients in the 24-hour regimen group (n=166) received a methylprednisolone infusion of 5.4 mg/kg per hour for 24 hours, those in the 48-hour regimen group (n=167) received a methylprednisolone infusion of 5.4 mg/kg per hour for 48 hours, and those in the tirilazad group (n=166) received a 2.5 mg/kg bolus infusion of tirilazad mesylate every 6 hours for 48 hours. MAIN OUTCOME MEASURES: Motor function change between initial presentation and at 6 weeks and 6 months after injury, and change in Functional Independence Measure (FIM) assessed at 6 weeks and 6 months. RESULTS: Compared with patients treated with methylprednisolone for 24 hours, those treated with methylprednisolone for 48 hours showed improved motor recovery at 6 weeks (P=.09) and 6 months (P=.07) after injury. The effect of the 48-hour methylprednisolone regimen was significant at 6 weeks (P=.04) and 6 months (P=.01) among patients whose therapy was initiated 3 to 8 hours after injury. Patients who received the 48-hour regimen and who started treatment at 3 to 8 hours were more likely to improve 1 full neurologic grade (P=.03) at 6 months, to show more improvement in 6-month FIM (P=.08), and to have more severe sepsis and severe pneumonia than patients in the 24-hour methylprednisolone group and the tirilazad group, but other complications and mortality (P=.97) were similar. Patients treated with tirilazad for 48 hours showed motor recovery rates equivalent to patients who received methylprednisolone for 24 hours. CONCLUSIONS: Patients with acute spinal cord injury who receive methylprednisolone within 3 hours of injury should be maintained on the treatment regimen for 24 hours. When methylprednisolone is initiated 3 to 8 hours after injury, patients should be maintained on steroid therapy for 48 hours.

[Validation of the French version of the Snaith-Hamilton Pleasure Scale (SHAPS, Snaith et al. 1995). Determination of the statistical parameters in 208 normal subjects and 103 hospitalized patients presenting with depression or schizophrenia]. / Etude de validation de la version française de l'échelle de plaisir de Snaith et Hamilton (Snaith-Hamilton pleasure scale, SHAPS, Snaith et al. 1995). Détermination des paramètres métrologiques chez 208 sujets sains et chez 103 malades hospitalisés présentant une dépression ou une schizophrénie.

The aim of the study is to explore the validity and the reliability of the French version of the SHAPS in two groups of subjects. 208 healthy students and 103 inpatients meeting the RDC criteria for schizophrenia or depression filled out the French versions of the SHAPS and the revised Physical Anhedonia Scale of Chapman and Chapman. The internal consistency of the SHAPS was measured in each group using first the Kuder Richardson coefficient (point biserial) between the items and the total score. The concurrent validity was studied using the Pearson correlation coefficient between the SHAPS and the PAS in each group. The predictive validity was determined by the comparison of the SHAPS score between the two groups. The reliability was studied using a test-retest in a sub-group (n = 32) extracted of the control group. The values of the KR 20 in the healthy and psychiatric groups were respectively 0.47 and 0.80. The mean of the correlations between the items and the total score were respectively 0.35 and 0.52. The values of the correlations between the SHAPS and the PAS were respectively in the normal and psychiatric groups 0.39 (p < 0.001) and 0.34 (p < 0.001). Psychiatric subjects had a higher score (m = 2.33, sd = 2.68) on the SHAPS than the normals (m = 0.89, sd = 1.18) [F (1,303) = 12.26, p = 0.0005]. The test-retest showed a correlation of 0.56 (p < 0.01) between the two passations of the SHAPS. The metrological parameters of the SHAPS were discussed as well as the utility of that scale compared to the PAS.

[Locus of control in alcoholics: comparative study of 64 alcoholics vs 50 hospitalized patients and 50 normal controls]. / Le lieu de contrôle chez les alcooliques: étude comparative de 64 alcooliques versus 50 témoins hospitalisés et 57 sujets sains.

The locus of control is a construct that consists of factors that influence and contribute to a person's belief concerning the extent and degree to which he or others can influence life events. The study had four purposes. They were to examine differences between psychiatric inpatients alcoholics and non alcoholics on the internal, chance and powerful others subscales of the French version of Levenson's scale, to evaluate differences on these scores between the alcoholics who accepted the alcohol program treatment proposed at the admission (hospitalization of 15 days with a directive and structured context) and those who refused this treatment, to assess the impact of treatment on the Levenson scale scores of the alcoholics, and finally to determine whether the Levenson scale scores differentiate between treatment successes and failures (evaluated at three months). 171 subjects were divided into three groups: 64 patients hospitalized in psychiatry for chronic alcoholism (ICD-10 criteria for alcohol dependency), 50 patients hospitalized in psychiatry for an other pathology (control group) and 57 healthy subjects (normal group). These subjects filled out the IPC (Internal, Powerful others, Chance) scale of Levenson, different from the I-E (Internal-External) Rotter's scale because it distinguishes two types of externality: one imprevisible, the chance, and the other previsible, the powerful others. The results showed that the alcoholics as the controls are more external than the healthy subjects (Chance and Powerful others subscales). They also showed that the alcoholics who refused the alcohol treatment program proposed at the admission were more internal than those who accepted. We also found that, during the treatment, the alcoholics' scores of Internality increased while their scores of externality (Chance and Powerful others) decreased. This decreasing was also found with the check inpatients. So these changes would have connections with an "hospitalization factor" and wouldn't be due to the alcohol treatment. As for the last purpose, there were no significant differences between the initial locus of control scores of the successes and those of the failures.

