Claus Meier

Peripheral nerve disorders in Lyme-Borreliosis

SummaryClinical, cerebrospinal fluid and nerve biopsy findings from eight patients with peripheral nervous system complications of Lyme-Borreliosis are reported. Five cases showed the typical features of the Garin-Bujadoux-Bannwarth syndrome (meningoradiculoneuritis), one patient had a multiple mononeuritis associated with acrodermatitis chronica atrophicans Herxheimer. Two cases could not be classified under these diagnostic categories. In all patients we observed a prompt relief of signs and symptoms after antibiotic treatment. Nerve biopsy studies showed gross infiltrations of epineurial vasa nervorum and small infiltrations around endoneurial capillaries. The infiltrations consisted of lymphocytes, histiocytes and plasma cells. We did not find necrotizing changes of the vessel walls, but thrombosis and recanalization was observed in some epineurial vessels. Seven biopsies showed a significant loss of myelinated axons due to axonal degeneration. Only in one biopsy did we observe segmental demyclination next to axonal degeneration. We conclude that the PNS complications of Lyme-Borreliosis in early and late stages of the disease are angiopathic due to vasculitis of the vasa nervorum and primarily caused by axonal degeneration.

Peripheral and central conduction times in hereditary pressure-sensitive neuropathy.

SummarySeven members of a family with histologically proven hereditary pressure-sensitive neuropathy (HPSN) agreed to be examined clinically and electrophysiologically. A sural nerve biopsy specimen taken from the propositus who suffered from a partial brachial plexus palsy showed typical ‘sausage-like’ myelin sheath thickenings reflecting a failure of axon-adjusted myelination. Reduced motor and sensory conduction velocities involving several nerves were found in the four family members with clinical signs of HPSN. In addition, central conduction times in the auditory and somatosensory pathways were determined measuring the interwave latency I–V in brainstem auditory-evoked potentials and the interpeak latency N14–N20 in median nerve sensory-evoked potentials. Central conduction times in both afferent systems were within normal limits. The absolute delay of peak N14 and N20 in median and P40 in tibial nerve-evoked potentials was probably due to an impaired conduction in the peripheral branch of the bipolar ganglion cell. Whether the central axon branch in the dorsal columns was also involved could not be decided.ZusammenfassungSieben Mitglieder einer Familie mit hereditärer druckempfindlicher Neuropathie wurden klinisch und elektrophysiologisch untersucht. Im Suralis-Bioptat des Propositus, der sich wegen einer inkompletten Armplexusparese vorstellte, zeigten sich die charakteristischen tomaculären (wurstförmigen) Myelinverdickungen als Hinweis auf eine gestörte Axon-Myelin-Relation. Bei den vier Familienmitgliedern mit den klinischen Zeichen der Erkrankung fanden sich verlangsamte motorische und sensible Leitgeschwindigkeiten an verschiedenen Nerven. Zusätzlich wurde die zentrale Leitungszeit in auditiven und somatosensorischen Bahnen bestimmt, wobei die Latenzdifferenzen von Welle I–V im akustisch evozierten Hirnstammpotential und von Peak N14–N20 in Medianus-SEP berechnet wurden. Die zentralen Leitungszeiten in beiden Systemen lagen im Normbereich. Die absolute Verzögerung von N14 und N20 in Medianus- sowie P40 in Tibialis-SEP ist wahrscheinlich Folge einer verlangsamten Leitung im peripheren Ast der bipolaren Ganglienzelle. Ob auch eine Affektion des zentralen Axonastes in den Hintersträngen vorliegt, kann anhand der Resultate dieser Arbeit nicht entschieden werden.

Sequence of morphological alterations in the nervous system of metachromatic leucodystrophy

SummaryLight and electronmicroscopic findings are reported in a case of metachromatic leukodystrophy diagnosed prenatally who died after iatrogenic abortion during the 23th week of gestation. The brain of this foetus was not yet myelinated while the spinal cord showed early, and the peripheral nerves advanced myelination. The onset and the degree of myelination were similar as in a normal foetus of the same age. Ultrastructurally there was evidence of sulphatide storage before the beginning of myelination. During myelination lysosomal storage material, staining metachromatically in acid cresyl violet preparations, appeared in oligodendrocytes and Schwann cells. Besides sulphatide storage material, prominent amounts of neutral lipids were found in oligodendrocytes. Myelin breakdown was encountered very seldom.

