Frühmanifestation einer frontotemporalen Demenz

The term frontotemporal dementia is used to describe a primary degenerative form of dementia, which is characterized by typical clinical, neuropsychological, radiological and neuropathological features. Its onset is usually before the age of 65 years; manifestations before the age of 30 years have rarely been described. We report the case of a 22-year old man, who showed symptoms of behavioural disorder such as social retreat, lack of initiative, mental rigidity, progressive reduction of speech, and stereotyped behaviour. The neuropsychological examination revealed disorders of the executive functions. The cerebral MRI investigations showed bifrontal atrophies corresponding with hypoperfusion areas on the SPECT. Other investigations including EEG, evoked potentials, duplex ultrasonography, cerebral angiography, laboratory tests and cerebrospinal fluid were normal. In the present case report we discuss the clinical presentation of frontotemporal dementia with early onset.     

Schädel und Hirn einer Microcephalin

Ohne Zusammenfassung     

Immobilisations-Hyperkalzämie als Komplikation einer Polyneuropathie

Prolonged immobilization or physical inactivity has been shown to produce increased bone resorption due to enhanced osteoclastic activity and diminished bone formation. These skeletal changes are a typical complication in tetraplegic patients, who are at risk of developing hypocalcemia. Hypercalciuria is the most characteristic symptom. However, some patients develop hypercalcemia, which is infrequent in these patients, and the hypercalcemia can become a life-threatening complication. Until now, it has been unclear why a small percentage of immobilized patients develop hypercalcemia. Here we present a case of symptomatic hypercalcemia (serum calcium: 3.5 mM/l) following immobilization due to a critical illness polyneuropathy. The diagnosis could be established after malignant hypercalcemia, primary hyperparathyroidism, and other causes of hypercalcemia were excluded. Treatment with intravenous saline, furosemide, and calcitonin was not effective in lowering serum calcium. Treatment with pamidronate (Aredia) was successful and reduced the serum calcium to normal values.     

Spätmanifestation einer Polyglykosankörpermyopathie

We report two patients with polyglycosan body disease manifesting in adulthood. Clinical, electrophysiological, and histopathological characteristics of their disorders are summarized, and diagnostic classification is discussed.     

Invasive Abklärung einer mesialen temporookzipitalen Epilepsie

In German-speaking countries, subdural electrodes are traditionally used in the invasive investigation of pharmacoresistant focal epilepsies. Occasionally, they are combined with a few intracranial depth electrodes. On the contrary, in countries like France and Italy, stereoelectroencephalography (SEEG) is the prevalent method, using multiple stereotactically implanted depth electrodes for the definition of the epileptogenic area. A case report which exemplifies the advantages of SEEG is presented. The establishment of SEEG at the Epilepsy Center Freiburg in 2008 has further expanded the center’s capacity for sophisticated presurgical epilepsy evaluations.     

Stehen wir vor einer Krise der Liquordiagnostik?

Ohne Zusammenfassung     

Infantizid als Folge einer postpartalen Bindungsstörung

Parental killing of their own children in the 1st year constitutes a rare phenomenon. Studies on infanticide show that mothers who kill their children are frequently psychiatrically disturbed. Depression is the most common postpartum disorder and may represent a vital danger for both mother and child. The association between depression and infanticide can usually be explained by maternal psychopathologic symptoms. The importance of a mother's bonding to her infant has not often been examined in respect to infanticide. The present case report underlines the importance of postpartum bonding disorder and its relation to a higher risk of infanticide.     

Spätmanifestation einer Glykogenose Typ II

Zusammenfassung Wir berichten über die Spätmanifestation eines primären Mangels an lysosomaler saurer Maltase bei einer 73-jährigen Patientin, die initial unter dem Verdacht auf eine Motoneuronerkankung vorgestellt wurde. Diskutiert werden die klinischen und elektrophysiologischen Charakteristika der Erkrankung sowie die histopathologische Differenzialdiagnose lysosomaler Glykogenspeichermyopathien.     