MASCIS evaluation of open field locomotor scores: effects of experience and teamwork on reliability. Multicenter Animal Spinal Cord Injury Study.

The Multicenter Animal Spinal Cord Injury Study (MASCIS) adopted a modified 21-point open field locomotor scale developed by Basso, Beattie, and Bresnahan (BBB) at Ohio State University (OSU) to measure motor recovery in spinal-injured rats. BBB scores categorize combinations of rat hindlimb movements, trunk position and stability, stepping, coordination, paw placement, toe clearance, and tail position, representing sequential recovery stages that rats attain after spinal cord injury. A total of 22 observers from 8 participating centers assessed 18 hindlimbs of 9 rats at 2-6 weeks after graded spinal cord injury. The observers were segregated into 10 teams. The teams were grouped into 3 cohorts (A, B, and C), consisting of one experienced team from OSU and two non-OSU teams. The cohorts evaluated the rats in three concurrent and sequential sessions. After viewing a rat for 4 min, individual observers first assigned scores without discussion. Members of each team then discussed and assigned a team score. Experience (OSU vs. non-OSU) and teamwork (individual vs. team) had no significant effect on mean scores although the mean scores of one cohort differed significantly from the others (p = 0.0002, ANOVA). However, experience and teamwork significantly influenced reliability of scoring. OSU team scores had a mean standard deviation or discordance of 0.59 points, significantly less than 1.31 points for non-OSU team scores (p = 0.003, ANOVA) and 1.30 points for non-OSU individual scores (p = 0.001, ANOVA). Discordances were greater at the upper and lower ends of the scale, exceeding 2.0 in the lower (< 5) and upper (> 15) ends of the scale but were < 1.0 for scores between 4 and 16. Comparisons of non-OSU and OSU team scores indicated a high reliability coefficient of 0.892 and a correlation index (r2) of 0.894. These results indicate that inexperienced observers can learn quickly to assign consistent BBB scores that approach those given by experienced teams, that the scores are most consistent between 4 and 16, and that experience improves consistency of team scores.

The neuroprotective effect of high-dose methylprednisolone in rat spinal cord hemisection.

Multiple studies support a neuroprotective effect for high-dose methylprednisolone (MP) in acute blunt spinal cord injury. We know of no study that addresses the role of MP in prophylaxis for surgical trauma to the spinal cord or for the treatment of non-missile penetrating injuries to the spinal cord. We examined the neuroprotective effect of MP as measured by the retrograde transport of the fluorescent tracer Fluoro-Gold in 20 rats undergoing C-2 hemisection. Mean cell counts of retrogradely labeled rubrospinal neurons were determined 1 week post-injury. The group receiving MP had a significantly higher (P < 0.0001) number of labeled cells (x = 594) compared to controls (x = 387). The highly significant increase in mean cell counts in rats receiving steroids suggests less secondary axonal injury in the MP group. These findings are the first report of a neuroprotective effect of MP in rat spinal cord hemisection. We suggest that MP may be beneficial as prophylaxis during planned or incidental surgical trauma to the spinal cord and after non-missile penetrating injuries to the spinal cord.

[Operationalization of the "locus of control" concept: translation and first validation study of the Levenson control scale (IPC: the internal powerful others and chance scale)]. / Opérationnalisation du concept de "lieu de contrôle": traduction et première étude de validation de l'échelle de contrôle de Levenson (IPC: the internal powerful others and chance scale).

This work presents the study of validation of the French version of the Internal Powerful others and Chance Scale (IPC) of Levenson. A principal components analysis was carried out on a group of 134 healthy subjects. The Cronbach alpha coefficient and the correlation between the items and the total score of each sub-scale of the IPC were calculated. Our results were discussed in comparison with others studies.

Rubrospinal neurons and retrograde transport of fluoro-gold in acute spinal cord injury--a dose-response curve.

