Clinical Neuroscience

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

BEKÉNY György1

MAY 01, 1969

Clinical Neuroscience - 1969;22(05)

[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. ]


  1. BOTE Neurológiai Klinika



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[Types of thermal nystagmus induced in healthy individuals ]

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[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.]

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Clinical Neuroscience

[Painful asymbolia]


[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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Lege Artis Medicinae

[LAM 30: 1990–2020. Facing the mirror: Three decades of LAM, the Hungarian medicine and health care system]


Clinical Neuroscience

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Clinical Neuroscience

Comparison of direct costs of percutaneous full-endoscopic interlaminar lumbar discectomy and microdiscectomy: Results from Turkey

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Microdiscectomy (MD) is a stan­dard technique for the surgical treatment of lumbar disc herniation (LDH). Uniportal percutaneous full-endoscopic in­terlaminar lumbar discectomy (PELD) is another surgical op­tion that has become popular owing to reports of shorter hos­pitalization and earlier functional recovery. There are very few articles analyzing the total costs of these two techniques. The purpose of this study was to compare total hospital costs among microdiscectomy (MD) and uniportal percutaneous full-endoscopic interlaminar lumbar discectomy (PELD). Forty patients aged between 22-70 years who underwent PELD or MD with different anesthesia techniques were divided into four groups: (i) PELD-local anesthesia (PELD-Local) (n=10), (ii) PELD-general anesthesia (PELD-General) (n=10), (iii) MD-spinal anesthesia (MD-Spinal) (n=10), (iv) MD-general anesthesia (MD-General) (n=10). Health care costs were defined as the sum of direct costs. Data were then analyzed based on anesthetic modality to produce a direct cost evaluation. Direct costs were compared statistically between MD and PELD groups. The sum of total costs was $1,249.50 in the PELD-Local group, $1,741.50 in the PELD-General group, $2,015.60 in the MD-Spinal group, and $2,348.70 in the MD-General group. The sum of total costs was higher in the MD-Spinal and MD-General groups than in the PELD-Local and PELD-General groups. The costs of surgical operation, surgical equipment, anesthesia (anesthetist’s costs), hospital stay, anesthetic drugs and materials, laboratory wor­kup, nur­sing care, and postoperative me­dication diffe­red significantly among the two main groups (PELD-MD) (p<0.01). This study demonstrated that PELD is less costly than MD.