Ein Fall von Selbstverstümmelung einer Geisteskranken

Ohne Zusammenfassung     

Ungewöhnliche Ursache einer selbstinduzierten fokalen Epilepsie

Epilepsies and psychiatric disorders such as psychoses are known to have reciprocal influence. There are problems in patient compliance and pharmacological interactions between the drugs used for treatment. We report an unusual, self-induced, focal, symptomatic epilepsy in a hitherto unrevealed schizophrenic psychosis. The interdisciplinary treatment of three different departments was necessary. The initial symptom of a diffuse frontal headache and the patient's penetration of his own skull in an attempt at self-treatment led to the development of a frontal abscess around a foreign body and the described focal epilepsy.     

Darstellung und Beschreibung einer intrauterin entstandenen Narbe in der rechten Hemisphäre des Gehirns einer chronisch Blödsinnigen

Ohne ZusammenfassungVorgetragen in der Sitzung der Berliner medicinischen Gesellschaft am 29. Juni 1887.     

Metastasierendes Schilddrüsenadenom unter dem Bilde einer Wirbelgeschwulst mit Kompressionsmyelitis nebst einer Methode zu Probeexzisionen von Knochenmark aus tiefliegenden Knochen

Ohne Zusammenfassung     

Versuch einer Einteilung der Rückenmarkshinterstränge

Ohne Zusammenfassung     

Versuch einer hirnphysiologischen Theorie der schizophrenen Grundstörung

Ohne Zusammenfassung     

Anencephalie als Folge einer Frühform der Neurofibromatose

Zusammenfassung Die eingehende neuropathologische Untersuchung eines 14 Tage überlebenden weiblichen Anencephalus zeigt Veränderungen am Rückenmark in Form von multiplen Neurinomknoten der Spinalwurzeln und einer Subarachnoidalgliose, die die Diagnose einer besonderen Frühform der Neurofibromatose Recklinghausen gestattet. Innerhalb eines primär gut angelegten und bis zu einem gewissen Grad auch ausdifferenzierten Groß-, Klein- und Mittelhirngewebes haben die blastomatösen Vorgänge zu sekundären Veränderungen im Sinne einer Meroanencephalie (Ernst) geführt.Mit 7 TextabbildungenHerrn Professor Dr. G. Schaltenbrand zum 65. Geburtstag in Verehrung gewidmet.     

Exophiala-Spezies — seltene Erreger einer Endokarditis mit zerebraler Abszedierung

Exophiala species belong to the dematiaceous fungi. Occurring worldwide, they are a rare cause of human infection. We present the case of a 75-year-old immunocompetent patient with a cardiogenic embolic anterior cerebral artery infarction. Echocardiography revealed endocarditis of the aortic valve. Antibiotic therapy was initiated after susceptibility testing (blood cultures were positive for Staphylococcus aureus, and liquor was normal), with a subsequent fall in serologic markers of infection and resolution of the signs of endocarditis on echocardiography. However, 6 weeks after initiation of antibiotic therapy, abscesses were seen in the infarct region on CT scan, although antimicrobial treatment was still continued. At this stage, the CSF showed an inflammatory process, and Exophiala species, susceptible to voriconazol, could be detected in liquor cultures. However, antifungal therapy with voriconazol could not prevent severe sepsis and death from multiorgan failure. Autopsy revealed the clinically supposed Exophiala endocarditis with metastatic cerebral abscesses. In accordance with other published case reports, a fatal outcome in disseminated Exophiala infection might only be prevented by aggressive therapy consisting of early surgical removal of the foci and combined antifungal agents.     

Tumorentwicklung aus einer persistierenden embryonalen Körnerschicht der Großhirnrinde bei einer Spätform der tuberösen Sklerose

Zusammenfassung Es wird über einen Fall berichtet, bei dem gegen Ende des 5. Lebensjahrzehntes die Symptome eines Tumor cerebri aufraten. Die Sektion deckte u. a. mehrere gliöse Tumoren der Grßhirnrinde und ein Meningiom des Felsenbeines auf. Es fanden sich Veränderungen, die den Fall als eine Spätform der tuberösen Sklerose (Kufs) einordnen ließen. Über Teilen der Hemisphären waren noch Reste der embryonalen Körnerschicht vorhanden. Es wird die Ansicht vertreten, daß die gliösen Rindentumoren aus dieser persistierenden Körnerschicht hervorgegangen sind.Mit 6 Textabbildungen