H J Christen

Georg-August-Universität Göttingen, Göttingen, Lower Saxony, Germany

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Publications (89)

  • H.-I. Huppertz · H.-J. Christen · V. Fingerle · U. Heininger
    Chapter · Jan 2013
  • Article · Mar 2011 · Neuropediatrics
  • B Assmann · GF Hoffmann · HJ Christen · [...] · P Gissen
    Article · Aug 2010 · Neuropediatrics
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    Full-text Book · Jan 2009
  • Chapter · Dec 2007
  • H. Hartmann · K. Brockmann · J. Riedel · [...] · H.-J. Christen
    Article · Sep 2007 · European Journal of Paediatric Neurology
  • [Show abstract] [Hide abstract] ABSTRACT: Alexander disease is a rare disorder of cerebral white matter due to a dysfunction of astrocytes. The most common infantile form presents as a megalencephalic leukodystrophy. Mutations of the GFAP gene, encoding Glial Fibrillary Acidic Protein, have been recognized as the cause of Alexander disease. Glial Fibrillary Acidic Protein is the major intermediate filament protein in astrocytes, its functional rod domain is conserved in sequence and structure among other intermediate filament proteins. We report here two cases of infantile Alexander disease with early onset and severe course, caused by DE NOVO mutations A364 V and Y366C. Both affected GFAP residues are part of a highly conserved coiled-coil trigger motif in the C-terminal end of segment 2B, probably required for the stability of intermediate filament molecules. Comparable effects are seen with mutations of the corresponding residues of the gene coding for keratin 14, another intermediate filament, this further supports the hypothesis that these positions of the trigger motif are generally critical for a normal function of intermediate filaments.
    Article · Jul 2007 · Neuropediatrics
  • [Show abstract] [Hide abstract] ABSTRACT: Definition. Durch Staphylokokken verursachte Krankheiten können lokal begrenzt bleiben, generalisiert auftreten oder sich als Intoxikation äußern. Die frühere Einteilung der Staphylokokken nach der Produktion eines gelben Pigmentes in S. aureus und S. albus ist weitgehend verlassen worden. Wegen der Korrelation mit klinischen Krankheitsbildern hat sich dagegen die Einteilung der Staphylokokken in koagulasepositive (KPS) und koagulasenegative Staphylokokken (KNS) bewährt.
    Chapter · Jan 2007
  • Article · Jan 2004 · Aktuelle Neurologie
  • H.-J. Christen · H. Eiffert
    [Show abstract] [Hide abstract] ABSTRACT: Die Lyme-Borreliose ist die hufigste durch Zecken bertragene Infektionskrankheit. Ihre Entdeckung vor 25Jahren bildete einen Meilenstein in der modernen Infektiologie. Kinder sind hufiger als Erwachsene von Zeckenstichen betroffen und tragen damit ein hheres Infektionsrisiko. Als Multisystemerkrankung manifestiert sich die Lyme-Borreliose bei 75% der Flle mit Hautsymptomen, das Zentralnervensystem und die Gelenke sind deutlich seltener betroffen. Die akute periphere Fazialisparese und die serse Meningitis prgen das klinische Bild der Neuroborreliose im Kindesalter. Lyme-Borreliose ist eine klinische Diagnose, bei der Anamnese, klinischer Befund und Antikrperbefunde gleichberechtigt gewrdigt werden. Die spezifische Antikrperdiagnostik ist mit Sensitivitts- und Spezifittsproblemen behaftet, was das Risiko der berdiagnose und -therapie beinhaltet. Speziell bei der Neuroborreliose ist der Liquorbefund wichtig: Eine Liquorpleozytose gilt als Conditio sine qua non fr die Diagnose. Die Prognose der Lyme-Borreliose ist umso gnstiger, je frhzeitiger antibiotisch behandelt wird. Gesicherte Langzeitfolgen einer Neuroborreliose sind bei Kindern nicht bekannt, knnen aber noch nicht gnzlich ausgeschlossen werden.Lyme borreliosis is the most frequent tick-borne disorder. Its discovery 25years ago was a milestone in the field of infectious diseases. Children are especially prone to tick bites and therefore at special risk for Lyme borreliosis. Lyme borreliosis is a multisystem disorder which affects the skin in three-fourths of the cases (mainly erythema migrans) and the central nervous system and the joints clearly less frequently. In childhood acute peripheral facial nerve palsy and aseptic meningitis dominate the clinical spectrum of neuroborreliosis. Lyme borreliosis is a clinical diagnosis which is established on the basis of the history, the clinical findings, and the antibody results. Specific antibody diagnosis is complicated by the problem of insufficient sensitivity and specificity. The consequence is the risk of overdiagnosis and overtreatment. In neuroborreliosis the CSF findings are especially helpful. CSF pleocytosis is mandatory for the diagnosis of neuroborreliosis. Early and effective antibiotic treatment guarantees a good prognosis of Lyme borreliosis with quick resolvement of symptoms. Long-term sequelae of neuroborreliosis have not been proved in children, but cannot yet be excluded.
