Nature of child sexual abuse and psychopathology in adult survivors: results from a clinical sample in Scotland.

The relationship between history of childhood sexual abuse and psychopathology in adult life is well established. However, understanding of the mechanisms by which abuse exerts its effects is limited. To our knowledge, this is the first study which investigates the relationship between a wide range of sexual abuse characteristics (i.e. age at onset, frequency of assaults, number of perpetrators and their relationship to the victim) and the severity of psychopathological disorders in a large sample of adult child sexual abuse (CSA) survivors who attended a specialist Psychotherapy Service for CSA survivors. CSA survivors in our study experienced severe sexual assault(s) in their early years and presented with severe pathology which could suggest a strong causal link. However, none of the examined trauma characteristics significantly predicted severity of psychopathology. This may suggest that for severely disordered, treatment-seeking CSA survivors post-abuse psychopathology could be caused by other factors. The study adds to the growing body of evidence suggesting that CSA effects may be dependent on factors which are not necessarily related to the nature of sexual abuse. The study findings will help improve clinicians' insight into the determinants of psychopathology.

[Globoid cell leukodystrophy of adult. A first case in Poland]. / Leukodystrofia globoidalna doroslych. Pierwszy przypadek w Polsce.

Krabbe disease (globoid cell leukodystrophy) is a progressive, autosomal recessive disorder affecting peripheral and central nervous system. This disease is associated with mutation in GALC gene and its locus has been mapped to chromosome 14q31. GALC gene codes lysosomal hydrolytic enzyme: galactocerebroside ß-galactosidase (galactosylceramidase) which is crucial for degradation of galactolipids, mostly galactosylceramide and galactosylsphingosine (psychosine). The disease may be subdivided into four types: infantile form with onset within the first six months, child form presenting between 6 months and 3 years, juvenile form presenting between 3 and 10 years and the rarest adult form with onset after 10 years. The diagnosis of Krabbe disease is based on clinical findings and confirmed with galactocerebroside ß-galactosidase deficiency. We have found family with adult-onset disease. To our knowledge, this is the first observation of patient with adult form of Krabbe disease in Poland.

A randomized, double-blind, placebo-controlled, parallel-group, enriched-design study of nabiximols* (Sativex(®) ), as add-on therapy, in subjects with refractory spasticity caused by multiple sclerosis.

BACKGROUND: Spasticity is a disabling complication of multiple sclerosis, affecting many patients with the condition. We report the first Phase 3 placebo-controlled study of an oral antispasticity agent to use an enriched study design. METHODS: A 19-week follow-up, multicentre, double-blind, randomized, placebo-controlled, parallel-group study in subjects with multiple sclerosis spasticity not fully relieved with current antispasticity therapy. Subjects were treated with nabiximols, as add-on therapy, in a single-blind manner for 4weeks, after which those achieving an improvement in spasticity of ≥20% progressed to a 12-week randomized, placebo-controlled phase. RESULTS: Of the 572 subjects enrolled, 272 achieved a ≥20% improvement after 4weeks of single-blind treatment, and 241 were randomized. The primary end-point was the difference between treatments in the mean spasticity Numeric Rating Scale (NRS) in the randomized, controlled phase of the study. Intention-to-treat (ITT) analysis showed a highly significant difference in favour of nabiximols (P=0.0002). Secondary end-points of responder analysis, Spasm Frequency Score, Sleep Disturbance NRS Patient, Carer and Clinician Global Impression of Change were all significant in favour of nabiximols. CONCLUSIONS: The enriched study design provides a method of determining the efficacy and safety of nabiximols in a way that more closely reflects proposed clinical practice, by limiting exposure to those patients who are likely to benefit from it. Hence, the difference between active and placebo should be a reflection of efficacy and safety in the population intended for treatment.

Islet amyloid deposition limits the viability of human islet grafts but not porcine islet grafts.

