F. van Landeghem

Plastic bronchitis in children with fontan palliation: Analogue to protein losing enteropathy?

AbstractWe studied a 6-year-old boy and a 2-year-old girl with bronchitis fibroplastica following Fontan operation. Large endobronchial casts of rubber-like consistency resulted in life-threatening pulmonary failure. In one patient symptoms improved after optimizing heart function with diuretics, and in the other a dramatic improvement with the resolution of the clinical symptoms and normalized serum albumin followed subcutaneous high-molecular-weight heparin treatment. The severe relapse after discontinuation of the heparin medication and the once more successful treatment with heparin suggest that in addition to optimizing heart function, high-molecular-weight heparin might be a therapeutic option for this poorly understood condition.

Transient loss of inhibition precedes spontaneous seizures after experimental status epilepticus.

The pathophysiological mechanisms that cause spontaneous seizures following status epilepticus are largely unknown. Erosion of inhibition is regarded as an important pathophysiological hallmark of ongoing status epilepticus. Therefore, we investigated if loss of inhibitory functions also plays an important role in the development of spontaneous seizures after status epilepticus. Furthermore, we analyzed possible changes in excitation that might contribute to epileptogenesis. Finally, neuronal cell loss in the dentate gyrus granule cell layer was analyzed. In rats, inhibition and excitation in the dentate gyrus were monitored 1, 4, and 8 weeks after electrically induced self-sustaining status epilepticus (SSSE). Control animals had electrodes implanted either without subsequent stimulation or with stimulation but under barbiturate anesthesia, neither of which resulted in subsequent spontaneous seizures or impairment of inhibition. Following SSSE 80% of animals developed seizures after 8 weeks. A pronounced impairment of inhibition 1 week after SSSE was followed by gradual recovery over 8 weeks. In the dentate gyrus, cell damage was highly variable most likely explaining the heterogeneity of changes in excitatory parameters. Loss of GABAergic inhibition in the dentate gyrus may facilitate initiation of epileptogenesis but impaired inhibition is not required for the process of epileptogenesis to be maintained.

Panarteritis nodosa – ein „klassischer“ Fall

ZusammenfassungDas klinische Bild der klassischen Panarteritis nodosa (c-PAN) wird aufgrund der Heterogenität der möglichen Frühsymptome auch heute noch häufig verkannt. Anhand des dargestellten Falls folgen wir dem Verlauf der Erkrankung vom ersten Symptom bis zur histologischen Diagnosesicherung, hier durch Biopsie des Nervus suralis. Diskutiert werden die pathogenetische Zuordnung der c-PAN innerhalb der Vaskulitiden, ihre Klassifikation durch die Kriterien des American College of Rheumatology (ACR) sowie aktuelle Therapiestrategien. Der Fall verdeutlicht den hohen Anspruch an interdisziplinäres Denken, den die Erkrankung an Neurologen, Dermatologen, Orthopäden und Internisten/Rheumatologen stellt.SummaryDiagnosis of polyarteritis nodosa is often delayed due to the vast heterogeneity of initial clinical symptoms. The case presented shows the clinical image of the disease, leading from the first symptoms up to verification of the diagnosis by sural-nerve biopsy. We discuss the classification of the disease among other types of vasculitis, the classification criteria proposed by the American College of Rheumatology (ACR) as well as current therapeutic options. This case underlines the interdisciplinary character of the disease, challenging neurologists, dermatologists, rheumatologists and orthopedics alike.Diagnosis of polyarteritis nodosa is often delayed due to the vast heterogeneity of initial clinical symptoms. The case presented shows the clinical image of the disease, leading from the first symptoms up to verification of the diagnosis by sural-nerve biopsy. We discuss the classification of the disease among other types of vasculitis, the classification criteria proposed by the American College of Rheumatology (ACR) as well as current therapeutic options. This case underlines the interdisciplinary character of the disease, challenging neurologists, dermatologists, rheumatologists and orthopedics alike.

