3 edition of Abstract of a clinical lecture on a case of the juvenile form of progressive muscular atrophy (Erbs dystrophia muscularis progressiva) found in the catalog.
|Statement||by James Stewart|
|Series||CIHM/ICMH Microfiche series = CIHM/ICMH collection de microfiches -- no. 44615, CIHM/ICMH microfiche series -- no. 44615|
|The Physical Object|
|Pagination||1 microfiche (10 fr.).|
|Number of Pages||10|
Juvenile Muscular Atrophy of Distal Upper Extremity (Hirayama Disease) Keizo Hirayama Abstract This disease is characterized by initially progressive mus-cular weakness and wasting of the distal upper limb(s) in young people predominantly in men, followed by a sponta-neous arrest within several years. This disease has been. View This Abstract Online; Predictability of the clinical course of juvenile dermatomyositis based on initial muscle biopsy: a retrospective study of 72 patients. (DM) is in doubt, a case has not been made for routine muscle biopsy (MB). We sought to determine whether MB findings prior to systemic therapy have prognostic value.
Skeletal muscle atrophy attributable to muscular inactivity has significant adverse functional consequences. While the initiating physiological event leading to atrophy seems to be the loss of muscle tension and a good deal of the physiology of muscle atrophy has been characterized, little is known about the triggers or the molecular signaling events underlying this process. Clinical variants of the disease may initially affect only the spinal lower motor neurones (progressive muscular atrophy variant); only the upper motor neurones (primary lateral sclerosis variant); or only the bulbar musculature (progressive bulbar palsy variant). With disease progression, the majority of patients will develop features of ALS.
to denote a characteristic symptom or syndrome present in a medical condition – as, for instance, in juvenile myoclonic epilepsy (JME), spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME), myoclonic epilepsy in infancy (MEI), etc. to denote a class of (six) disorders, termed jointly "progressive myoclonus epilepsies". A diagnosis of Huntington's disease may come as quite a shock. There's a lot to take in. But tapping into a support system, such as a social worker, therapist, or support group, can make the.
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Add tags for "Abstract of a clinical lecture on a case of the "juvenile form" of progressive muscular atrophy (Erb's "dystrophia muscularis progressiva")". Be the first. Similar Items. When Howard Tooth returned to the topic of progressive muscular atrophy in what amounts to a wide-ranging book review of the kind for which Brain has always had a reputation, he aims at a comprehensive account of the clinical features and pathological basis for ‘the disease, or rather group of diseases, known as Progressive Muscular Atrophy.
Abstract of a clinical lecture on a case of the "juvenile form" of progressive muscular atrophy (Erb's "dystrophia muscularis progressiva") [electronic resource] / By James Stewart.
Abstract 'Reprinted from the "Canada Lancet." September, 'Cover onic of access: Internet.4Author: James Stewart. The classical form of severe spinal muscular atrophy (SMA type 1; Werdnig-Hoffmann disease) has a very consistent clinical phenotype that is well recognized by paediatricians.
Progressive muscular atrophy (PMA) is a form of motor neuron disease, but its outcome is not well defined, and the aim was to study the pattern of clinical progression in PMA.
We studied Introduction: Juvenile muscular atrophy of the distal upper extremities (JMADUE) is a rare, sporadic disorder that affects adolescent males and is characterized by progressive but self‐limited weakness of the distal upper etiology is unknown, but cervical hyperflexion has been hypothesized.
Methods: We report a case of an adolescent male who presented with typical JMADUE but. A clinical and genetic study of spinal muscular atrophy of adult onset: the autosomal recessive form as a discrete disease entity. Pearn JH, Hudgson P, Walton JN. Brain, (4), 01 Dec Cited by 33 articles | PMID: The clinical progression was clearly that of progressive muscular atrophy and not of spinal muscular atrophy.
Also in the late nineteenth century emerged a new diagnosis - "chronic polio- myelitis". A search of the classic literature of infantile paralysis*. In Dr. Roth started his studies of progressive muscular atrophy, as suggested to him by Prof.
Kozhevnikov. In Dr. Roth gave the information in the Moscow Medical Newspaper about patient K., aged 22 (Fig. 3) with muscle affection similar to Duchenne's progressive muscular atrophy of childhood. After the patient's death of lung. An accepted nomenclature also defines and limits a syndrome.