Islet transplantation is a promising treatment for diabetes but long-term success is limited by progressive graft loss. Aggregates of the beta cell peptide islet amyloid polypeptide (IAPP) promote beta cell apoptosis and rapid amyloid formation occurs in transplanted islets. Porcine islets are an attractive alternative islet source as they demonstrate long-term graft survival. We compared the capacity of transplanted human and porcine islets to form amyloid as an explanation for differences in graft survival. Human islets were transplanted into streptozotocin-diabetic immune-deficient mice. Amyloid deposition was detectable at 4 weeks posttransplantation and was associated with islet graft failure. More extensive amyloid deposition was observed after 8 weeks. By contrast, no amyloid was detected in transplanted neonatal or adult porcine islets that had maintained normoglycemia for up to 195 days. To determine whether differences in IAPP sequence between humans and pigs could explain differences in amyloid formation and transplant viability, we sequenced porcine IAPP. Porcine IAPP differs from the human sequence at 10 positions and includes substitutions predicted to reduce its amyloidogenicity. Synthetic porcine IAPP was considerably less amyloidogenic than human IAPP as determined by transmission electron microscopy, circular dichroism, and thioflavin T binding. Viability assays indicated that porcine IAPP is significantly less toxic to INS-1 beta cells than human IAPP. Our findings demonstrate that species differences in IAPP sequence can explain the lack of amyloid formation and improved survival of transplanted porcine islets. These data highlight the potential of porcine islet transplantation as a therapeutic approach for human diabetes.

Impaired proinsulin processing is a characteristic of transplanted islets.

We sought to determine whether recipients of islet transplants have defective proinsulin processing. Individuals who had islet allo- or autotransplantation were compared to healthy nondiabetic subjects. Insulin (I), total proinsulin (TP), intact proinsulin and C-peptide (CP) were measured in samples of fasting serum by immunoassay, and the ratios of TP/TP+I and TP/CP were calculated. Islet allotransplant recipients had elevated TP levels relative to nondiabetic controls (16.8 [5.5-28.8] vs. 8.4 [4.0-21.8] pmol/L; p < 0.05) and autologous transplant recipients (7.3 [0.3-82.3] pmol/L; p < 0.05). Islet autotransplant recipients had significantly higher TP/TP+I ratios relative to nondiabetic controls (35.9 +/- 6.4 vs. 13.9 +/- 1.4%; p < 0.001). Islet allotransplant recipients, some of whom were on insulin, tended to have higher TP/TP+I ratios. The TP/CP ratio was significantly higher in both islet autotransplant (8.9 [0.6-105.2]; p < 0.05) and allotransplant recipients (2.4 [0.8-8.8]; p < 0.001) relative to nondiabetic controls (1.4 [0.5-2.6]%). Consistent with these findings, TP/TP+I and TP/CP values in islet autotransplant recipients increased significantly by 1-year posttransplant compared to preoperative levels (TP/CP: 3.8 +/- 0.6 vs. 23.3 +/- 7.9%; p < 0.05). Both allo- and autotransplant subjects who received <10,000 IE/kg had higher TP/CP ratios than those who received >10,000 IE/kg. Islet transplant recipients exhibit defects in the processing of proinsulin similar to that observed in subjects with type 2 diabetes manifest as higher levels of total proinsulin and increased TP/TP+I and TP/CP ratios.

Activity of selected enzymes in erythrocytes and level of plasma antioxidants in response to single whole-body cryostimulation in humans.

The influence of extremely low temperatures on the human body and physiological reactions is not fully understood. The aim of this research was to estimate the influence of a single exposure to cryogenic temperature (-130 degrees C), without subsequent kinesiotherapy, on the activity of the most crucial antioxidant enzymes in erythrocytes: superoxide dismutase (SOD), catalase (CAT), glutathione reductase (R-GSSG), glutathione peroxidase (GPx) and glutathione transferase (T-GSH). In the plasma, the concentrations of glutathione, uric acid, albumins and extra-erythrocyte haemoglobin as components of the non-enzymatic antioxidant system were evaluated. The subjects were 10 healthy young men. Blood was sampled in the morning on the day of cryostimulation, 30 min after cryostimulation and the next morning. The enzymatic response of the antioxidant defence to the influence of the extremely low temperature resulted in an immediate, significant, increase in GPx and R-GSSG activities, but a decrease in CAT and T-GSH activities. We observed an increase in the concentrations of all the examined non-enzymatic antioxidants, especially extra-erythrocyte haemoglobin and uric acid, which had both increased further the day after cryostimulation. The results indicate that a single stimulation with cryogenic temperatures results in oxidative stress in a healthy body, but that the level of stress is not very high. It seems that in this case the most significant role in the antioxidant mechanisms is played by peroxidase.

