Frequency of an intrathecal IgM synthesis and MRZ reaction in children with MS.

BACKGROUND: Multiple sclerosis (MS) is a chronic inflammatory and demyelinating disease of the CNS. An intrathecal IgM synthesis is associated with a more rapid progression of MS and the intrathecal immune response to measles -, rubella -and varicella zoster virus (MRZR) which, if present, increases the likelihood of a diagnosis of MS in adults. OBJECTIVE: To evaluate the frequency of an intrathecal IgM synthesis and MRZR in children with MS. MethodsChildren with MS and a data set including clinical and treatment history, MRI at onset, in addition to a CSF analysis, and determination of antibody index (AI) of measles, rubella, and zoster antibodies, were eligible. The presence of an intrathecal IgM synthesis and/or a positive MRZ reaction were compared to biomarkers of a more progressive disease course. RESULTS: In 75 children with MS, OCBs were present in 93.3 %). 49,2 % experienced their first relapse within 6 months. 50.7 % had a total lesion load of more than 10 lesions in the first brain MRI. Spinal lesions were identified in 64 %. 23.5 % had a positive MRZR and 40.3 % an intrathecal IgM synthesis. No significant associations were detected between the presence of an intrathecal IgM synthesis and MRZR and parameters including the relapse rate in the first two years. CONCLUSION: An intrathecal IgM synthesis and a positive MRZR are found in a subset of MS children but are not associated with markers associated with a poor prognosis.

Manifestation of migraine in adolescents: Does it change in puberty?

PURPOSE: To analyze the association between pubertal stage, menstrual cycle and migraine attacks in girls with migraine. In addition, headache frequency, accompanying symptoms, duration and onset in relation to the specific phase of the cycle were investigated. METHODS: Girls between 7 and 18 years old, diagnosed with headaches that met "International Classification of Headache Disorders II" diagnostic criteria for migraine without aura, kept a daily headache and menstrual cycle diary over 8 weeks. Ovulatory cycles were identified by weekly progesterone saliva tests. RESULTS: 47 girls participated in the study and were divided into three groups according to Tanner stage and onset of regular menstruation: pre- (n = 16), peri- (n = 19) and post-pubertal (n = 12). A significant difference in migraine frequency was found between pre- and post-pubertal girls (p = 0.005). No significant differences with regard to headache characteristics were detected. Interestingly, a higher frequency of attacks in follicular phase occurred compared to luteal phase in peri- and post-pubertal girls (p = 0.030). CONCLUSION: During puberty, migraine patterns in girls change to a typical adult pattern of migraine in a stepwise manner not clearly related to menarche. The first sign of this transition phase could be the higher frequency of migraine attacks in post-pubertal girls.

Diagnostic value of cerebrospinal fluid CXCL13 for acute Lyme neuroborreliosis. A systematic review and meta-analysis.

OBJECTIVES: The utility of cerebrospinal fluid (CSF) CXCL13 for diagnosis of acute Lyme neuroborreliosis (LNB) has been debated and the test is not yet routinely performed. This study's aim was to evaluate its overall diagnostic accuracy through meta-analysis. METHODS: Electronic searches in PubMed MEDLINE and Web of Science were performed to identify relevant articles published before January 2018. A summary receiver operating characteristic curve and an optimal cut-off were estimated modelling multiple cut-offs. Publication bias was evaluated using a funnel plot and the associated regression test. RESULTS: A total of 18 studies involving 618 individuals with acute LNB and 2326 individuals with other neurological disorders meeting the eligibility criteria were identified. The pooled sensitivity for CSF CXCL13 was 89% (95% CI 85%-93%) and the pooled specificity was 96% (95% CI 92%-98%), using the identified optimal cut-off value of 162 pg/mL. There was marked heterogeneity between studies, caused by differences in the designs of the study populations and age distribution. The optimal cut-off in the seven studies with a cross-sectional design was 91 pg/mL (sensitivity 96%, 95% CI 92%-98%; specificity 94%, 95% CI 86%-97%) and in the 11 case-control studies it was 164 pg/mL (sensitivity 85%, 95% CI 78%-91%; specificity 95%, 95% CI 90%-98%). CSF CXCL13 values above the optimal cut-off level (determined in this meta-analysis) were also detectable in some other central nervous system disorders, namely neurosyphilis and central nervous system lymphoma. CONCLUSIONS: Our meta-analysis shows that CSF CXCL13 has the potential to become a useful adjunct in the diagnosis of acute LNB.