Several of Charcot's predecessors had also recognised the clinical features of ALS, for example, the Edinburgh surgeon Sir Charles Bell (–), Aran (–) who probably first recognised progressive muscular atrophy (PMA) and Cruveilhier himself.
Twenty-seven patients with juvenile nonprogressive muscular atrophy localized in the hand and forearm were analyzed. The clinical characteristics were juvenile male occurrence, insidious onset, specific distribution of localized muscular atrophy and a stationary course. On electromyography, denervation voltage (or giant NMU) is found in the atrophied muscles and sometimes in contralateral.
Occurrence of nonprogressive juvenile-onset SMA primarily in males manifesting in peripubertal period, strict topographic localisation of symptoms and signs and its occurrence in brothers, and twins suggests a role of genetic factor in the pathogenesis.
Spinal muscular atrophy is clinically and genetically heterogeneous. Spinal muscular atrophy is a genetic disorder characterized by weakness and wasting (atrophy) in muscles used for movement (skeletal muscles).It is caused by a loss of specialized nerve cells, called motor neurons that control muscle movement.
The weakness tends to be more severe in the muscles that are close to the center of the body (proximal) compared to muscles away from the body's center. INTRODUCTION. Juvenile dermatomyositis (DM) commonly involves skin in diagnostic patterns, skeletal muscle, and occasionally the gastrointestinal tract or other organs (1, 2).Although the morbidity and mortality of juvenile DM improved after introduction of corticosteroid therapy, the long‐term outcome of treated juvenile DM differs substantially from patient to patient ().
Feline X-linked MD - A hypertrophic form of X-linked MD has been described in young domestic shorthair cats. 34,35 In contrast to the atrophy present in dogs, the disease in cats is characterized by muscular hypertrophy with potentially lethal complications.
36 Clinical signs are present at approximately 3 months of age and include muscular. Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by a combination of symptoms that affect both the autonomic nervous system (the part of the nervous system that controls involuntary action such as blood pressure or digestion) and movement.
After taking a clinical history and performing a brief. 8 Hirayama K. Non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama's disease). In: Vinken PJ, Bruyn GW, Klawans HL, DeJong JMBV, eds Diseases of Motor System, Vol.
59 (Handbook of Clinical Neurology.) Amsterdam; Elsevier Science Publishers tion. Progressive JSSMA may be categorized in the clinical spectrum between the spinal muscular atro- phies and amyotrophic lateral sclerosis.
Liu GT, Specht LA. Progressive juvenile segmental spi- nal muscular atrophy. Pediatr Neurol ; neurogenic basis of the weakness; nerve conduction stud- ies are normal.
Juvenile muscular atrophy of the distal upper extremity (JMADUE, Hirayama disease) was first reported in as ‘juvenile muscular atrophy of unilateral upper extremity’. Since then, similar patients in their teens or 20s have been described, under a variety of names, not only in Japan, but also in other Asian countries, as well as Europe.
Hirayama K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease). Intern Med. Hirayama K, Tokumaru Y. Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology.
Hashimoto O, Asada M, Ohta M, Kuroiwa Y. Clinical observations of juvenile nonprogressive muscular atrophy localized in hand and forearm.
J Neurol. Jan 14; (2)– Hirayama K. [Juvenile non-progressive muscular atrophy localized in the hand and forearm--observations in 38 cases]. Rinsho Shinkeigaku. Jul; 12 (7)– Kikuchi S, Tashiro K. Juvenile muscular atrophy of distal upper extremity (Hirayama’s disease).
In: Jones Jr HR, De Vivo DC, Darras BT, editors. Neuromuscular disorders of infancy, childhood, and adolescence: a Clinician’s approach. 1st ed. Philadelphia: Butterworth-Heinemann; p.
– Google Scholar.Progressive Muscular Atrophy—Tumor of the Cerebellum—The Pathology of Spasm—Death of Dr. John C. Dalton—The Middleton Goldsmith Lecture.
Dr. Landon Carter Gray, who has recently been elected Chairman of the Section on Neurology of the Academy of Medicine, presented at the last meeting of the Section a remarkable case of Progressive.