    Article · Jan 2003 · Monatsschrift Kinderheilkunde
  • T. Lücke · H.J. Christen · N. Kanzelmeyer · [...] · A.M. Das
    [Show abstract] [Hide abstract] ABSTRACT: The 3 main clinical features of immuno-osseous-dysplasia Schimke (IODS) are spondyloepiphyseal dysplasia, nephropathy and immunopathy. Neurologic symptoms are found in >50% of patients with IODS. Headache, double vision, aphasia, hemiparaesthesia and hemiparesis were reported and transitory ischaemic attacks as well as cerebral infarctions on the basis of early arteriosclerosis were shown to cause these symptoms. Despite successful kidney-transplantation neurologic symptoms may occur, while vaso-occlusive disease does not reoccur in the transplanted organ. Presence of neurologic symptoms in IODS seems to correlate with the severity of the disease. A phenotype- genotype-correlation seems to exist. Patients with a more severe course of the illness show nonsense-, frame-shift- or splicing-mutations on both alleles of the SMARCAL 1 gene, while mildly affected patients show missense mutations on each allele. An effective therapy of IODS or of the neurologic symptoms in IODS is missing. The underlying pathophysiology of the disease is unclear.
    Article · Jan 2003 · Nervenheilkunde
  • P Huppke · C Roth · H J Christen · [...] · F Hanefeld
    [Show abstract] [Hide abstract] ABSTRACT: To determine whether primary or secondary growth hormone (GH) deficiency has a causative role in linear growth retardation, a key feature in Rett syndrome (RTT). In 38 patients with Rett syndrome a variable set of investigations was performed including assays of growth and thyroid hormones, gonadotropins, gonadal and adrenal steroids and determination of bone age. Not all measurements were attainable from all patients. In three patients the 24-h growth hormone secretion profile was evaluated using the pulsar method. The bone age determined in 24 patients was found to be normal in 8, retarded in 9 and accelerated in 7 patients. Insulin-like growth factor (IGF)-1 was low in 8 out of 23 patients. IGF-binding protein (IGFBP)-3 and insulin and arginine-stimulated growth hormone secretion were both normal, indicating normal GH secretion in the majority of patients. The 24-h GH secretion profile in the first patient showed a normal day/night rhythm and a normal increase in nocturnal GH secretion. The second patient's overall GH secretion was normal but there was no day/night rhythm. The third patient showed borderline low GH secretion. Normal age-appropriate plasma values were found for the thyroid hormones (T4, TSH), TSH-night rhythm, oestradiol, prolactin and cortisol (08.00, 18.00). Our study provides no evidence that growth retardation in RTT is caused by growth hormone deficiency. A disturbed hypothalamic control cannot be excluded but it is unlikely that this is the major cause of growth retardation in RTT.