The safety of paediatric dentistry procedures under general anaesthesia. A five-year experience of a tertiary care center.

AIM: Frequently general anaesthesia (GA) is used to treat noncompliant children. Especially in children with morbid diseases general anaesthesia can be a challenging procedure for anaesthetists. The aim of this paper was to evaluate the risks and adverse reactions with a special focus on the impact of existing medication conditions and syndromes. MATERIALS AND METHODS: and methods Records of children up to 10 years of age, who were admitted for paediatric dentistry procedures under GA from January 2011 to December 2016 at the University Hospital of the University of Aachen (Germany), were reviewed. A special attention was paid to the intra- and perioperative critical adverse reactions and concomitant systemic conditions and their impact on treatment outcome. RESULTS: Two hundred and twenty patients were admitted for dental restorations. Critical adverse reactions occurred in 4% of the treated patients and they were statistically significantly (p=0.004) related to the ASA classification above II. The use of a laryngeal mask airway was significantly associated (p<0.001) with a shorter duration of surgery. Most common concomitant medical conditions were congenital heart disease, mental retardation and inherited syndromes. CONCLUSION: Although the administration of general anaesthesia in infants and children can be regarded as a safe procedure, clinically significant adverse reactions can occur, especially in patients with an existing medical condition.

Chemokine levels and parasite- and allergen-specific antibody responses in children and adults with severe or uncomplicated Plasmodium falciparum malaria.

Chemokine and antibody response profiles were investigated in children and adults with severe or uncomplicated Plasmodium falciparum malaria; the aim was to reveal which profiles are associated with severe disease, as often seen in nonimmune children, or with mild and uncomplicated disease, as seen in semi-immune adults. Blood samples were obtained from children under 5 years of age as well as adults with falciparum malaria. Classification of malaria was performed according to parasite densities and hemoglobin concentrations. Plasma levels of chemokines (IL-8, IP-10, MCP-4, TARC, PARC, MIP-1δ, eotaxins) were quantified, and antibody responses (IgE, IgG1, and IgG4) to P. falciparum, Entamoeba histolytica-specific antigen, and mite allergen extracts were determined. In children with severe malaria proinflammatory, IL-8, IP10, MIP-1δ, and LARC were at highly elevated levels, suggesting an association with severe disease. In contrast, the Th2-type chemokines TARC, PARC, and eotaxin-2 attained in children the same levels as in adults suggesting the evolution of immune regulatory components. In children with severe malaria, an elevated IgG1 and IgE reactivity to mite allergens and intestinal protozoan parasites was observed. In conclusion, exacerbated proinflammatory chemokines together with IgE responses to mite allergens or E. histolytica-specific antigen extract were observed in children with severe falciparum malaria.

Clinical and neuroradiological differences of paediatric acute disseminating encephalomyelitis with and without antibodies to the myelin oligodendrocyte glycoprotein.

BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG) antibodies have been recently described in children with acute disseminating encephalomyelitis (ADEM), but the clinical and neuroradiological characterisation of this subgroup is lacking. OBJECTIVE: To compare the clinical and neuroradiological features of paediatric ADEM with and without MOG antibodies. METHODS: Clinical course, cerebrospinal fluid (CSF)-, MRI studies, outcome and MOG status of 33 paediatric ADEM prospectively studied were reviewed. RESULTS: MOG antibodies (median 1:2560; range 1:160-1:20 480) were detected in 19 children with ADEM. The majority of children showed a decline of serum MOG-IgG titres over time. Children with MOG antibodies did not differ in their age at presentation, sex ratio, the presence of oligoclonal bands, clinical symptoms or initial severity, apart from a higher CSF cell count (p=0.038), compared with children without MOG antibodies. In addition, further relapsing demyelinating episodes associated with MOG antibodies were observed only in children with MOG antibodies. All 19 children with MOG antibodies had a uniform MRI pattern, characterised by large, hazy and bilateral lesions and the absence of atypical MRI features (eg, mainly small lesions, well-defined lesions), which was significantly different compared to that of children without MOG antibodies (p=0.003; and p=0.032, respectively). In addition, children with MOG antibodies had involvement of more anatomical areas (p=0.035) including the myelon characterised by a longitudinally extensive transverse myelitis (p=0.003), more often a complete resolution of lesions (p=0.036) and a better outcome (p=0.038). CONCLUSIONS: Patients with ADEM with MOG antibodies in our cohort had a uniform MRI characterised by large, bilateral and widespread lesions with an increased frequency of longitudinal extensive transverse myelitis and a favourable clinical outcome in contrast to children lacking MOG antibodies.

