The history of progressive muscular atrophy

  title={The history of progressive muscular atrophy},
  author={Jeldican Visser and Jody M. de Jong and M. Visser},
  pages={723 - 727}
Since its first description more than a century ago, there has been much debate about the diagnostic entity progressive muscular atrophy (PMA). Initially, PMA included all forms of progressive amyotrophy. With the identification of several myogenic and neurogenic diseases and the recognition of amyotrophic lateral sclerosis (ALS), PMA was deemed to disappear as a nosologic entity at the end of the 19th century. In the last century, various other lower motor neuron syndromes were distinguished… 

Progressive Muscular Atrophy.

Progressive muscular atrophy and other lower motor neuron syndromes of adults

Determining which patients with PMA actually have UMN dysfunction has therefore become the subject of a flourishing industry of brain imaging and electrodiagnosis, including diffusion tensor imaging, diffusion weighted imaging, positron emission tomography (PET) scanning, magnetic resonance spectroscopy, and transcranial magnetic stimulation to measure conduction velocity in the corticospinal tracts.

Unilateral progressive muscular atrophy with fast symptoms progression.

Lockhart Clarke's contribution to the description of amyotrophic lateral sclerosis.

The definition of the clinicopathological entity of amyotrophic lateral sclerosis evolved over half a century, and several aspects of the exquisite clinical histories documented as part of both studies, one by Charles Bland Radcliffe, resonate with contemporary debates concerning the evolution of disease in amyotoxicity.

The diagnostic dilemma of progressive muscular atrophy.

A 45-year-old female patient who presented with unilateral foot drop and rapidly progressed to profound weakness in muscles of all limbs, neck and back is presented, guided in right direction after muscle biopsy, nerve conduction studies and electromyography.

A long-term prospective study of the natural course of sporadic adult-onset lower motor neuron syndromes.

Patients with lower motor neuron syndromes and a disease duration of at least 4 years usually have a favorable prognosis if muscle involvement has a segmental distribution, and in patients with a generalized phenotype, progression is relentlessly progressive and eventually leads to death due to respiratory insufficiency.

Amyotrophic lateral sclerosis of long clinical course clinically presenting with progressive muscular atrophy

  • T. MatsubaraM. Oda Y. Izumi
  • Biology, Medicine
    Neuropathology : official journal of the Japanese Society of Neuropathology
  • 2019
An atypical case of sporadic ALS that progressed very slowly, persisted for 19 years, and clinically appeared to only affect the lower motor neurons is presented; however, upper motor neuron degeneration was detected at autopsy.

Traces of disease in amyotrophic lateral sclerosis

The findings in the first part of this thesis are in support of disease spread guided by the brain’s network structure, which might provide new targets for disease modifying treatments.

Flail arm syndrome with cytoplasmic vacuoles in remaining anterior horn motor neurons: A peculiar variant of amyotrophic lateral sclerosis

  • Shuji HinoS. Sasaki
  • Medicine, Psychology
    Neuropathology : official journal of the Japanese Society of Neuropathology
  • 2015
This is a unique autopsy case with a long‐standing clinical course that suggests that FA syndrome is an atypical form of ALS.



AMYOTROPHIC LATERAL SCLEROSIS: A Clinicoanatomic Study of Fifty-Three Cases

These conditions form the group known as "motor neuron disease," which also includes some special types characterized by aberrant age of onset (the progressive muscular atrophy of Werdnig and Hoffmann) or involvement of specific nuclei, such as Duchenne's progressive bulbar palsy.

A case of juvenile muscular atrophy confined to one upper limb.

A 25-year-old woman with unilateral paresis and atrophy of the muscles of the right arm and shoulder is described, it is to be expected that the pathological anatomical features will resemble those described by Marie and Foix as tephromalacia.

Mimic syndromes in sporadic cases of progressive spinal muscular atrophy

Patients initially diagnosed with progressive spinal muscular atrophy (PSMA), in whom further evaluation established another diagnosis, are described, in whom a potential treatment was available: multifocal motor neuropathy, chronic inflammatory demyelinating polyneuropathy, inflammatory myopathy, and MG.

Benign focal amyotrophy. Variant of chronic spinal muscular atrophy.

A man whose condition clinically fit the description of benign focal amyotrophy was examined and some evidence suggestive of a more diffuse motor neuron disease was found.

Progressive proximal spinal and bulbar muscular atrophy of late onset

The question of diagnosis is answered by examining the X chromosome and finding expanded cytosine, adenosine, and guanine (CAG) repeats encoding a polyglutamine tract of the androgen receptor protein, similar to CAG repeats of four other degenerative diseases of late onset that affect specific cell types.

Corticospinal tract degeneration in the progressive muscular atrophy variant of ALS

A patient presenting with PMA with rapid clinical evolution likely has the pathology and pathophysiology of ALS whether or not upper motor neuron signs evolve.

Juvenile type of distal and segmental muscular atrophy of upper extremities

Clinical features consisted of juvenile onset, male preponderance, unique distribution of the muscular atrophy in the hand and forearm, tendon reflexes hypoactive in most cases but hyperactive in some, no definite sensory disturbances, no involvement of the cranial nerves, and autonomic nerve disorders in the affected region.

Benign calf amyotrophy: clinicopathologic study of 8 patients.

Benign calf amyotrophy is a variant of the benign focal amyot rophy disorders, and the electromyographic and muscle histopathologic findings were consistent with a chronic neuropathic disorder.