No abstract

A brain biopsy obtained from a twenty-eight month old boy with ceroidlipofuscinosis was studied by light and electron microscopy. There were widespread intracellular deposits of autofluorescent material taking the fat stains. Cytoplasmic inclusions were plentiful in neurons, astrocytes, oligocytes, M cells and vascular elements. Their substructure ranged from that of variably dense aggregates of essentially homogeneous or granular appearance to that of miscellaneous collections of lamellar pairs and/or tubular structures of variable length. Stacks of 2 to 4 linear profiles with a curved outline were rarely seen and then almost exclusively inside cytosomes of endothelial cells. Similar observations were made in peripheral nerve, skin and liver biopsies. The granules of peripheral blood neutrophilic leukocytes were unremarkable. A small percentage of lymphocytes contained granular cytoplasmic bodies not unlike those known to be an ordinary feature of some lymphocytes of the average blood sample. However, a certain resemblance between these bodies and some of the cytosomes seen in the patient's tissues was also apparent. Myeloperoxidase activity was tested with paraphenylenediamine and was found to be normal on two occasions. The patient's age, cytosome morphology and distribution and results of peroxidase assay add special interest to this case of generalized ceroidlipofuscinosis. However, none of these features, either singly or in combination, warrants creation of a distinct subtype within this group of disorders. Myeloperoxidase deficiency is probably just another phenotypical marker of some patients with generalized ceroidlipofuscinosis rather than the genetic defect of Batten disease.

Encephalomyelitis was produced in adult albino mice by intracerebrM inoculation of Zimmern virus, a group B arbovirus belonging to the TBE complex. By electron microscopy, newly synthesized virions could be found inside numerous cortical and spinal neurons. They were usually situated within membrane-bound compartments of the endoplasmic reticulum and Golgi apparatus. The most conspicuous changes exhibited by tile virus-infected nerve cells were formation of vesicular and vacuolar aggregates in the cytoplasm, disorganization or reduction of the endoplasmic reticulum and chromatolysis of variable severity. Moreover, peculiar tubular structures ~bout 280 A_ in outer diameter could be observed, side by side with newly formed virus particles, within several channels of the endoplasmic reticulum. High resolution electron micrographs of these unique tubules showed that they had indeed a two-strand helical type of configuration. It was not possible to clarify the reM nature of these microhelices. In particular, it had to be left undecided whether they were somehow pertinent to the viral replication and/or assembly. Zusammen~assungBei erwachsenen weil3en M/iusen wurde durch intracerebrale Inokul~tion yon Zimmern-Virus, ein dem TBE-Komplex angehSrendes Arbovirus der Gruppe B, eine Encephalomyelitis erzeugt. Elektronenmikroskopiseh konnten in zahlreichen cortikMen und spinalen Nervenzellen neugebildete Viruspartikel beobachtet werden. Diese lagen gew6hnlich in membranbegrenzten R~umen des endoplasmatischen Retiknlums und des Golgi-Apparates. Die hervorsteehendsten morphologischen Ver/mderungen der virusinfizierten Neuronen waren das Auftreten yon Ansammlungen kleiner B1/tsehen und Vakuolen im Cytoplasma, eine auffMlend unregelm/~gige Anordnung oder deutliehe Verminderung der Zisternen 1 Dedicated to Prof. Dr. G. PETERS on the occasion of his 65 th birthday. BLINZINGER et al.: Microhelices in Murine Neurons Infected with an Arbovirus 195 des endoplasmatischen Retikulums sowie eine mehr oder minder ausgepr~igte Chromatolyse. Dartiber hinaus waren in manehen Kan/~lchen des endoplasmatischen Retikulums unmittelbar neben neu synthetisierten Virionen eigenttimliche tubul~re Gebilde mit einem Augendurchmesser yon etwa 280 A anzutreffen. Bei h6herer AuflSsung fanden sich Hinweise daftir, dal3 diese merkwiirdigen Tubuli in Wirklichkeit doppelstr~ngige Helices darstellen. Bisher ist es Mlerdings nicht gelungen, iiber die tatsttchliche Natur dieser Mikrohelices Aufschlug zu gcwinnen. Insbesondcre lieI~ es sich nicht kl/~ren, ob sic mit der Virusvermehrung und/oder Virusmaturation in irgendeinem Zusammenhang stehen.

No abstract

In quest of a more precise and stringent diagnosis for a case of generalized ceroidlipofuscinosis the patient was reexamined and additional laboratory tests were performed. For the third time myeloperoxidase activity was normal. On two separate occasions the serum lecithin fatty acid pattern was found to be abnormal and to resemble closely the pattern reported to be characteristic of polyunsaturated fatty acid lipidosis. The tau fraction was measured on one occasion and found to be normally present. Head circumference was confirmed to be normal. Pigmentary retinal changes were observed by fundoscopic examination. The child had prominent epileptic activity in form of myoclonic jerks. The case does not fit exactly into the picture reported recently for children with polyunsaturated fatty acid lipidosis. Doubt is voiced that these children have other than an infantile type of generalized ceroidlipofuscinosis. Thus, for the time being we regard ours as a case of generalized ceroidlipofuscinosis of infantile onset with an abnormal serum lecithin fatty acid composition. Furthermore, it seems to us that the infantile form, in the same manner and to the same extent as the late infantile and juvenile forms, does not constitute an entirely homogeneous group within the ceroidlipofuscinoses.