[CXCL13: a biomarker for acute Lyme neuroborreliosis: investigation of the predictive value in the clinical routine]. / CXCL13 als Biomarker der akuten Neuroborreliose : Überprüfung des prädiktiven Wertes in der klinischen Routine.

BACKGROUND: The level of CXCL13 is a cerebrospinal fluid (CSF) biomarker for acute Lyme neuroborreliosis (LNB) with a high sensitivity. As the concentration rapidly declines during antibiotic therapy CXCL13 can also be used as a follow-up parameter. However, CXCL13 is not yet in use as a routine parameter due to concerns about the specificity. OBJECTIVES: The sensitivity, specificity and predictive value of CXCL13 in the clinical routine work-up of suspected LNB was analyzed. MATERIAL AND METHODS: Since July 2010 the CSF of all patients (n = 204) with suspected acute LNB was not only analyzed for the routine parameters (i.e. pleocytosis and intrathecal production of Borrelia-specific antibodies, AI) but also for CXCL13. In cases of incongruent findings, a follow-up puncture after antibiotic therapy was carried out. The cut-off level for acute LNB was set at 250 pg/ml. RESULTS: This study included 179 patients who were not pretreated with antibiotics. Of these patients 15 suffered from definite LNB, 3 had a probable LNB and all had a CXCL13 value above the cut-off level. Only 2 of the 161 patients with a non-LNB diagnosis (both with a lymphoma) had a CXCL13 value in the CSF higher than 250 pg/ml. Especially noteworthy were two patients without pleocytosis in the CSF but with CXCL13 levels above the cut-off level in whom LNB could be confirmed in the follow-up CSF analysis. CONCLUSIONS: The biomarker CXCL13 has a higher sensitivity (100 % vs. 87 %) with a specificity (99 %) comparable with the established diagnostic markers for LNB, e.g. CSF pleocytosis and Borrelia-AI in the investigated patient population. The negative predictive value of CXCL13 is 100 %. Therefore, a normal CXCL13 level virtually excludes LNB. In the clinical routine CXCL13 is a valuable and practical diagnostic marker for LNB and can even detect an acute LNB in patients without CSF pleocytosis.

Generation of coherent 19- and 38-nm radiation at a free-electron laser directly seeded at 38 nm.

Initiating the gain process in a free-electron laser (FEL) from an external highly coherent source of radiation is a promising way to improve the pulse properties such as temporal coherence and synchronization performance in time-resolved pump-probe experiments at FEL facilities, but this so-called "seeding" suffers from the lack of adequate sources at short wavelengths. We report on the first successful seeding at a wavelength as short as 38.2 nm, resulting in GW-level, coherent FEL radiation pulses at this wavelength as well as significant second harmonic emission at 19.1 nm. The external seed pulses are about 1 order of magnitude shorter compared to previous experiments allowing an ultimate time resolution for the investigation of dynamic processes enabling breakthroughs in ultrafast science with FELs. The seeding pulse is the 21st harmonic of an 800-nm, 15-fs (rms) laser pulse generated in an argon medium. Methods for finding the overlap of seed pulses with electron bunches in spatial, longitudinal, and spectral dimensions are discussed and results are presented. The experiment was conducted at FLASH, the FEL user facility at DESY in Hamburg, Germany.

Proinflammatory and regulatory cytokines and chemokines in infants with uncomplicated and severe Plasmodium falciparum malaria.

