News about Medicine - Part 187

Amyotrophic Lateral Sclerosis (Lou Gehrig’s Disease) and Other Motor Neuron Diseases

Motor neuron diseases are characterized by weakness and wasting of affected muscles, without accompanying sensory changes. Werdnig-Hoffman disease and Kugelberg-Welander syndrome occur in infants or children. Motor neuron disease in adults generally commences between 30 and 60 years of age. There is degeneration of the anterior horn cells in the spinal cord, the motor nuclei of the lower cranial nerves, and the corticospinal and corticobulbar pathways. The disorder
is usually sporadic, but familial cases may occur.

Classification
Five varieties have been distinguished on clinical grounds.
A. Progressive Bulbar Palsy: Bulbar involvement predominates owing to disease processes affecting primarily the motor nuclei of the cranial nerves.
B. Pseudobulbar Palsy: Bulbar involvement predominates in this variety also, but it is due to bilateral corticobulbar disease and thus reflects upper motor neuron dysfunction.
C. Progressive Spinal Muscular Atrophy: This is characterized primarily by a lower motor neuron deficit in the limbs due to degeneration of the anterior horn cells in the spinal cord.
D. Primary Lateral Sclerosis: There is a purely upper motor neuron deficit in the limbs.
E. Amyotrophic Lateral Sclerosis (Lou Gehrig’s Disease): A mixed upper and lower motor neuron deficit is found in the limbs. This disorder is sometimes associated with dementia or parkinsonism.

Clinical Findings
A. Symptoms and Signs: Difficulty in swallowing, chewing, coughing, breathing, and talking (dysarthria) occur with bulbar involvement. In progressive bulbar palsy, there is drooping of the palate, a depressed gag reflex, pooling of saliva in the pharynx, a weak cough, and a wasted, fasciculating tongue. In pseudobulbar palsy, the tongue is contracted and spastic and cannot be moved rapidly from side to side. Limb involvement is characterized by motor disturbances
(weakness, stiffness, wasting, fasciculations) reflecting lower or upper motor neuron dysfunction; there are no objective changes on sensory examination, though there may be vague sensory complaints. The sphincters are generally spared.
The disorder is progressive and usually fatal within 3–5 years; death usually results from pulmonary infections. Patients with bulbar involvement generally have the poorest prognosis.

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Advances in Medicine

As the history of medicine has evolved, a number of trends and prevailing opinions have swept the profession. One of the most subtle, and yet most revealing results of these sweeping trends manifests itself by altering the tone in medical conversations and dialogues, often available to the non-medical person in the form of texts and literature. A relatively current example appears in the form of Perri Klass’ A Not Entirely Benign Procedure, a text dedicated to the experiences of the author at Harvard Medical School. Published in 1987, Klass’ work offers an interesting, if not shocking comparison to Philippe Pinel’s “The Clinical Training of Doctors,” an article published in 1783. It seems that, despite the obvious advancements and progress in medical technology and general care, the modern Klass presents less certainty about the profession and its abilities than does the eighteenth century article. In Pinel’s article, however, a distinct tone of holistic healing pervades the proposed training of physicians—the lack of which Klass bemoans in her work. The contrast between the two works affords the reader a view into two parallel transitions in medicine: the decline of certainty and the decline of holistic care.

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