Species identification of Borrelia burgdorferi sensu lato from tick and human isolates in Styria (Austria) by PCR-RFLP analysis.

Seventy-one isolates of Borrelia burgdorferi sensu lato (B.b.s.l.) derived from Ixodes ricinus ticks (50 strains) and patients (21 strains) were characterised by PCR-RLFP analysis. In four cases the human isolates were obtained from the cerebrospinal fluid (CSF) of patients with clinical symptoms of neuroborreliosis and in 17 cases from skin biopsies of patients with dermatological manifestation of Lyme borreliosis. Ixodes ricinus isolates originated from 14 localities in three regions (Mur valley, eastern and western Styria) in Styria. Thirty six strains of B.b.s.l. were isolated from nymphal ticks, nine strains from female and five strains from male ticks. Species identification of human isolates revealed three B. garinii and one B. afzelii isolates in CSF. In the PCR-RFLP analysis of 17 skin specimens a pattern for B. afzelii was found in ten cases, while six could be identified as B. garinii and one as a mixed infection of B. afzelii and B. garinii. Genetic characterisation of tick isolates resulted in 24 strains of B. afzelii (48%), 11 strains of B. garinii (40%) and 5 strains of B. burgdorferi s.st. (10%); one isolate showed a mixed infection of B. afzelii and B. garinii. Our findings indicate that B. afzelii and B. garinii predominate over B. burgdorferi s.str. in Ixodes ricinus ticks from Styria, which is similar to findings in neighbouring countries. This also reflects the occurrence of different pathogenic Borrelia strains in human samples.

[Cyclical vomiting as the leading symptom of regression]. / Zyklisches Erbrechen als Leitsymptom einer Regression.

A 17-year old boy with primary mental retardation of unknown aetiology and cyclic vomiting syndrome (CVS) is reported. Missing an organic cause of the episodes therapeutic procedures remained symptomatic for a 15 years time period. The report focusses the CVS as a regressive mechanism triggered by a mental retardation of the child which permanently disturbed the interaction between parents and child. Psychotherapeutic approach was successful in the following respects: The advantage by illness to vomit at home was continuously reduced by admitting the boy to a hospital at every ongoing attack. Hypnotherapeutic techniques were able to substitute the "malign" regression (CVS) successively by inducing a "benign" kind of regression. Family therapy could induce new and more intense non-verbal patterns of communication which where hitherto unknown in this family.

Neopterin concentrations in cerebrospinal fluid and serum as an aid in differentiating central nervous system and peripheral infections in children.

Neopterin is a sensitive indicator for cellular immune activation. Its concentrations were determined in cerebrospinal fluid (CSF) and serum specimens from 91 children with no evidence of central nervous system (CNS) or peripheral inflammations, 43 with definite neuroborreliosis, 51 with other CNS infections, and 33 with peripheral infections. The aim of our study was (a) to establish a range of normal CSF neopterin concentrations in control children, and (b) to inquire into the diagnostic potential of neopterin measurements in both body compartments for aiding in differential diagnosis of inflammatory vs noninflammatory diseases, and CNS vs peripheral inflammations. CSF neopterin concentrations in controls were invariably low (up to 9.3 nmol/L), but in children with neuroborreliosis and, even more so, with other CNS infections neopterin concentrations were significantly (P <0.0001) increased. Children with peripheral infections, however, rarely showed raised CSF neopterin concentrations. Serum concentrations of neopterin, on the other hand, were not significantly different between controls and children with neuroborreliosis. Although serum concentrations were significantly different between controls and children with other CNS infections, diagnostic efficiency was poor for this comparison. Peripheral infections, in contrast, were associated with significantly higher (P <0.0001) serum neopterin concentrations when compared with controls. A classification tree was constructed on the basis of CSF and serum neopterin concentrations, allowing with high accuracy the discrimination between controls, children with CNS infections, and children with peripheral infections. Thus, on the basis of a comparatively large control group, our data underline the diagnostic validity of neopterin as an aid in differential diagnosis of inflammatory vs noninflammatory diseases, and confirm that CSF neopterin concentrations are not correlated with serum neopterin concentrations, and, therefore, CSF neopterin appears to be produced intrathecally.

Acrodermatitis chronica atrophicans in an 11-year-old girl.

