Clinical Neuroscience

[Tissue development of muscle spindles]

STIPULA Magda1, ABONYI Eszter2

MAY 01, 1969

Clinical Neuroscience - 1969;22(05)

[We studied muscle spindles from fetuses and infants of different ages, from 2-month-old embryos to 6-month-old infants. Our observations are summarised below: 1. intrafusal fiber formation from the myotube stage begins in humans at embryonic month 2. 2. 3. The formation of nuclear sac fibres is initiated in the 3rd fetal month in connection with further proliferation and aggregation of the nuclei. 4. The connective tissue sheath of the muscle spindle begins to form in the 4th fetal month. 5. In the 6th fetal month, the innervational pattern of the muscle spindles corresponds to the so-called simpla muscle spindles. In the newborn, so-called "complex" muscle spindles are found, where the subnerual apparatus of the intrafusal fibres is also clearly visible. 7. Intrafusal fibres in foetus and infant have a larger diameter than extrafusal fibres. 8. In adult human muscle spindles, intrafusal fibres were found only under abnormal conditions.]

AFFILIATIONS

  1. Budapesti Orvostudonányi Egyetem I. sz. Neurológiai és Psychiatriai Klinika
  2. Budapesti Orvostudományi Egyetem I. sz. Neurológiai és Psychiatriai Klinika

COMMENTS

0 comments

Further articles in this publication

Clinical Neuroscience

[Three cases of juvenile pseudomyopathy of spinal muscular atrophy (Kugelberg-Welander type)]

BEKÉNY György

[The author described a juvenile pseudomyopathic (Kugelberg-Welander) form of spinal muscular atrophy (SI) in 3 cases. Juvenile SI can be differentiated from progressive dystrophia musculorum by the presence of muscle fasciculopathies and neurogenic EMG and muscle biopsy findings. The recognition of pseudomyopathic SI is of practical importance because of its much better prognosis than muscular dystrophy. Kugelberg-Welander juvenile and Werdnig-Hoffmann infantile SI cannot be considered as separate genetic types. In our case 2, SI started at the age of 11 years. In the muscle biopsy at 14 years of age, in addition to neurogenic atrophy, we found so-called myopathic lesions and lymphorrhagia. The relationship between these three types of pathophysiological syndromes is left to conjecture. Primary muscle lesions are considered to be independent of SI. The latter, i.e. lymphorrhagias and muscle fibre degeneration, may have a common (autoimmune?) pathomechanism. In our second case, during the 4-year follow-up, physiotherapy and exercise resulted in an increase in muscle strength instead of the previous permanent decrease in muscle strength. In our 3rd case, juvenile SI was associated with myositis. Presumably it was a coincidence of the two lesions. Author summarizes in a table the data used to distinguish between the ascending form of Kugelberg-Welander SI and dystrophia musculorum progressiva. ]

Clinical Neuroscience

["Gangliocytoma" cerebelli ]

PÁSZTOR Emil, SLOWIK Felicia

[We describe a rare case of a so-called "gangliocytoma cerebelli". We discuss the clinical picture and the possibilities of an effective surgical solution. Based on our pathological studies, we consider this lesion, which is still controversial in the literature, to be a hamartoblastoma. ]

Clinical Neuroscience

[Types of thermal nystagmus induced in healthy individuals ]

BODÓ György

[The author studied 71 healthy individuals with an electronystagmograph wall and the types of nystagmus induced by thermal stimulation. Five basic types were identified, as follows: 1. regular reactio, where the amplitude and frequency of nystagmus are moderate, 2. weak reactio, where the nystagmus strokes are rare and the amplitude is small. This group also includes fibrillation and floating movements of the eyeball. 3. large reactio, with a high frequency of nystagmus beats and a rapid frequency, 4. uneven reactio, the frequency and amplitude of the strokes are uneven, 5. clustering, in which the nystagmus is interrupted by pauses. The author considers it possible that by further study of the typus of the thermal nystagmus, useful information about the functional typus and the instantaneous state of the central nervous system can be obtained.]

Clinical Neuroscience

[Painful asymbolia]

TEMESVÁRI Péter

[Our 13-year-old right-handed patient presented with the following symptoms after a left centroparietal regio lesion: right homonymous inferior quadrans hemianopia; right hemiparesis; right hemihypaesthesia and hemihypalgesia (needle prick was marked as sharp all over the body); amnestic aphasia; acalculia, alexia; constructive apraxia; painful (danger) asymbolia. In the face of painful stimuli, his vegetative reactions were preserved, his motor reactions were virtually absent, his behavioural responses were pale, his psychic reactions and experience were paradoxically ambivalent: the latter may be taken as the dominant symptom of pain asymbolia. Nor did the patient fully grasp the danger of the situation. The pain asymbolia appears to be a specific disorder of categorical behaviour; it is a local sign, indicating damage to the dominant parietal lobe.]

All articles in the issue

Related contents

Clinical Neuroscience

Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Lege Artis Medicinae

[Vaccines against COVID-19 pandemic]

FALUS András, SZEKANECZ Zoltán

[The rapidly spreading SARS-CoV2 respiratory virus has evoked an epidemic with serious aftermath around the world. In addition to the health effects, the global economic damage is actually unpredictable. At the same time, the pandemic has launched a series of unprecedented collaborative scientific research, including the development of vaccines. This study summarizes up-to-date information on vaccines, immune memory, and some emerging clinical effects.]