    Article · Dec 2001 · Acta Paediatrica
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    Full-text Article · Jun 2001 · Monatsschrift Kinderheilkunde
  • [Show abstract] [Hide abstract] ABSTRACT: Infektionskrankheiten unterscheiden sich von allen anderen Krankheiten dadurch, daß sie nach einem Kontakt aus der Auseinandersetzung zweier Lebewesen entstehen — dem Menschen („Wirt“) auf der einen und dem Mikroorganismus („ungebetener Gast“) auf der anderen Seite. Verschiedene Fachdisziplinen haben im Lauf des letzten Jahrhunderts die 3 genannten Aspekte — Mikroorganismen, Makroorganismen und Kontakte — erforscht und sich dabei voneinander abgegrenzt. Im Laborbereich beschäftigt sich die medizinische Mikrobiologie mit den Mikroorganismen, die Immunologie mit den Makroorganismen. Die Hygiene untersucht die Kontaktmuster zwischen (ungebetenem) Gast und Wirt mit dem Ziel, Methoden zu entwickeln, die eine Übertragung des Erregers auf den Wirt verhindern. Daraus hat sich v. a. in den angelsächsischen Ländern die Infektionsepidemiologie entwickelt, welche die Regeln der Verbreitung von Infektionskrankheiten und ihren Erregern erforscht. Infektiologen haben spezielle Kenntnisse auf jedem der 4 vorgenannten Fachgebiete und betreuen als Ärzte für Kinderheilkunde oder innere Medizin eigenverantwortlich Patienten mit Infektionskrankheiten.
    Chapter · Jan 2001
  • D. Hobusch · H.-J. Christen · H.-I. Huppertz · R. Noack
    [Show abstract] [Hide abstract] ABSTRACT: Lyme borreliosis is the most frequent tick-borne disease of man in the northern hemisphere. A variety of systems may be involved. The most frequent manifestations in childhood include erythema migrans, meningitis, cranial nerve palsy and arthritis. Erythema migrans is usually easily recognised and determination of antibodies to Borrelia burgdorferi should not be performed. Childhood neuroborreliosis is characterised mostly by aseptic meningitis with or without cranial nerve palsy, in most cases facial palsy. Basic CSF findings often show a combined evidence of lymphocytic pleocytosis, IgM-class dominance in intrathecal humoral immun response, and blood-CSF barrier dysfunction. Calculation of the Borrelia burgdorferi specific IgM antibody index (according to REIBER) proved to be the most sensitive method for detecting intrathecal synthesis of specific antibodies. Lyme arthritis presents initially as episodic oligo-arthritis, mostly involving the knee joint, and may turn into chronic monoarthritis of the knee; usually high titers of IgG antibodies to Borrelia burgdorferi are found. The rarer manifestations such as encephalomyelitis, chronic arthritis, carditis and inflammatory eye disease may be difficult to diagnose due to clinical ambiguity and problems in the interpretation of serological results. Antibodies to Borrelia burgdorferi found by sensitive ELISA must always be confirmed by immunoblot analysis, but sometimes immunoblot analysis is more sensitive than ELISA. Treatment is by antibiotics, amoxicillin or doxyciclin for erythema migrans, and i.v. third generation cephalosporins for all other manifestations. Even after successful antibiotic therapy, antibodies may persist for months and years and no further antibiotic treatment is necessary in the absence of attributable clinical manifestations. The differentiation between a persisting immune response and a persisting infection therefore has to be based upon the clinical symptoms, non-specific laboratory data and the development of the antibody titers.
    Article · Jan 2001 · Tagliche Praxis
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    M Wilke · H Eiffert · H J Christen · F Hanefeld
    [Show abstract] [Hide abstract] ABSTRACT: As part of an ongoing study aiming to define the clinical spectrum of neuroborreliosis in childhood, we have identified four patients with unusual clinical manifestations. Two patients suffered from a primarily chronic form of neuroborreliosis and displayed only non-specific symptoms. An 11 year old boy presented with long standing symptoms of severe weight loss and chronic headache, while the other patient had pre-existing mental and motor retardation and developed seizures and failure to thrive. Two further children who presented with acute hemiparesis as a result of cerebral ischaemic infarction had a cerebrovascular course of neuroborreliosis. One was a 15 year old girl; the other, a 5 year old boy, is to our knowledge the youngest patient described with this course of illness. Following adequate antibiotic treatment, all patients showed substantial improvement of their respective symptoms. Laboratory and magnetic resonance imaging findings as well as clinical course are discussed and the relevant literature is reviewed.