We report a case of acrodermatitis chronica atrophicans in an 11-year-old girl living in an area endemic for Lyme borreliosis. The diagnosis was first made on the basis of clinical, histopathological and serological findings. Moreover, Borrelia burgdorferi-specific DNA was amplified from lesional skin by polymerase chain reaction. Intravenous treatment with ceftriaxone (2 g once daily) for 2 weeks was initiated. The skin changes clearly responded to the therapy, and Borrelia burgdorferi-specific gene segments were no longer detectable by polymerase chain reaction. This is the first report of molecular-proven acrodermatitis chronica atrophicans in childhood. The occurrence of this late skin manifestation of Lyme borreliosis in children is reviewed.

beta-lactam antibiotics in the treatment of neuroborreliosis in children: preliminary results.

In vitro beta-lactam antibiotics like ceftriaxone and penicillin G sodium have been shown to be active against Borrelia burgdorferi. Results of quantitative determinations of both antibiotic substances in the CSF for children are limited. Seventy-five children (median age 96 months, range 10 to 176 months) with probable or definite neuroborreliosis were treated with ceftriaxone (1 x 50-90 mg/kg/day) or penicillin G sodium (4 x 80,000-120,000 IU/kg/day) intravenously. On day 10 of therapy levels of penicillin G sodium (1,1.5,2,3,4, 5, or 6 h after i.v. administration), and ceftriaxone (1,2,4,6,12 or 24 h after i.v. administration) in serum and CSF were measured with a micro agar diffusion bioassay. Results demonstrate that after 5 h penicillin G sodium in CSF was above the minimal inhibitory concentration (MIC) but after 6 h penicillin G sodium levels were below the determination limit in 60% of the cases. All ceftriaxone results in CSF-even after 24 h-were above MIC. Penicillin G sodium serum values ranged from 46.6 to 0.1 mg/L (1 to 6 h post dose) and ceftriaxone serum values from 261 to 5 mg/l (1 to 24 h post dose). The role of penicillin G sodium and ceftriaxone and administration intervals of both antibiotics in the therapy of neuroborreliosis in children are discussed.

Evaluation of the polymerase chain reaction for diagnosis of herpes simplex virus encephalitis.

Cerebrospinal fluid samples from 257 patients with suspected herpes simplex virus encephalitis were prospectively analyzed by herpes simplex virus polymerase chain reaction. The polymerase chain reaction indicated herpes simplex virus encephalitis in 9 serologically proven cases and in 14 additional patients. Increased polymerase chain reaction signals were observed together with more severe neurological symptoms (P < 0.01) and within the first days of acyclovir treatment (P < 0.05).

Lyme borreliosis of central nervous system (CNS) in children: a diagnostic challenge.

Within 24 months in a consecutive series of 84 children with neurological symptoms indicative of Lyme borreliosis of the central nervous system (CNS) 45 seronegative children (group III), 17 seropositive (group II), and 22 children with specific Borrelia burgdorferi results in cerebrospinal fluid (CSF)-i.e. B. burgdorferi antibodies and/or intrathecally produced B. burgdorferi antibodies and/or positive B. burgdorferi culture in CSF were observed. The results show that intrathecally produced B. burgdorferi antibodies are the most important marker for the diagnosis of neuroborreliosis (with 71.4% positives) and B. burgdorferi cultivation directly from CSF may be successful in the earliest phase of the disease. Since each of the specific CSF parameters may be false negative in some cases, a careful synopsis of laboratory parameters was done. It shows that CSF protein and CSF cell values are higher in group I than in II or III. Neither can seronegativity exclude nor can seropositivity confirm the diagnosis of neuroborreliosis as in only 71% of group I serum B. burgdorferi antibodies were detected. In view of these aspects clinical and laboratory results are discussed.

Penicillin G sodium and ceftriaxone in the treatment of neuroborreliosis in children--a prospective study.