[SUNCT syndrome--case report]. / Zespól SUNCT--opis przypadku.

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tears (SUNCT) syndrome is a very rare disorder, first described by Sjaastad et al. in 1989. The authors report a case of that type of headache in an 80 years old woman. They also discuss the pathophysiology and treatment.

[The myasthenic syndrome after chloroquine]. / Zespól miasteniczny po stosowaniu arechiny.

The author presents a case of a 52-year old woman who suffered from dermatitis seborrhoica. She was treated with chloroquine. During the third month of treatment with chloroquine the symptoms of myasthenia appeared and led to hospitalization. The neurological and additional examinations confirmed the diagnosis. Four days after withdrawal of chloroquine treatment the myasthenic symptoms began to disappear and they finally ceased after two weeks. At this all other drugs were also withdrawn. The control examination after two months showed normal neurological status.

[Night headache: report of 2 cases]. / Nocny ból glowy--opis 2 przypadków.

The pathogenesis of hypnic headache is still unknown. Some authors supposed that the genesis of hypnic disorder is a decrease of melatonin secretion. It is a rare, moderately severe headache that occurs in middle-aged or elderly patients and affects both sexes. It appears exclusively during sleep and often with alarm clock regularity. The attack lasts from two to 6 hours, it may be unilateral or diffuse, pulsating or boring, without autonomic system features. Case 1. A 49 year old man reported the history of two week nocturnal headache that awakened him every night from sleep. The headache lasted two hours. There were no autonomic system features. Case 2. A 52 year old man suffered from nocturnal headaches for 6 weeks. The pain occurred between 2 and 5 almost every night and woke the patient. He suffered from diabetes mellitus too. Both patients were treated with flunarizine with good results.

[A trial of progressive supranuclear palsy with carbamazepine]. / Próba zastosowania karbamazepiny w postepujacym porazeniu nadjadrowym.

We present a 61-year old patient with diagnosis of progressive supranuclear palsy. 2 years after the beginning of her complaints the diagnosis was based on the results of neurological examination. The ENG examination (among others vertical gaze paralysis) and the MR examination (2 of 3 criteria were met). The course of the disease was complicated by epilepsy and the treatment with carbamazepine was commenced. A significant improvement of her neurological and mental status was observed. The authors suggest checking this observation on larger number of patients with PSP.

[A case of asymptomatic central pontine myelinolysis]. / Przypadek asymptomatcznej centralnej mielinolizy mostu.

In 1959 year Adams, Victor and Mancella described central pontine myelinolysis (CPM) as a disease of fulminant course and fatal prognosis. In the typical cases progressive tetraparesis with bulbar signs results in severe crippledom or death. The disease was diagnosed before only by autopsy, as soon as the modern radiological technics i.e., CT and MRI were developed it is sometimes successfully diagnosed with patients alive. The authors present the case of 62-year old woman suffering from slight hemiparesis which made use to perform the CT examination. It showed central pontine lesion which was confirmed by MRI examination. The authors suggest that there are not only typical cases of CPM but also the asymptomatic ones as presented above.

[A case of multiple system atrophy treated with erythropoietin]. / Przypadek zaniku wieloukladowego leczony erytropoetyna.