Dynamic aspects of peripheral nerve changes in progressive neural muscular atrophy

SummarySerial nerve biopsies were performed at an early, and at an advanced stage of the disease in 2 patients with progressive neural muscular atrophy. The early biopsy showed a complete loss of the large diameter and thickly myelinated fibres, as well as an expansion of the endoneurial interstitium in both cases. Myelinated and unmyelinated fibres exhibited axonal degeneration in all biopsies occasionally. “Onion bulb” formation, a typical feature of peripheral neuropathy in neural muscular atrophy, was found to be prominent only in the latter biopsies. As regards the formal pathogenesis of hypertrophic neuropathy in neural muscular atrophy, axonal dystrophy and interstitial changes of the endoneurium were regarded as primary phenomena, demyelination and “onion bulb” formation as secondary. A possible causal relation between axonal dystrophy and interstitial changes, observed in these cases, is discussed in the light of the present literature.ZusammenfassungBei 2 Patienten mit progressiver neuraler Muskelatrophie wurden Nervenbiopsien jeweils in einem frühen und in einem fortgeschrittenerem Stadium der Erkrankung entnommen und verglichen. In beiden Fällen zeigten bereits die frühen Biopsien ein völliges Fehlen der großkalibrigen, dickbemarkten Axone. Ebenfalls als frühe Veränderung wurde eine Erweiterung des endoneuralen Interstitiums festgestellt. Eine geringe Anzahl der vorhandenen bemarkten und unbemarkten Axone in allen Biopsien wies degenerative Veränderungen auf. Die für die progressive neurale Muskelatrophie typische Zwiebelschalenbildung der Schwannschen Zellen — möglicherweise eine Reaktion auf wiederholte De-und Remyelinisierungsvorgänge um dystrophische Axone — trat erst in den späteren Biopsien deutlicher hervor. Hinsichtlich der formalen Genese der hypertrophischen Neuropathie bei neuraler Muskelatrophie sind nach diesen Beobachtungen axonale Dystrophie und interstitielle Veränderungen des Endoneuriums als primäre Entmarkung und Zwiebelschalenbildung als sekundäre Phänomene zu betrachten. Die Möglichkeit einer kausalen Beziehung zwischen axonaler Dystrophie und interstitiellen Veränderungen wird an Hand der vorliegenden Befunde und Literatur diskutiert.

Qualitative und quantitative Untersuchungen der Hautnerven bei Psoriasis

SummaryThis study was aimed at investigating the role of the nerves in the pathomechanism of psoriasis. The structure of skin nerves of 10 patients with active psoriasis and of 6 healthy control persons has been studied by means of the microscope and the electron-microscope. Eight of the patients were subjected to punch biopsies of psoritatic plaques as well as clinically normal skin.No qualitative difference could be detected between the skin nerves from psoriatic patients and from healthy persons. Quantitative investigations showed that the density of myelinated and unmyelinated axons in psoriatic skin nerves did not differ from that in normal skin nerves in a statistically significant degree. This means that the ratio of myelinated to unmyelinated axons was alike in both normal and psoriatic skin nerves. Also the diameter distribution of myelinated and unmyelinated axons was similar. The difference in the degree of myelinisation and in the myelinperiodicity between psoriatic and control skin was not statistically significant.Neither qualitative nor quantitative morphologic alterations in the skin nerves of psoriatic persons could be detected.ZusammenfassungAls Beitrag zur Aufklärung der pathogenetischen Rolle des Nervensystems bei der Schuppenflechte wurden die Hautnerven von 10 Psoriatikern und 6 Kontrollpersonen licht- und vor allem elektronenmikroskopisch untersucht und miteinander verglichen. Von den Psoriatikern wurden jeweils Biopsien aus Plaques und aus klinisch unauffälligen Hautbezirken entnommen. Bei der qualitativen Analyse ließen sich keine sicheren Unterschiede zwischen den Hautnerven von Kontrollpersonen und von Psoriatikern feststellen.Auch in der quantitativen Auswertung fanden sich keine signifikanten Unterschiede in den Hautnerven von Kontrollpersonen und von Psoriatikern in bezug auf die Dichte der myelinisierten und unmyelinisierten Axone. Auch das Verhältnis der bemarkten zu den unbemarkten Axonen ist in den drei untersuchten Gruppen nahezu gleich; dasselbe gilt für die Größenverteilung der myelinisierten und unmyelinisierten Axone. Auch Myelinisierungsgrad und Myelinperiodizität der Markfasern von Psoriatikern erwiesen sich als völlig normal.Es ließ sich weder qualitativ noch quantitativ eine Veränderung der Hautnerven bei Psoriasis nachweisen.This study was aimed at investigating the role of the nerves in the pathomechanism of psoriasis. The structure of skin nerves of 10 patients with active psoriasis and of 6 healthy control persons has been studied by means of the microscope and the electron-microscope. Eight of the patients were subjected to punch biopsies of psoritatic plaques as well as clinically normal skin. No qualitative difference could be detected between the skin nerves from psoriatic patients and from healthy persons. Quantitative investigations showed that the density of myelinated and unmyelinated axons in psoriatic skin nerves did not differ from that in normal skin nerves in a statistically significant degree. This means that the ratio of myelinated to unmyelinated axons was alike in both normal and psoriatic skin nerves. Also the diameter distribution of myelinated and unmyelinated axons was similar. The difference in the degree of myelinisation and in the myelinperiodicity between psoriatic and control skin was not statistically significant. Neither qualitative nor quantitative morphologic alterations in the skin nerves of psoriatic persons could be detected.