Cytokine and chemokine levels were studied in infants (<5 years) with uncomplicated (MM) and severe malaria tropica (SM), and in Plasmodium falciparum infection-free controls (NEG). Cytokine plasma levels of interleukin (IL)-10, IL-13, IL-31 and IL-33 were strongly elevated in MM and SM compared to NEG (P<0·0001). Inversely, plasma concentrations of IL-27 were highest in NEG infants, lower in MM cases and lowest in those with SM (P<0·0001, NEG compared to MM and SM). The levels of the chemokines macrophage inflammatory protein (MIP3)-α/C-C ligand 20 (CCL20), monokine induced by gamma interferon (MIG)/CXCL9 and CXCL16 were enhanced in those with MM and SM (P<0·0001 compared to NEG), and MIP3-α/CCL20 and MIG/CXCL9 were correlated positively with parasite density, while that of IL-27 were correlated negatively. The levels of 6Ckine/CCL21 were similar in NEG, MM and SM. At 48-60 h post-anti-malaria treatment, the plasma concentrations of IL-10, IL-13, MIG/CXCL9, CXCL16 and MIP3-α/CCL20 were clearly diminished compared to before treatment, while IL-17F, IL-27, IL-31 and IL-33 remained unchanged. In summary, elevated levels of proinflammatory and regulatory cytokines and chemokines were generated in infants during and after acute malaria tropica. The proinflammatory type cytokines IL-31 and IL-33 were enhanced strongly while regulatory IL-27 was diminished in those with severe malaria. Similarly, MIP3-α/CCL20 and CXCL16, which may promote leucocyte migration into brain parenchyma, displayed increased levels, while CCL21, which mediates immune surveillance in central nervous system tissues, remained unchanged. The observed cytokine and chemokine production profiles and their dynamics may prove useful in evaluating either the progression or the regression of malarial disease.

Spin precession and alternating spin polarization in spin-3/2 hole systems.

The spin density matrix for spin-3/2 hole systems can be decomposed into a sequence of multipoles which has important higher-order contributions beyond the ones known for electron systems [R. Winkler, Phys. Rev. B 70, 125301 (2004)]. We show here that the hole spin polarization and the higher-order multipoles can precess due to the spin-orbit coupling in the valence band, yet in the absence of external or effective magnetic fields. Hole spin precession is important in the context of spin relaxation and offers the possibility of new device applications. We discuss this precession in the context of recent experiments and suggest a related experimental setup in which hole spin precession gives rise to an alternating spin polarization.

[Comparison of open versus percutaneous treatment for humeral head fracture]. / Vergleich der offenen vs. perkutanen Versorgung der Oberarmkopffraktur.

BACKGROUND: The hypothesis of this study was that percutaneous techniques lower the risk of post-traumatic avascular necrosis. MATERIALS AND METHODS: In this retrospective study 83 patients were followed up clinically and radiologically for signs of avascular necrosis and nonunion after open or percutaneous treatment of proximal humerus fractures. Mean age was 50 years. Fractures were classified in 22 patients (26.5%) as two part, in 21 patients (25.3%) as three part, in 39 patients (47%) as four part, and in 1 patient (1.2%) as fracture dislocation (Neer classification). Fractures were treated in 12 patients (14.5%) by ORIF (open reduction and internal fixation) and in 71 patients (85.5%) by CRPF (closed reduction and percutaneous fixation). Both groups were statistically equally distributed according to fracture type (Mann-Whitney U, p=0.267) and age (One-way-Annova, p=0.740). The postoperative regime did not differ between the two groups. RESULTS: Patients suffered significantly more avascular necrosis after open treatment [five patients (50%) versus eight patients (12.7%) in the percutaneous group, Mann-Whitney, p=0.004]. The risk for avascular necrosis and nonunion increased with age. Mean age of patients with avascular necrosis was 57 years, and the age of patients with nonunion was 67 years. CONCLUSION: Percutaneous treatment of humeral head fractures seems to be a reliable method for lowering the risk of avascular necrosis in young patients.

Involuntary vocalisations and a complex hyperkinetic movement disorder following left side thalamic haemorrhage.

A variety of involuntary speech phenomena as for example palilalia have been described as consequences of neurological disorders. Palilalia is the involuntary repetition of syllabels, words and phrases in ongoing speech. We describe a 73 year old woman who suffered from a hypertensive thalamic haemorrhage. MRI revealed that the lesion was predominantly located within the pulvinar, extending to the lateroposterior thalamic nuclei and to the pretectal area with possible involvement of the medial geniculate body. Few months after the event she developed involuntary vocalisations with whole words and meaningless syllables being rapidly reiterated. In contrast to typical palilalia these vocalisations were not meaningfully related to the ongoing speech of the patient. In addition, the patient developed a complex hyperkinetic movement disorder with right-sided painful hemidystonia and bilateral clonic jerks and a right-sided postural tremor.

[The current value of percutaneous transhepatic biliary drainage]. / Gegenwärtiger Stellenwert der perkutanen transhepatischen Cholangiodrainage.

