Daniela Prayer

[123I]β-CIT spect in multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration

Differentiation between Parkinsons disease (PD) and other neurodegenerative disorders with parkinsonian features, such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD), is difficult on clinical grounds. We studied the pattern of dopaminergic degeneration in 18 patients with probable MSA, 8 patients with PSP, 4 patients with CBD, 48 patients with PD and a similar degree of disability, and 14 control subjects performing single photon emission computed tomography (SPECT) 20 hours after injection of [123I]β‐CIT. Overall striatal binding was significantly reduced in MSA (−51% of normal mean), PSP (−60%), CBD (−35%), and PD (−58%), without overlap with control values. Asymmetry of striatal β‐CIT binding was significantly increased in patients with CBD and PD, as compared with control subjects. Although asymmetry seemed to be less pronounced in MSA and PSP than in PD, this was not statistically significant. Putamen–caudate nucleus ratios in patients with PD, MSA, and PSP, but not with CBD, were significantly reduced, as compared with control subjects. In conclusion, [123I]β‐CIT SPECT reliably enables the visualization of the presynaptic dopaminergic lesion in patients with MSA, PSP, and CBD. In most patients, however, it does not seem to be possible to differentiate these disorders from PD with this method.

A study of the effects of patient anxiety, perceptions and equipment on motion artifacts in magnetic resonance imaging

We investigated to see if motion artifacts (MA) occurring in magnetic resonance imaging (MRI) are related to prescan anxiety measures and test the feasibility of identifying patients at risk for the development of MA before scanning. Furthermore, to determine a possible influence of constructional differences between a 1.5 and a 0.5 tesla scanner on the frequency of MA. Two hundred and ninety-seven first time MRI patients were surveyed before and after imaging with anxiety and attitude questionnaires. Frequency and impact on diagnostic quality of MA were documented. 12.8% of all scans showed MA not related to normal body pulsations. In 6.4% the diagnostic quality was impaired. Constructional differences did not influence the frequency of MA. Also, anxiety as determined with the most common anxiety measuring instrument was not related to the development of MA. Concern about the technical apparatus identified 70.6% of all individuals developing MA. Patients at risk for the development of MA can be identified prior to scanning. It seems necessary to further develop reliable methods to detect them and to evaluate strategies to prevent MA.

Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis.

Central diabetes insipidus (CDI) is a rare disorder associated with various underlying diseases. Among the systemic diseases that may cause CDI, Langerhans cell histiocytosis (LCH) is the most common. Therefore, in patients with endocrinologically proven CDI, a comprehensive diagnostic evaluation is crucial to identify possible extracranial sites of LCH. The goal of the diagnostic evaluation is to yield histopathological proof of the underlying disease. If possible, this histopathological proof should be provided by a biopsy of extracranial lesions to avoid a potentially hazardous biopsy of the pituitary stalk.

Rosette-forming glioneuronal tumor of the fourth ventricle

Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle has been reported recently as a novel type of primary CNS neoplasm. We present the case of a 35-year-old male patient with RGNT of the fourth ventricle. The tumor was found incidentally; the patient did not suffer from any neurological symptoms. The tumor mass involved the caudal cerebellar vermis, filled the fourth ventricle and protruded into the caudal part of the mesencephalic aquaeduct. Smaller tumor nodules were visible in the adjacent right cerebellar hemisphere. Histologically, prominent neurocytic rosettes with synaptophysin expression were embedded in a glial tumor component resembling pilocytic astrocytoma. Clinicopathological features of our case closely resemble those reported in the original description. Thus, our case confirms RGNT as a new distinct type of primary CNS neoplasm. Due to its distinct features, adoption of RGNT as a new entity into the WHO classification of tumors should be considered.

High frequency of EEG and MRI brain abnormalities in panic disorder.

The frequency and quality of brain abnormalities in panic disorder (PD) were assessed with magnetic resonance imaging (MRI). The use of electroencephalography (EEG) to detect PD patients with a high probability of morphologic brain abnormalities was also explored. Consecutive PD patients (n = 120) were screened with routine EEG examinations and were divided into the following subgroups on the basis of their EEG findings: patients with non-epileptic EEG abnormalities (EEG-A group, n = 28), matched patients with normal EEG results (EEG-N group, n = 28) and matched healthy controls (n = 28). PD patients showed a higher than expected rate of non-epileptic EEG abnormalities (29.2%; 35 of 120). EEG screening was effective in identifying patients with a high probability of morphologic brain abnormalities. MRI abnormalities were found in 60.7% of the EEG-A patients, 17.9% of the EEG-N patients, and only 3.6% of the controls. A high frequency of septo-hippocampal abnormalities was found. Further research should focus on attempts to subtype PD on the basis of neuroanatomic and functional brain abnormalities.

Feasibility of Long-Term Intraventricular Therapy with Mafosfamide (n = 26) and Etoposide (n = 11): Experience in 26 Children With Disseminated Malignant Brain Tumors