Clinical Neuroscience

Atypical presentation of late-onset Sandhoff disease: a case report

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

Sandhoff disease is a rare type of hereditary (autosomal recessive) GM2-gangliosidosis, which is caused by mutation of the HEXB gene. Disruption of the β subunit of the hexosaminidase (Hex) enzyme affects the function of both the Hex-A and Hex-B isoforms. The severity and the age of onset of the disease (infantile or classic; juvenile; adult) depends on the residual activity of the enzyme. The late-onset form is characterized by diverse symptomatology, comprising motor neuron disease, ataxia, tremor, dystonia, psychiatric symptoms and neuropathy. A 36-year-old female patient has been presenting progressive, symmetrical lower limb weakness for 9 years. Detailed neurological examination revealed mild symmetrical weakness in the hip flexors without the involvement of other muscle groups. The patellar reflex was decreased on both sides. Laboratory tests showed no relevant alteration and routine electroencephalography and brain MRI were normal. Nerve conduction studies and electromyography revealed alterations corresponding to sensory neuropathy. Muscle biopsy demonstrated signs of mild neurogenic lesion. Her younger brother (32-year-old) was observed with similar symptoms. Detailed genetic study detected a known pathogenic missense mutation and a 15,088 base pair long known pathogenic deletion in the HEXB gene (NM_000521.4:c.1417G>A; NM_000521:c.-376-5836_669+1473del; double heterozygous state). Segregation analysis and hexosaminidase enzyme assay of the family further confirmed the diagnosis of late-onset Sandhoff disease. The purpose of this case report is to draw attention to the significance of late-onset Sandhoff disease amongst disorders presenting with proximal predominant symmetric lower limb muscle weakness in adulthood.

Hypertension and nephrology

[Association between cyclothymic affective temperament and hypertension]

NEMCSIK János, BATTA Dóra, KŐRÖSI Beáta, RIHMER Zoltán

[Affective temperaments (cyclothymic, hypertymic, depressive, anxious, irritable) are stable parts of personality and after adolescent only their minor changes are detectable. Their connections with psychopathology is well-described; depressive temperament plays role in major depression, cyclothymic temperament in bipolar II disorder, while hyperthymic temperament in bipolar I disorder. Moreover, scientific data of the last decade suggest, that affective temperaments are also associated with somatic diseases. Cyclothymic temperament is supposed to have the closest connection with hypertension. The prevalence of hypertension is higher parallel with the presence of dominant cyclothymic affective temperament and in this condition the frequency of cardiovascular complications in hypertensive patients was also described to be higher. In chronic hypertensive patients cyclothymic temperament score is positively associated with systolic blood pressure and in women with the earlier development of hypertension. The background of these associations is probably based on the more prevalent presence of common risk factors (smoking, obesity, alcoholism) with more pronounced cyclothymic temperament. The scientific importance of the research of the associations of personality traits including affective temperaments with somatic disorders can help in the identification of higher risk patient subgroups.]

Lege Artis Medicinae

[Second game, 37th move and Fourth game 78th move]

VOKÓ Zoltán

[What has Go to do with making clinical decisions? One of the greatest intellectual challenges of bedside medicine is making decisions under uncertainty. Besides the psychological traps of traditionally intuitive and heuristic medical decision making, lack of information, scarce resources and characteristics of doctor-patient relationship contribute equally to this uncertainty. Formal, mathematical model based analysis of decisions used widely in developing clinical guidelines and in health technology assessment provides a good tool in theoretical terms to avoid pitfalls of intuitive decision making. Nevertheless it can be hardly used in individual situations and most physicians dislike it as well. This method, however, has its own limitations, especially while tailoring individual decisions, under inclusion of potential lack of input data used for calculations, or its large imprecision, and the low capability of the current mathematical models to represent the full complexity and variability of processes in complex systems. Nevertheless, clinical decision support systems can be helpful in the individual decision making of physicians if they are well integrated in the health information systems, and do not break down the physicians’ autonomy of making decisions. Classical decision support systems are knowledge based and rely on system of rules and problem specific algorithms. They are utilized widely from health administration to image processing. The current information revolution created the so-called artificial intelligence by machine learning methods, i.e. machines can learn indeed. This new generation of artificial intelligence is not based on particular system of rules but on neuronal networks teaching themselves by huge databases and general learning algorithms. This type of artificial intelligence outperforms humans already in certain fields like chess, Go, or aerial combat. Its development is full of challenges and threats, while it presents a technological breakthrough, which cannot be stopped and will transform our world. Its development and application has already started also in the healthcare. Health professionals must participate in this development to steer it into the right direction. Lee Sedol, 18-times Go world champion retired three years after his historical defeat from AlphaGo artificial intelligence, be­cause “Even if I become the No. 1, there is an entity that cannot be defeated”. It is our great luck that we do not need to compete or defeat it, we must ensure instead that it would be safe and trustworthy, and in collaboration with humans this entity would make healthcare more effective and efficient. ]