Percutaneous transhepatic biliary drainage (PTBD) is a well established method in the treatment of obstructive jaundice. Major indications are malignant diseases. PTBD may be necessary preoperatively in cases with severe jaundice or cholangitis or as part of palliative treatment concepts. In the past, it has been proposed that a period of preoperative PTBD may improve the morbidity rates of surgery. Various studies could not prove this theory. The significance of preoperative PTBD has changed, as observed during a 15 years period in our own institution, the indications for preoperative PTBD have decreased by half. At present, the majority of treatments with PTBD are palliative (almost 70 % of all procedures). The diagnostic opportunities of the transhepatic approach (intraductal sonography, cholangioscopy, biopsy) are exploited only in few selected cases. Since the radiological approach ist considered to be invasive and related to serious complications most patients are being referred to endoscopic drainage first. Radiologists are consulted in complicated cases of jaundice and when endoscopic approaches have failed. The retrospective evaluation of more than 1000 procedures over a period of 16 years demonstrates good results with a low rate of serious complications. During the two observed periods of nine and seven years, respectively, there occurred complications like sepsis in 1.9 %/0.5 %, peritonitis in 0.5 %/0.7 %, severe bleeding in 0.5 %/1.5 %, procedure-related death in 0.8 %/0.7 %. The overall rate of serious complications was 5 %/3.4 %. These results are comparable to those of the endoscopic approach with a complication rate of 3.6-14 % and a mortality rate of 0.5 %.

Direct ring fission of salicylate by a salicylate 1,2-dioxygenase activity from Pseudaminobacter salicylatoxidans.

In cell extracts of Pseudaminobacter salicylatoxidans strain BN12, an enzymatic activity was detected which converted salicylate in an oxygen-dependent but NAD(P)H-independent reaction to a product with an absorbance maximum at 283 nm. This metabolite was isolated, purified, and identified by mass spectrometry and (1)H and (13)C nuclear magnetic resonance spectroscopy as 2-oxohepta-3,5-dienedioic acid. This metabolite could be formed only by direct ring fission of salicylate by a 1,2-dioxygenase reaction. Cell extracts from P. salicylatoxidans also oxidized 5-aminosalicylate, 3-, 4-, and 5-chlorosalicylate, 3-, 4-, and 5-methylsalicylate, 3- and 5-hydroxysalicylate (gentisate), and 1-hydroxy-2-naphthoate. The dioxygenase was purified and shown to consist of four identical subunits with a molecular weight of about 45,000. The purified enzyme showed higher catalytic constants with gentisate or 1-hydroxy-2-naphthoate than with salicylate. It was therefore concluded that P. salicylatoxidans synthesized a gentisate 1,2-dioxygenase with an extraordinary substrate range, which also allowed the oxidation of salicylate.

Enhanced long-latency somatosensory potentials in major depressive disorder.

Bodily misperceptions are a frequent symptom in major depressive disorder. A reduced ability to deflect attention from somatosensory stimuli may contribute to the generation of unpleasant bodily sensations and co-occur with altered habituation of the brain electric reactions to somatosensory stimuli. The aim of the present study was to explore whether attention-related components of somatosensory evoked potentials (SSEP) and the habituation of these components are altered in major depression. Fifteen patients with major depressive disorder were compared to an age- and gender-matched group of 15 healthy controls. A series of identical, intrusive but not painful electric stimuli were applied to the left index finger for 48 min. Averaged SSEP were computed from multichannel EEG recordings for consecutive recording blocks of the experiment, each block containing 162 stimuli. Based on these data the habituation process of late components of the SSEP was analysed in two latency intervals (50-150, 170-370 ms). Patients showed significantly enhanced reactions throughout the entire experiment. The persistence of enhanced SSEP components throughout the habituation process may be caused by a deficit in reducing the activity of attention-related brain processes concerned with intrusive, yet behaviourally irrelevant, continued stimulation in the state of major depression.

Non-convulsive status epilepticus with marked neuropsychiatric manifestations and MRI changes after treatment of hypercalcaemia.

We describe a 77-year-old woman who developed a confusional state, cognitive impairment, behavioural abnormalities and dysphasia after treatment of hypercalcaemia. Repeated EEG recording revealed rhythmic sharp-wave activity over the right parietal-occipital lobe. Magnetic resonance imaging (MRI) showed marked hyperintense signal changes bilaterally. The diagnosis of a non-convulsive status epilepticus (NCSE) was made. With antiepileptic treatment the patient improved and MRI as well as EEG changes were almost all reversible. NCSE is an important differential diagnosis of patients with neuropsychiatric symptoms and can develop after rapid lowering of serum calcium levels in hypercalcaemia.