    Full-text Article · Aug 2000 · Archives of Disease in Childhood
  • U. Heininger · H. J. Christen
    Article · Jun 2000 · Monatsschrift Kinderheilkunde
  • [Show abstract] [Hide abstract] ABSTRACT: Rhabdoid tumors of the central nervous system are rare malignancies with a still almost uniformly fatal outcome. There is still no proven curative therapy available. We report our experience with nine patients with central nervous system rhabdoid tumors. Gross complete surgical removal of the tumor was achieved in six patients. Seven patients received intensive chemotherapy. Four of these were treated in addition with both neuroaxis radiotherapy and a local boost directed to the tumor region, while two patients received local radiotherapy only. The therapy was reasonably well tolerated in most cases. Despite the aggressive therapy, eight of the nine patients died from progressive tumor disease, and one patient died from hemorrhagic brain stem lesions of unknown etiology. The mean survival time was 10 months after diagnosis. Conventional treatment, although aggressive, cannot change the fatal prognosis of central nervous system rhabdoid tumors. As these neoplasms are so rare, a coordinated register would probably be a good idea, offering a means of learning more about the tumor's biology and possible strategies of treatment.
    Article · May 2000 · Child s Nervous System
  • H J Christen · F Hanefeld · E Kruse · [...] · M Finkenstaedt
    [Show abstract] [Hide abstract] ABSTRACT: Foix-Chavany-Marie syndrome (FCMS) is a distinct clinical picture of suprabulbar (pseudobulbar) palsy due to bilateral anterior opercular lesions. Symptoms include anarthria/severe dysarthria and loss of voluntary muscular functions of the face and tongue, and problems with mastication and swallowing with preservation of reflex and autonomic functions. FCMS may be congenital or acquired as well as persistent or intermittent. The aetiology is heterogeneous; vascular events in adulthood, nearly exclusively affecting adults who experience multiple subsequent strokes; CNS infections; bilateral dysgenesis of the perisylvian region; and epileptic disorders. Of the six cases reported here, three children had FCMS as the result of meningoencephalitis, two children had FCMS due to a congenital bilateral perisylvian syndrome, and one child had intermittent FCMS due to an atypical benign partial epilepsy with partial status epilepticus. The congenital dysgenetic type of FCMS and its functional epileptogenic variant share clinical and EEG features suggesting a common pathogenesis. Consequently, an increased vulnerability of the perisylvian region to adverse events in utero is discussed. In honour of Worster-Drought, who described the clinical entity in children 40 years ago, the term Worster-Drought syndrome is proposed for this unique disorder in children.
    Article · Mar 2000 · Developmental Medicine & Child Neurology
  • P Huppke · H J Christen · B Sattler · F Hanefeld
    [Show abstract] [Hide abstract] ABSTRACT: We report two brothers with mental retardation, lymphoedema of the limbs and facial anomalies. Hennekam et al. (Am J Med Genet 34:593-600; 1989) described four patients with identical signs and intestinal lymphangiectasia. To confirm the diagnosis of Hennekam syndrome we undertook a duodenal biopsy from the older brother which revealed intestinal lymphangiectasia. So far only one patient with Hennekam syndrome and cerebral abnormalities has been described. This patient presented with pachygyria in the parietal area. Cerebral MRI in our two cases revealed small subcortical hyperintensities in both patients and a large cystic lesion in the younger patient probably representing an old media infarction.
    Article · Feb 2000 · Clinical Dysmorphology