A controlled clinical study was set up to examine whether penicillin G sodium (PG) or ceftriaxone (C) is superior in the treatment of acute neuroborreliosis in childhood. Within a time period of 18 months 77 children with symptoms indicative of Lyme borreliosis of the central nervous system (CNS) were seen. In 23 of these children Borrelia burgdorferi specific cerebrospinal fluid (CSF) parameters confirmed the diagnosis of a neuroborreliosis. These children were treated at random with intravenous (i.v.) PG 400,000-500,000 I.U./kg/day for 14 days (group I) or with i.v. ceftriaxone 75-93 mg/kg/day for 14 days (group II), respectively. Clinical examination and a set of diagnostic laboratory parameters were done at admission, right after therapy, three, six and partly 12 months after therapy. The general condition of all children in both groups improved dramatically during antibiotic therapy, and no relapse occurred within the observation period. Considering the clear and comparable decrease of B. burgdorferi serum titres and the clinical outcome (duration of disease and follow-up for at least six months) in children of both groups no difference between both antibiotic drugs can be demonstrated.

Preliminary characterization of Borrelia burgdorferi CSF isolates.

Borrelia burgdorferi was cultivated from three cerebrospinal fluid (CSF) samples of children (aged three and a half, four and a half and eight years) who were admitted to the hospital because of acute facial palsy, aseptic meningitis, and aseptic meningitis plus facial palsy. CSF was taken on day one in two cases and on day two in the remaining case after onset of symptoms. All three strains showed a very similar SDS-PAGE pattern, without an OspB and 20kD band. However, of nine monoclonal antibodies (Moab) raised against B. burgdorferi B31, the Moab H5332 recognized two strains, one of them very weakly, and the flagella specific Moabs H9724, H605, and H6TS (less intensively) recognized all strains. This preliminary characterization reveals heterogeneity among CSF Borrelia isolates of cases from a very close geographic area.

Mitochondrial encephalomyopathy--two years follow-up by MRI.

A case of mitochondrial encephalomyopathy (MEM) followed-up by MRI for 2 1/2 y is presented. MRI showed gross, diffusely distributed white matter lesions in both hemispheres predominantly in frontal, parietal, temporal and occipital locations a marked ventriculomegaly indicative of cerebral atrophy. Except a slight increase of the cerebral atrophy there were no changes in the follow-up examinations. There are no specific MRI findings in MEM, the diagnosis is established by the synopsis of MRI, laboratory data and muscle biopsy.

Whistling face syndrome. A case report and literature review.

The cranio-carpo-tarsal or "whistling face" syndrome was first described by Freeman and Sheldon in 1938. More than 60 cases with great variability of expression are known till now and autosomal dominant as well as recessive inheritance and sporadic cases suggest a genetic heterogeneity. We review 60 well-documented cases of the literature and present a patient with a severe form, who died of bronchopneumonia at the age of 9 months. The facial stigmata of his mother and the ulnar deviations of his maternal grandfather support the autosomal inheritance of the syndrome.

Multiple sclerosis in childhood: contribution of serial MRI to earlier diagnosis.

The authors report six children (five girls, one boy) aged 11 to 13 years, of whom four had clinically definite multiple sclerosis (MS) and two had laboratory-supported definite MS. All had brain white matter abnormalities indicative of MS. In three cases, positive findings on the first MRI contributed significantly to their early diagnosis. Follow-up MRI studies over an average period of five months detected morphological changes in three of the children, although there was no concomitant clinical evidence. This raises the question of whether changes in clinically 'silent' lesions on follow-up MRI are antecedents of the essential MS criterion of dissemination over time, which could lead to earlier diagnosis of childhood MS. With cranial computerized tomography (CT) during the first clinical attack, a large focus with a lamellar structure mimicked a brain tumour in two patients. As CT also misses additional small lesions, it should no longer be used as the primary diagnostic method.

Multiple sclerosis in children: value of serial MR studies to monitor patients.

A series of six children with clinical (4) and laboratory (2) evidence of multiple sclerosis is described. The mean age at onset was 12 years and the female-male ratio was 5:1. All patients had white matter abnormalities on initial MR scans. On follow-up MR studies, performed every 3 to 5 months, all children exhibited changing patterns of CNS signal abnormalities. In three cases, clinically silent brain lesions were detected. In four patients with an acute clinical attack, large lesions were present, demonstrating a lamellar structure on T1- and T2-weighted images. The lesions were seen best on long TR/short TE spin-echo sequences. Combined sagittal and axial series with EKG gating and flow-compensation technique were best for MR follow-up studies. Our results show that MR is useful for monitoring patients with multiple sclerosis.