Aage Harmsen

ULTRASTRUCTURE OF MUSCLE BIOPSIES IN RECENT, SHORT‐TERM AND LONG‐TERM JUVENILE DIABETES

Muscle biopsies from juvenile diabetics were studied by electron microscopy and the results correlated to earlier published light microscopical abnormalities of the same biopsies. A total of 32 striated muscle biopsies from 29 juvenile recent, short‐term and long‐term diabetes were examined. The ages ranged from 16 to 43 years with a diabetes duration from 1 week to 32 years. The material was compared with biopsies from healthy persons of the same ages. The electron microscopy revealed degenerative changes of the striated muscle fibres which signify a neurogenic atrophy. No myopathic alterations were found. The capillaries in both the recent and long‐term diabetics showed an extremely pronounced pinocytosis indicating an increased metabolic activity. In the long‐term group the intramuscular capillaries both from upper and lower extremities displayed a definitely thickened basement membrane. In the recent and short‐term group the capillary wall alterations were debatable. The motor end plate in the recent diabetics revealed minimal alterations. Contrary to this the long‐term group showed severe alterations with occasional disappearance of the nerve fibres.

Qualitative and quantitative histological study of neuromuscular biopsies from healthy young men

Abstract Neuromuscular biopsies were taken from palmaris longus in 5 healthy young male volunteers. Measurements were made of muscle fibre cross-sectional area, of synaptic area and of the terminal innervation ratio. Marked differences in mean cross-sectional area of the muscle fibres, extending from 2832 to 4854 μ2 were observed which could not be correlated with either the height and weight of the subject, or with their customary physical activity. Mean values for synaptic area extended from 177.4 to 224.9 μ2, and there was a proportionality between this figure and the mean cross-sectional area of the muscle fibres. Statistical analysis of the frequency distribution curve for muscle fibre cross-sectional area and synaptic area established that they correspond to a normal or a logarithmic-normal distribution. There is no indication of a heterogeneity of muscle fibres and of end-plate populations, as far as their dimensions are concerned. Collateral branching occurs in 7.3–18.4% of the motor axons and more than one bifurcation along these fibres is exceptional. The pooled terminal innervation ratio of 1.12 1 is very near to the value previously considered to be normal.

Cerebral abscess A long-term follow-up

ABSTRACT – During the period 1935–1976, 200 cases of brain abscess were treated in the University Clinic of Neurosurgery, Rigshospitalet, Copenhagen. 119 patients survived. Out of 40 deaths during the follow‐up period, 37 were unrelated to the previous brain abscess. 12 patients emigrated and were lost to follow‐up. History and neurologic examination of the remaining 67 surviving patients with follow‐up periods of between 3 and 40 years (mean 18 years) showed no sequelae in 23, unrestricted working capacity in 46, epilepsy in 37, neurologic deficit in 18, and intellectual impairment in 13 patients. The most serious sequelae, incapacitating epilepsy and severe mental reduction, were seen in patients with brain abscess during childhood.

PSYCHOLOGICAL EVALUATION OF INTELLIGENCE AND PERSONALITY IN PARKINSONISM BEFORE AND AFTER STEREOTAXIC SURGERY

In a series of Parkinsonian patients subjected to stereotaxic surgery, the mental function was studied by means of psychological tests. The purpose was to throw light on the possible postoperative mental changes which together with fear of speech disturbances seem to be the most important contra‐indications for neurosurgical treatment. In the study, 41 patients operated on for Parkinsonism were subjected to preoperative and postoperative psychological tests (average observation period 8.7 months). Bilateral operation was performed in seven cases. The mean age of the patients was 60 years. The tests used were the Wechsler Adult Intelligence Scale and the Rorschach test. In the series as a whole, no significant quantitative changes in the postoperative mental function were revealed by the two tests. The Rorschach test revealed as an interesting finding that all the Parkinsonian patients preoperatively showed some mental reduction and that there was no significant difference between the patients with paralysis agitans and those with postencephalitic Parkinsonism. Slight postoperative changes, viz. a diminished and more rigid activity, were demonstrated in three clinical groups: patients subjected to operation on the globus pallidus, patients with postencephalitic Parkinsonism, and those with unilateral affection in the dominant hemisphere. The results of the study thus seem to indicate that a certain reluctance should be exercised in offering stereotaxic surgery to postencephalitic patients and to those with unilateral affection in the dominant hemisphere. Apart from this, it seems justified to conclude that in patients who otherwise meet the indications, the risk of postoperative mental impairment does not constitute a contra‐indication for this type of surgery.

The pattern of terminal motor innervation in healthy young adults.

