Clinical Neuroscience

[Painful asymbolia]

TEMESVÁRI Péter1

MAY 01, 1969

Clinical Neuroscience - 1969;22(05)

[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.]

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  1. Országos Reuma Fürdőügyi Intézet Rehabilitációs Idegosztály

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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

[Tissue development of muscle spindles]

STIPULA Magda, ABONYI Eszter

[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.]

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[The Effects of the Children’s Temperament and their Parents’ Dental Fear on developing Dental Fear]

APRÓ Zoltán, NÉMETH Anikó

[The aim the present study was to assess the level of dental fear among 5-7 years old children and its correlations with dental-hygienic habits and their temperament. Furthermore, the relationship of the dental fear of parents and children was also analysed. This cross-sectional study was conducted by the means of a self-constructed questionnaire in 2017. The responses of 70 people were analysed with the help of SPSS 22.0; descriptive statistics, 2-sample T-probes, Mann-Whitney probes, analyses of variance and correlations were calculated (p<0.05). High level of dental fear was detected by 30% of the included children. There was no correlation between the dental fear of the parents and their children’s. Dental fear had no effect on the frequency of tooth-brushing. Children’s dental fear had no correlation with either previous painful experiences at the dentist’s or the temperament of the child. Dental fear is present among children and not only family but dental hygienists might play an important role in reducing it. ]

Clinical Neuroscience

REM sleep, REM parasomnias, REM sleep behaviour disorder

SZÛCS Anna , MUTTI Carlotta, PAPP Anikó, HALÁSZ Péter, PARRINO Liborio

We review the literature on REM parasomnias, and their the underlying mechanisms. Several REM parasomnias are consistent with sleep dissociations, where certain elements of the REM sleep pattern emerge in an inadequate time (sleep paralysis, hypnagogic hallucinations and cataplexy) or are absent/partial in their normal REM sleep time (REM sleep without atonia, underlying REM sleep behavior disorder). The rest of REM parasomnias (sleep related painful erection, catathrenia) may have other still unclear mechanisms. REM parasomnias deserve attention, because in addition to disturbing sleep and causing injuries, they may shed light on REM sleep functions as well as the heterogeneous etiologies of parasomnias. One of them, REM sleep behavior disorder has special importance as a warning sign of evolving neurodegenerative conditions mainly synucleinopathies (some cases synucleinopathies themselves) and it is a model parasomnia revealing that parasomnias may have by autoimmune, iatrogenic and even psychosomatic etiologies.

Clinical Neuroscience

[The quality of life of the cluster headache patients during the active phase of the headache]

DIÓSSY Mária, BALOGH Eszter, MAGYAR Máté, GYÜRE Tamás, CSÉPÁNY Éva, BOZSIK György, ERTSEY Csaba

[Introduction - Cluster headache (CH), which affects 0.1% of the population, is one of the most painful human conditions: despite adequate treatment, the frequent and severe headaches cause a significant burden to the patients. According to a small number of previous studies, CH has a serious negative effect on the sufferers’ quality of life (QOL). In the current study, we set out to examine the quality of life of the CH patients attending our outpatient service between 2013 and 2016, using generic and headache-specific QOL instruments. Methods - A total of 42 CH patients (16 females and 26 males; mean age: 39.1±13.5 years) completed the SF-36 generic QOL questionnaire and the headache- specific CHQQ questionnaire (Comprehensive Headache- related Quality of life Questionnaire), during the active phase of their headache. Their data were compared to those of patients suffering from chronic tension type headache (CTH) and to data obtained from controls not suffering from significant forms of headache, using Kruskal-Wallis tests. Results - During the active phase of the CH, the patients’ generic QOL was significantly worse than that of normal controls in four of the 8 domains of the SF-36 instrument. Apart from a significantly worse result in the ‘Bodily pain’ SF-36 domain, there were no significant differences between the CH patients’ and the CTH patients’ results. All the dimensions and the total score of the headache-specific CHQQ instrument showed significantly worse QOL in the CH group than in the CTH group or in the control group. Conclusion - Cluster headache has a significant negative effect on the quality of life. The decrease of QOL experienced by the patients was better reflected by the headache-specific CHQQ instrument than by the generic SF-36 instrument. ]

Clinical Neuroscience

Case report of a woman with anti amphiphysin positive stiff person syndrome

MANHALTER Nóra, GYÖRFI Orsolya, BOROS Erzsébet, BOKOR Magdolna, FAZEKAS Ferenc, DÉNES Zoltán, FABÓ Dániel, KAMONDI Anita, ERÕSS Loránd

Stiff person syndrome is a rare neuroimmunological disease, characterized by severe, involuntary stiffness with superimposed painful muscle spasms, which are worsened by external stimuli. The classical form is associated with high levels of antibodies against glutamic acid decarboxylase. One of the variant forms is associated with antibodies against amphiphysin. This entity is a paraneoplastic syndrome, caused primarily by breast cancer, secondarily by lung cancer. Symptomatic therapy of anti amphiphysin positive stiff person syndrome includes treatment with benzodiazepines and baclofen (including intrathecal baclofen therapy). The effect of immunological therapies is controversial. Treatment of the underlying cancer may be very effective. In this report, we describe a 68 year old female presenting with an unusally rapidly developing anti amphiphysin positive stiff person syndrome, which was associated with breast cancer. Her painful spasms abolished after intrathecal baclofen treatment was initiated. Her condition improved spontaneously and significantly after cancer treatment, which enabled to start her complex rehabilitation and the simultaneous dose reduction of the intrathecal baclofen. The bedridden patient improved to using a rollator walker and the baclofen pump could be removed 18 monthes after breast surgery. This highlights the importance of cancer screening and treatment in anti amphiphysin positive stiff person syndrome cases.

Clinical Neuroscience

Burning mouth syndrome: Evaluation of clinical and laboratory findings

HALAC Gulistan, TEKTURK Pinar, EROGLU Saliha, CIKRIKCIOGLU Ali Mehmet, CIMENDUR Ozlem, KILIC Elif, ASIL Talip

Background and purpose - Burning mouth syndrome is a chronic and persistent painful condition characterized by burning sensation in the oral mucosa. We investigated the etiological factors of patients presented with the history of burning in the mouth who admitted our outpatient clinics over the 8-years period and who had no underlying identifiable local factors. We also tried to determine their demographic and clinical characteristics. Our aim was to investigate the association between burning mouth and psychiatric disorders such as depression and anxiety, chronic diseases like diabetes mellitus (DM) and other laboratory studies in patients complaining of solely burning in the mouth. Methods - The study included patients with the history of burning in mouth who presented in our outpatient clinic between 2005 and 2012. They were evaluated by a neurologist, a psychiatrist, an internist, and a dentist. Complete blood counts, biochemical analysis and cranial magnetic resonance imaging (MRI) were performed for all patients. Results - A total of 26 (22 (84%) females, 4 (15%) males; mean age 55.9 years) patients were enrolled in this study. Five (19.2%) of the patients had depression, 2 (7.7%) had anxiety disorder, 2 (7.7%) had diabetes mellitus, 8 (30%) had B12 vitamin deficiency, 3 (11.5%) had decreased ferritin levels in blood, and 1 (3.8%) had folic acid deficiency. Cranial MRI of all patients were normal. Nine patients (34.6%) had no etiological causes. Conclusion - A multidisciplinary approach in the management of burning mouth and establishment of common criteria for the diagnosis would provide insight into the underlying pathophysiological mechanism.