Treatment options for leptomeningeal disseminated brain tumors are limited by the lack of effective drugs for intrathecal therapy of non-hematologic malignancies. We report on our experience with an intraventricular therapy consisting of mafosfamide, a preactivated cyclophosphamide derivative, and etoposide. Between May 1994 and 2002, 26 patients aged 2–19 years with various intensely pretreated disseminated brain tumors received intraventricular mafosfamide via an indwelling subcutaneous reservoir. Twenty-three of them received a dose of 20mg. Mafosfamide was administered once or twice weekly until remission was achieved and every 2–6 weeks thereafter as maintenance therapy for a total of 736 administrations (2–63/patient). Since March 1998, two patients were switched to receive intraventricular etoposide and nine received etoposide alternating with mafosfamide. Etoposide was given at a dose of 0.5mg×5d every 3–6 weeks for a total of 122 courses (1–29/patient).Immediate toxicities such as transient headaches, nausea, and vomiting occurred with mafosfamide but were manageable with premedication. Etoposide did not cause any discomfort. No long-term toxicities attributable to intrathecal therapy as evidenced by magnetic resonance imaging or neurologic evaluation were observed. Since all patients received some sort of concurrent anti-cancer therapy, the efficacy of intrathecal therapy cannot be assessed independently. However, seven of 13 patients evaluable for response by cerebrospinal fluid (CSF) cytology developed CSF dissemination under systemic chemotherapy and cleared their CSF only after administration of intrathecal mafosfamide. In conclusion, intraventricularly administered mafosfamide at a dose of 20mg and etoposide at a dose of 0.5mg×5d for patients over 2 years of age are feasible and safe and may produce responses.

Psychometric- and Quality-of-life Assessment in long-term Glioblastoma survivors

AbstractBackground: Multimodal treatment of patients with glioblastoma multiforme (GBM) allows an increasing number of patients to survive beyond one year. On account of various neurological and psychophysiological impairments, however, these patients may not benefit in terms of quality of life (QOL). We evaluated the subjective QOL, clinical psychophysiological and cognitive functions in patients with GBM surviving 18 months after diagnosis.nPatients and methods: Thirteen patients underwent psychophysiological and psychometric measurements for central-nervous activation, habituation of skin-conductance reaction, crystallized intelligence, verbal and psychovisual memory. QOL was assessed by the symptom check-list for somatization (SCS-Score).nResults: We found various impairments such as central-nervous deactivation (n = 9) or high activation (n = 3), psychovegetative overexcitement (n = 3) or attenuation (n = 1), reduced verbal (n = 5) and/or psychovisual (n = 5) memory and loss in attention (n = 7) or concentration (n = 5). Severe physical symptoms (grade 5) were fatigue, convulsion, headache, nausea and micturition difficulties. Eleven patients expressed high satisfaction with life in general, whereas only 4 were satisfied with their general state of health. All patients were independent and 8 patients returned to work.nConclusion: Despite various psychophysiological and cognitive impairments, subjective QOL appears mostly unaffected in this patient setting.

Magnetic Resonance Imaging for Recurrent Headache in Childhood and Adolescence

We investigated 429 consecutive patients, aged 5 to 18 (mean: 11.0 ± 3.1) years, diagnosed with migraine or tension‐type headache. The patients underwent either MRI or exclusively clinical follow‐up examinations. Magnetic resonance imaging revealed normal findings in 82.3% and structural changes in 17.7%. However, the vast majority of these changes had minimal or no pathological relevance, and a causal relationship to the patient’s headache could not be proven in any case. In the non‐MRI group, clinical follow‐up examinations confirmed the initial diagnosis in all patients and MRI was not required in any of these subjects. In conclusion, our study shows a poor relation between recurrent headache fulfilling the criteria of migraine and tension‐type headache and structural changes incidentally detected by MRI. In addition, it suggests that clinical follow‐up examinations are reliable. Accordingly, MRI is not required for routine examination of recurrent headache in children and adolescents, but it should be performed in patients with abnormal neurological findings, atypical headache pattern, or significant change of preexisting headache.

Recurrent spinal ependymoma showing partial remission under Imatimib

Summary.Background. There are few treatment options for recurrent spinal ependymoma after surgery and radiation therapy.Clinical presentation. We present a patient with recurrent spinal ependymoma who received radiation therapy after laminectomy and partial tumor resection. Months later, the patient developed gait paresis. MRT showed tumor recurrence and partial resection was again performed. Oral cytotoxic chemotherapy with Temozolomide was initiated. After a short relief, there was clinical worsening and MRT showed progressive disease. As the tumor had stained positively for platelet derived growth factor (PDGF) receptor, treatment with Imatimib was initiated.Findings. The patient experienced improvement in neurological symptoms and the following MRT revealed slight tumor regression. She remained stable for a total of 11 months.Conclusion. Imatimib should be considered a potential therapeutic option in recurrent ependymomas expressing PDGF receptor.

Long-Term MR Imaging Course of Neurodegenerative Langerhans Cell Histiocytosis

BACKGROUND AND PURPOSE: MR imaging signal intensity abnormalities in the cerebellum, the pons, and the basal ganglia, compatible with a neurodegenerative process (ND) were reported in up to 10% of patients with Langerhans cell histiocytosis (LCH). Although the imaging features of ND-LCH have been extensively described, the temporal course of ND-LCH has not been assessed as of yet. The purpose of this study was to describe the long-term course of MR imaging signal intensity abnormalities in ND-LCH on T1- and T2-weighted images. MATERIALS AND METHODS: In this retrospective study, 9 patients with ND-LCH with an observation time of at least 5 years were included. Three or more MR imaging studies per patient, performed in 3-year intervals (±11 months), were reviewed. Signal intensity abnormalities on T1- and T2-weighted images in the cerebellum, the pons, and basal ganglia were scored for their signal intensity quality and their extension. In addition, the severity of cerebellar atrophy was scored. RESULTS: The signal intensity alterations were not resolved in any of the patients. Instead, a progression of the signal intensity alterations either in the cerebellum or basal ganglia was observed in all of the patients but did not correlate with a clinical deterioration. Overt and severe neurologic symptoms were reported in only 2 patients in whom some form of atrophy was noted. CONCLUSIONS: ND-LCH appears to be a slowly progressive process. The increase of signal intensity abnormalities in the cerebellum and basal ganglia does not correlate with neurologic deterioration. MR imaging appears to be a sensitive technique to detect and monitor radiologic ND-LCH.