The functional, or true terminal innervation ratio (TIR) was estimated on 13 neuromuscular biopsies taken from 5 palmaris longus and 7 tibialis anterior muscles in 7 male and 6 female healthy young volunteers. Axonal ramification resulting in the innervation of more than 1 muscle fibre by 1 subterminal axon occurs in 10.6% of the total number of axons studied. Most of the branching axons (94%) innervate 2 muscle fibres. Only 5.1 of them supply 3 muscle fibres by trifurcation. Innervation of 4 muscle fibres by 1 ramified subterminal axon is extremely rare and was observed in 0.9% of branching fibres, i.e. in 0.1% of all axons studied. There is no significant difference between the TIR of palmaris longus and that of tibialis anterior, nor between the values obtained in males and females. A mean TIR of 1.12 was obtained (range 1.05–1.20). Statistical analysis indicates that values higher than 1.25 are outside the normal range of distribution.

Tod durch maligne Hyperthermie in der Narkose

SummaryThree fatal cases of malignant Hyperthermia are reported and the aetiology of the syndrome is discussed on the basis of recent literature. The syndrom is subdivided in a heriditary type with familiar occurrence of anaesthetic complications, with myopathia and high serum creatinine phosphokinase levels, and a symptomatic type where these characteristics are lacking, but where there is a local or diffuse disorder in the central nervous system.In the reported cases all patients developed typical clinical symptoms of malignant hyperthermia during anaesthesia with Suxamethonium-chloride and Halothane. The postmortem examinations demonstrated: 1. chronic meningo-encephalo-myeloradiculitis, 2. cerebral glucolipoidosis and 3. encephalopathia due to perinatal anoxia and previous bilateral frontal lobotomia. There were no signs of myopathia in any case.The cases belonged to the symptomatic type, with signs of preexisting brain disease, in two cases without clinical symptoms while one patient was mentally deficient.The classification in the two types is important, because there is no increased risks for anaesthetic complications for the remaining family in the symptomatic type.ZusammenfassungDie Ätiologie des Syndroms maligne Hyperthermie wird anhand der Literatur diskutiert. Drei Fälle werden referiert, bei denen bestehende Krankheitszeichen in Gehirngewebe gefunden wurden, aber nicht im Muskelgewebe und in keinem Fall kam maligne Hyperthermie in der Familie vor.Die Fälle wurden deshalb als symptomatische maligne Hyperthermie im Gegensatz zu der heriditären Form klassifiziert, bei der das Syndrom familiär auftritt und man in der quergestreiften Muskulatur vieler Familienmitglieder Veränderungen findet; diese haben das Risiko, die Symptome während der Narkose zu entwickeln, deshalb scheint die Klassifikation in zwei Gruppen von Bedeutung. Man empfiehlt, Fälle dieser Art immer zur rechtsmedizinischen Untersuchung unter Mitwirkung anästhesiologischer Expertise gelangen zu lassen und besonders Muskelgewebe und Gehirngewebe genaustens untersuchen zu lassen.Die Atiologie des Syndroms maligne Hyperthermie wird anhand der Literatur diskutiert. Drei Falle werden referiert, bei denen bestehende Krankheitszeichen in Gehirngewebe gefunden wurden, aber nicht im Muskelgewebe und in keinem Fall kam maligne Hyperthermie in der Familie vor.

INTRATHECAL CYTOTOXIC TREATMENT OF CEREBROSPINAL LEUKAEMIA THROUGH A RICKHAM RESERVOIR

Intrathecal injection of cytotoxic agents in the treatment of cerebrospinal leukaemia has been used for several years. Systemic treatment of leukaemia with this localization is not effective as most cytotoxic drugs cannot pass the blood-brain barrier. Usually a lumbar puncture was used as the route of injection, but this method has certain disadvantages. Obliteration of the subarachnoidal space can make repeated injections impossible. Furthermore, a lumbar puncture in the presence of increased intracranial pressure involves severe risks. To meet these risks the authors treated six acute leukaemic patients with cerebrospinal complications. Methotrexate was injected into the lateral ventricle through a Rickham reservoir. This consists of a metal funnel inserted in a bur hole connected with the lateral ventricle by a silicone tube and covered by a silicone membrane. One of the six patients (aged 57) suffered from acute myeloblastic leukaemia with no remission, and died during treatment. Another patient (aged 17) had stem-cell leukaemia, and the treatment was unsuccessful. The remaining four patients were between 5 and 21 years old, had acute lymphoblastic leukaemia and obtained good remissions of several months’ duration. DifficuIties encountered in the treatment were onIy slight and were, in part, referable to the infections complicating the generalized leukaemia. In three of the six patients, cortical biopsies showed leukaemic infiltrations.