Using the fluorescent tracer Fluoro-Gold, we studied the relationship between severity of spinal cord trauma and the number of retrogradely labeled rubrospinal neurons in the rat. We compared the mean cell counts of retrogradely labeled rubrospinal neurons in rats receiving 0, 20, 40, and 60 g/cm concussive spinal cord traumas. For each incremental increase in trauma a significant reduction in mean cell counts took place (P < 0.0001). We demonstrate a dose-response relationship between trauma severity and Fluoro-Gold retrogradely labeled rubrospinal neurons in acute spinal cord injury of the rat. This relationship may be helpful in quickly assessing the efficacy of therapeutic interventions in acute spinal cord injury. Previous studies with HRP failed to demonstrate such a dose-response curve. Fluoro-Gold may be a more sensitive indicator of neuronal survival than HRP in the traumatized rat spinal cord.

Protective effect of methylprednisolone on vascular injury in rat spinal cord injury.

High-dose methylprednisolone (MP) given to patients within 8 h of traumatic spinal cord improved neural function at 6 and 12 months, suggesting a probable secondary injury process that may be amenable to therapeutic intervention. Vascular injury plays an important role in the secondary injury process of CNS trauma. We have examined the effect of MP on vascular changes, including tissue edema, vascular permeability, and polymorphonuclear (PMN) cell infiltration in a rat model of spinal cord impact injury. MP significantly reduced extravasation of fluorescein isothiocyanate dextran (FITC-D), a macromolecular tracer, by 64.3% and 50.7% with trauma forces of 20 and 40 g-cm, respectively, when MP was administered IV immediately after trauma at a bolus of 165 mg/kg, with a subsequent continuous MP infusion at 31.5 mg/kg/h for 23 h. MP reduced the water content in the 40 g-cm traumatic cord lesion to 73.0% compared to the traumatic control (74.3%, p < 0.001) at the same schedule of large dose 24-h infusion. The same doses of MP showed a trend to decrease the extent of neutrophil infiltration as determined by myeloperoxidase (MPO) activity, but the change was not significant. MP had little effect in decreasing FITC-D extravasation and cord edema when given at a lower dose (bolus of 30 mg/kg with continued infusion of 1.3 mg/kg/h for 23 h). MP did not reduce extravasation of FITC-D and edema when administered IV as one bolus injection at high (165 mg/kg) or low (30 mg/kg) doses.(ABSTRACT TRUNCATED AT 250 WORDS)

A sensitive fluorometric method for measurement of vascular permeability in spinal cord injury.

A sensitive fluorometric method was modified for the evaluation of drug action upon vascular permeability in rat spinal cord injury. Fluorescein isothiocyanate-conjugated dextran (FITC-D MW 71,200), used as a macromolecular tracer, was injected iv 2 hours before sacrifice. The optimal pH for FITC-D fluorescence was 8.2. The recovery in spinal cord was 101.4 +/- 4.0% (mean +/- SD). The extent of FITC-D extravasation, expressed as the vascular injury index (VII), was increased in proportion to the trauma force. The peak of VII after trauma was at 2 hours. This fluorometric method is sensitive, simple, and reliable for evaluation of drug effects upon vascular permeability in CNS trauma.

Leukotriene B4 release and polymorphonuclear cell infiltration in spinal cord injury.

Activation of arachidonic acid occurs after spinal cord injury. Leukotriene B4 is a lipoxygenase metabolite of arachidonic acid. In a rat model of experimental spinal cord injury, we found that the leukotriene B4 content was less than the sensitivity of our assay (8 pg/mg of protein) in non-traumatized spinal cord. Leukotriene B4 was detectable in traumatized cord (mean +/- SE, 25 +/- 5 pg/mg of protein; n = 3). Release of leukotriene B4 from spinal cord slices into the incubation medium was also noted after trauma (9 +/- 1 pg/mg of protein; n = 12) and was enhanced by exposure of traumatized spinal cord slices to the calcium ionophore A23187 (375 +/- 43 pg/mg of protein; n = 12). The amount of leukotriene B4 released corresponded to the extent of post-traumatic polymorphonuclear cell infiltration determined by a myeloperoxidase assay. Results from this study suggest that the source of leukotriene B4 in spinal cord injury is infiltrating polymorphonuclear cells.

Establishing brain death in South Carolina: a clinician's guide.

With recent technological and medical advances, basic cardiopulmonary function can now be prolonged in many patients. Concurrently, organ transplantations have become more common and interest in living wills has increased. As a result, the South Carolina physician is increasingly obligated to determine whether a patient receiving cardiopulmonary support is dead due to irreversible cessation of brain function (ICBF) (brain dead). Here we review the bedside clinical valuation of brain death (ICBF), the adjunctive use of the EEG and other tests, and the South Carolina laws pertaining to this complex decision.

Intracranial fibro-osseous lesion. Case report.

A rare tumor closely associated with the trigeminal ganglion produced a syndrome of atypical facial pain in a 44-year-old man. Two previous reports have designated similar tumors as "fibro-osseous lesions." The distinctive morphological appearance is illustrated and possible histogenesis discussed.