[Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos)]. / Dysplastický gangliocytom mozecku (Lhermitte-Duclos).

In a surgical specimen from a 33-year old woman with features of expansive cerebellar symptomatology, dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos) was diagnosed by histology. The lesion was characterized by typical proliferation of dysplastic neuronal elements in the cortical granular and Purkinje cell layers. Immunohistology showed focal positivity of synaptophysin in some cells, thus confirming their neuronal origin. In electron microscopy, peculiar features of ER in some of dysplastic cells was also noted. Genetic examination proved familiar incidence of Cowden's disease in several members. Chromosomal examination of the patient (karyotype 46, XX) was without abnormalities.

Correlation of histopathological and clinical findings in neuromuscular diseases.

Our file comprises bioptic and necroptic materials of skeletal muscles: progressive spinal muscle atrophy of adults 16, amyotrophic lateral sclerosis 12, infantile progressive spinal amyotrophy Werdnig-Hoffmann 9, chronic progressive amyotrophy Oppenheim 6, syringomyelia and syringobulbia 3, conditions after spinal cord accidents. Diagnosis was difficult because one time the neurological marks were at the head and other time the muscular ones. In the clinical picture there was muscle weakness and atrophy, the spontaneous and palpation painfulness was missing. Muscle weakness und its intensity did not correspond to the degree of muscle atrophies. The discrepancies of electromyographic and histopathologic pictures are explained by occurrence of so-called concomitant myopathies, which arise most probably from the disorders of histioceptive-proprioceptive system.

Histopathological changes in paravertebral muscles by chronic discopathies.

Samples of paravertebral muscles were obtained from the lesion sites of forty patients operated on for recurrent lumboischiadic syndrome of discogenic etiology. None of the specimens was physiological. All of them were pathologically altered in various grades of involvement. The histopathological changes were highly varied. The findings included muscle fibre atrophy of a nonspecific type, neurogenic atrophy of typical fascicular distribution. Apart from atrophic fibres there were hypertrophic ones either at the same time or in connection with another histopathological process. Changes of myopathic nature were also present with rounded up muscle fibres and shift of nuclei from the subsarcolemnic spaces into the centre of the muscle fibre, multiplication of the connective tissue and vicarious growth of adipose tissue. The visible neuromuscular spindles contained thickened connective tissue capsules and atrophied intrafusal fibres. The present authors' conclusion of their interpretation of these histopathological changes is that they are not produced by one but by a whole set of factors. What results is a cyclic nature of the changes, when it is hard to decide whether we are facing a primary cause or its sequela. The regular company of discopathies are repeated microtraumas during recurrent paravertebral contractures and spinal blocks. A not negligible part can be played also by primary muscle diseases affecting, among others, back muscles, e.g. progressive muscular dystrophy of Duchenne's and Becker's type, various myopathies, nonspecific myositis and paravertebral polymyositis, and other rheumatological involvement. These muscular diseases with disturbed proprioception causing unphysiological posture and loading the spine can share in the origin degenerative processes on intervertebral disks.

Paravertebral polymyositis.

Pains in the back are usually of vertebrogenic etiology, though such discomfort can be caused by a primary muscular involvement as well - polymyositis of paravertebral muscles. Five patients were studied in the present investigation with this quite rare disease. Four of them had the diagnosis confirmed by histology, the last patient could not have the biopsy performed as she was already on a successful corticosteroid therapy. Histopathology of the muscles demonstrated a polymyositis of the parenchymatous type. Owing to a considerable polymorphism showed by the troubles and often also coincidence with the vertebrogenic component, the diagnosis and classification of primary chronic polymyositis face a series of difficulties. The disease becomes clinically manifested by muscle pain, both spontaneous and on palpation, atrophies and frequent muscle contractions. These symptoms can then result in disorders affecting the dynamics and statics of the spine. The possible participation of repeated microtraumas as one of the factors contributing to the origin of polymyositis is also discussed on the present study. The disease is treated in the same way as most of the other collagenoses, the primary chronic forms showing a much more favourable prognosis than the secondary chronic ones developing from acute and subacute forms.

Thyroid surgery in advanced age.

The total of 1,931 patients had been operated on in the Second Department of Surgery, Faculty Hospital and Policlinic, in Olomouc for thyroid disorders. The present paper deals with the operation on the thyroid gland in advanced age. The symptoms of the disease, types of interventions and kinds of complications, factors influenced by age and the secondary diseases are pointed out. In these terms, a group of 69 patients older than 60 and operated on within the period of 1981-1987 are analyzed in detail.

Oesophageal diverticula.

The classification and etiopathogenesis of oesophageal diverticula is discussed, based on the experience with the treatment of 91 (68 Zenker's, 7 parabronchial, and 16 epiphrenic) diverticulectomies performed at the First and Second Departments of Surgery in Olomouc from 1948 to 1987. The tactics and technique of surgical management and its results in the single groups of diseases are analyzed in detail. Parapharyngeal diverticula represent typical true diverticula associated with hypertonia of the upper oesophageal sphincter. No causal relationship could be proved to exist between the origin of this diverticulum and the gastrooesophageal reflux. Thoracic diverticula are probably rather of congenital than traction origin. Epiphrenic diverticula arise most often from hypertonia of the lower oesophageal sphincter. They can also be of congenital origin. Traction etiology could be observed in the presence of a leiomyoma in the diverticulum. The surgical management of Zenker's diverticulum consists in the excision of the diverticulum and cricopharyngeal myotomy. Excision of parabronchial diverticulum is seldom indicated for causing little discomfort, as a rule only when it is very large. Epiphrenic diverticula are usually associated with hypertonia of the lower oesophageal sphincter. In such cases, myotomy after Heller is performed and, as a rule, the diverticulum is excised.

[Alcoholic myopathy]. / Alkoholické myopatie.

Six cases of alcoholic myopathy (two acute and four chronic) are reported. Histologically, cases of acute alcoholic myopathy showed dystrophic changes of the sarcoplasm (especially in the muscles of lower extremities), ending with total rhabdomyolysis. In chronic cases, histology revealed selective atrophy of muscle fibers of the 2nd type. Both direct and indirect effects of alcohol are discussed in the pathogenesis as well as the metabolic disturbances caused by acetaldehyde, a metabolic product of ethanol. Possible predisposition factors are taken into consideration and the differential diagnosis mainly between alcoholic myopathy and the clinically heterogeneous group of myopathies is surveyed.

Etiopathogenesis and classification of esophageal diverticula.

The classification and etiopathogenesis of esophageal diverticula is discussed on the basis of our experience with the surgical management of 74 such diverticula (51 parapharyngeal, 7 thoracic, 167 epiphrenic). Parapharyngeal diverticula always represent true diverticula, their walls comprising muscular tissue as well as mucosa and submucosa. A causal relationship between esophageal reflux disease and Zenker's diverticulum has not yet been proved. Thoracic diverticula are probably more often of a congenital than traction origin. Epiphrenic diverticula result either from hypertonia of the lower esophageal sphincter (esophageal achalasia) or, if the latter functions normally, are of congenital origin. Traction etiology can in special cases be due to the presence of a leiomyoma in the esophageal wall.