Panarteritis nodosa – ein „klassischer“ Fall@@@Polyarteritis nodosa – a “classical” case

ZusammenfassungDas klinische Bild der klassischen Panarteritis nodosa (c-PAN) wird aufgrund der Heterogenität der möglichen Frühsymptome auch heute noch häufig verkannt. Anhand des dargestellten Falls folgen wir dem Verlauf der Erkrankung vom ersten Symptom bis zur histologischen Diagnosesicherung, hier durch Biopsie des Nervus suralis. Diskutiert werden die pathogenetische Zuordnung der c-PAN innerhalb der Vaskulitiden, ihre Klassifikation durch die Kriterien des American College of Rheumatology (ACR) sowie aktuelle Therapiestrategien. Der Fall verdeutlicht den hohen Anspruch an interdisziplinäres Denken, den die Erkrankung an Neurologen, Dermatologen, Orthopäden und Internisten/Rheumatologen stellt.SummaryDiagnosis of polyarteritis nodosa is often delayed due to the vast heterogeneity of initial clinical symptoms. The case presented shows the clinical image of the disease, leading from the first symptoms up to verification of the diagnosis by sural-nerve biopsy. We discuss the classification of the disease among other types of vasculitis, the classification criteria proposed by the American College of Rheumatology (ACR) as well as current therapeutic options. This case underlines the interdisciplinary character of the disease, challenging neurologists, dermatologists, rheumatologists and orthopedics alike.Diagnosis of polyarteritis nodosa is often delayed due to the vast heterogeneity of initial clinical symptoms. The case presented shows the clinical image of the disease, leading from the first symptoms up to verification of the diagnosis by sural-nerve biopsy. We discuss the classification of the disease among other types of vasculitis, the classification criteria proposed by the American College of Rheumatology (ACR) as well as current therapeutic options. This case underlines the interdisciplinary character of the disease, challenging neurologists, dermatologists, rheumatologists and orthopedics alike.

Sporadische zerebrale Amyloidangiopathie mit ischämischer Leukenzephalopathie und Mikroblutungen

ZusammenfassungWir berichten über einen 72-jährigen Patienten ohne vaskuläre Risikofaktoren mit einem rasch progredienten demenziellen Syndrom und einem erstmalig aufgetretenen komplex-fokalen epileptischen Anfall. Im zerebralen MRT zeigten sich eine Leukenzephalopathie mit Beteiligung der U-Fasern ohne Kontrastmittelaufnahme sowie kortikosubkortikale Mikroblutungen auf der T2*-Sequenz. Die Hirnbiopsie bestätigte die Diagnose einer sporadischen zerebralen Aβ-Amyloidangiopathie (CAA). Der Fall illustriert die differenzialdiagnostische Bedeutung der CAA als Ursache einer Leukenzephalopathie mit rasch progredienter Demenz und unterstreicht den diagnostischen Stellenwert der T2*-MR-Bildgebung bei der klinischen Evaluierung eines demenziellen Syndroms.SummaryWe describe a 72-year-old patient with rapidly progressive dementia and a complex focal seizure. Magnetic resonance (MR) imaging revealed leukoencephalopathy with the involvement of the U-fibers as well as cortical and subcortical microbleeds. Brain biopsy confirmed the diagnosis of cerebral Aβ amyloid angiopathy (CAA). The presented case illustrates the significance of CAA as a cause of rapidly progressive dementia and leukoencephalopathy and points out the importance of T2-weighted MR imaging in the evaluation of dementia.

Sporadische zerebrale Amyloidangiopathie mit ischämischer Leukenzephalopathie und Mikroblutungen@@@Sporadic cerebral amyloid angiopathy with ischemic leukoencephalopathy and microbleeds: Ursache eines rasch progredienten demenziellen Syndroms@@@Cause for a rapidly progressive dementia syndrome

ZusammenfassungWir berichten über einen 72-jährigen Patienten ohne vaskuläre Risikofaktoren mit einem rasch progredienten demenziellen Syndrom und einem erstmalig aufgetretenen komplex-fokalen epileptischen Anfall. Im zerebralen MRT zeigten sich eine Leukenzephalopathie mit Beteiligung der U-Fasern ohne Kontrastmittelaufnahme sowie kortikosubkortikale Mikroblutungen auf der T2*-Sequenz. Die Hirnbiopsie bestätigte die Diagnose einer sporadischen zerebralen Aβ-Amyloidangiopathie (CAA). Der Fall illustriert die differenzialdiagnostische Bedeutung der CAA als Ursache einer Leukenzephalopathie mit rasch progredienter Demenz und unterstreicht den diagnostischen Stellenwert der T2*-MR-Bildgebung bei der klinischen Evaluierung eines demenziellen Syndroms.SummaryWe describe a 72-year-old patient with rapidly progressive dementia and a complex focal seizure. Magnetic resonance (MR) imaging revealed leukoencephalopathy with the involvement of the U-fibers as well as cortical and subcortical microbleeds. Brain biopsy confirmed the diagnosis of cerebral Aβ amyloid angiopathy (CAA). The presented case illustrates the significance of CAA as a cause of rapidly progressive dementia and leukoencephalopathy and points out the importance of T2-weighted MR imaging in the evaluation of dementia.