The patient, a women aged 52, with a three-year history of treatment for arterial hypotension, was admitted to hospital because of progressing worsening of symptoms. The whole clinical syndrome made possible the recognition of multiple system atrophy as the cause of her symptoms. In view of failures in previous treatment it was decided to give her erythropoietin injections for four weeks. The treatment led to improvement of her status. The authors discuss the probable action mechanism of erythropoietin in this case.

The immunohistochemistry and ultrastructure of ganglioglioma with chromosomal alterations: a case report.

Ganglioglioma, together with its counterparts-ganglioneuroma and gangliocytoma are relatively uncommon neoplasms of the brain composed of neoplastic neurons (ganglion and ganglioid cells) and glial cells. We report here a case of ganglioglioma studied by electron microscopy. The case was further characterized by peculiar chromosomal alterations, 46,XX[6]/43,XX[1], der(1)t(1;5)(q21;q12), der(8;13)(q10;q10),-9,i(10)(q10). Routine light microscopy revealed mixed neuro-glial tumor composed of pilocytic astrocytes with abundant Rosenthal fibers and relatively numerous ganglion cells. The latter were immunoreactive with antibodies (Abs) against synaptophysin and neurofilament protein (NFP). Anti-NFP Abs also immunostained numerous distorted axons embedded in the tumor mass. Some of these showed bullous swellings and thus were analogous to dystrophic neurites or spheroids. Ganglion cells were characterized by abundant intracytoplasmic dense-core vesicles, absence of intermediate filaments and numerous microtubules. Occasionally a close apposition of ganglion cells and Rosenthal fibers were seen. Dense-cored vesicles were pleomorphic and ranged in diameter from small synaptic vesicles to large lysosome-like neurosecretory granules. The former occasionally formed characteristic dumbbell shapes. Neoplastic astrocytes were identical to those of other glial tumors of astrocytic lineage; numerous Rosenthal fibers were frequently seen.

[The level of Interleukin-6 in patients with amyotrophic lateral sclerosis]. / Poziom interleukiny--6 u chorych ze stwardnieniem zanikowym bocznym.

The role of immune factors in the pathogenesis of ALS is taken into account and, in view of this, it was decided to study the role of interleukins (IL) taking IL-6 as an example. Its concentration was determined in blood and CSF with Amersham RIA kits. The study was carried out on 16 ALS patients and 16 patients with low back pain who served as controls. The IL-6 level in blood and CSF did not vary between these groups statistically significantly. Then from the ALS group the cases with only bulbar symptoms or with predominance of bulbar symptoms were isolated. This subgroup comprised 6 patients, their mean age was higher, and disease duration was shorter. The IL-6 level in the CSF was 27.1 +/- 8.6 nmol/ml and was statistically significantly higher than in the remaining cases. The specificity of this finding is discussed.

Creutzfeldt-Jakob disease: ultrastructural study of brain biopsy: unusual interaction between astrocytes and oligo- and microglia.

We report a case of Creutzfeldt-Jakob (CJD) disease in a 72-year-old man without characteristic EEG alterations. Unusually abundant pleomorphic PrP-immunopositive amyloid plaques were found in the cerebellum. Despite of this similarity with Gerstmann-Straussler-Scheinker (GSS) disease the case cannot be classified as GSS lacking classical GSS 102 PRNP gene mutation. The ultrastructural findings were similar to those described previously for CJD. The close contact of reactive astrocytes with both oligodendroglia and microglia in this case suggests their possible functional interaction.

[Electronystagmographic examination in patients with amyotrophic lateral sclerosis (ALS)]. / Badanie elektronystagmograficzne u chorych z SLA.

18 patients suffering from ALS underwent ENG examinations. Motor function of eyeballs in respect of optic and vestibular stimulus was evaluated. The study demonstrated that 8 patients exhibited lesion of central part of the vestibular system and in 2 cases features of peripheral type of vestibular disturbances were found.

[Basilar migraine: case report]. / Przypadek migreny podstawnej.

The authors describe a case of basilar migraine in 35-year old woman. The diagnostic examinations performed in accordance with our expectations did not show abnormalities. Like other authors we think that the symptoms are caused by disturbances of vascularisation in basilar artery area.