A comparison of the effects of clozapine and olanzapine on the EEG in patients with schizophrenia.

Clozapine is known to induce epileptic seizures and changes in EEG-patterns, including slowing and the appearance of epileptiform activity. Olanzapine, a new antipsychotic drug, shares many pharmacological and clinical properties with clozapine. However, in patients treated with olanzapine, no case of seizure induction has been reported so far, and the EEG has not been studied systematically. We examined the EEGs of patients with schizophrenia treated with either olanzapine (N = 9) or clozapine (N = 9) prior to medication and 3 to 7 weeks afterwards. Clozapine induced significant EEG slowing present in 78% of the patients, and definite epileptiform activity appeared in 33%. Olanzapine also induced significant EEG slowing, but less frequently (in 44% of the patients) and less pronounced than clozapine. Olanzapine had no significant effect an epileptiform activity, but in one patient, an isolated sharp/slow-wave complex was observed. These preliminary data suggest that olanzapine induces EEG slowing to a lower extent than clozapine. Olanzapine's possible effect an the seizure threshold deserves further attention.

Magnetic resonance imaging findings in corticobasal degeneration.

Two women (patient 1, 77 years old, and patient 2, 63 years old) with strong clinical evidence for corticobasal degeneration (CBD) are presented. Patient 2 was in an early stage of the disease with only a mild disability of her left hand. In addition to the clinical characteristics, both patients presented the typical cortical reflex myoclonus. Magnetic resonance imaging studies for both patients revealed nearly identical hyperintense lesions somatotopic from the left-hand primary motor cortex (M1), extending to the midline and possibly supplementary motor area (SMA) in patient 2. To our knowledge, this has not been previously described in patients with CBD. These lesions may play a role in the etiology and the development of CBD with involvement of the M1 and may correspond to the underlying pathology of demyelination or gliosis.

Orienting response and frontal midline theta activity: a somatosensory spectral perturbation study.

OBJECTIVE: Brain electric activity in the theta frequency band has been associated with the encoding of new, and the retrieving of previously stored, information. We studied the time course of stimulus-to-stimulus changes of theta activity under repetitive somatosensory stimulation. MATERIALS AND METHODS: Twelve healthy subjects participated in the study. Repetitive electric stimuli, grouped into 48 stimulus trains, were applied to the left index finger. The stimulus trains contained 27 stimuli (0.9 Hz, 2.5 times sensory threshold). Each stimulus train of 30 s was followed by a stimulus-free break of 30 s. This stimulation paradigm allowed the separate estimation of effects for each position of the stimulus in the train and an analysis of stimulus-to-stimulus changes. Multichannel EEG recordings allowed a topographic analysis of the event-related spectral perturbation effects in the theta frequency band. The brain electric novelty response triggered by the stimulus train onset was analyzed by 3 methods: (1) event-related potentials; (2) event-related power spectra for the investigation of spectral perturbation effects on theta activity; and (3) an approach to break down the stimulus-induced theta activity into phase-locked activity and effects on the spontaneous, ongoing theta activity using digital filtering. RESULTS: The main findings are a frontal midline activation in the theta band with the beginning of the stimulus train, which habituates during the subsequent stimulation cycles, as well as evidence that distinct effects of the first stimulus on the ongoing, non-phase-locked, theta activity exist.

[Hallervorden-Spatz syndrome. Differential diagnosis of early onset dementia]. / Die Hallervorden-Spatz-Erkrankung. Eine Differentialdiagnose des präsenilen Demenzsyndroms.

We report the history of a 38 year old patient who began to develop mental deterioration at the age of 26. After a time of 7 years neurological signs like writing dystonia occurred. Hallervorden-Spatz-Disease (HSD) was diagnosed at the age of 36 in vivo with the clinical presentation of severe dystonia, rigidity, dementia, and typical signal loss in the globus-pallidus the reticular part of the substantia nigra, and the nucleus ruber in the T-2 weighted MRI. The "eye-of-the-tiger"-sign, a bilateral hyperintensity in the rostral globus pallidus, was not observed in follow-up examinations. HSD is a rare autosomal-recessive or sporadic disease of unknown etiology. In one third of the patients a dementing process is the first clinical sign of the disorder, and is a rare differential diagnosis of early onset dementia.