Fibrositis (Fibromyalgia)

Fibrositis (also called fibromyalgia) affects about 3–10% of the population, making it one of the most common rheumatic syndromes in general medicine. It shares many features with the chronic fatigue syndrome, namely, an increased frequency among women aged 20–50, absence of objective findings, and absence of diagnostic laboratory tests. While many of the clinical features of the two conditions overlap, musculoskeletal pain predominates in fibrositis whereas lassitude dominates the chronic fatigue syndrome.

The cause is unknown, but sleep disorders, depression, viral infections, and aberrant perception of normal stimuli have all been proposed. Fibrositis can be a complication of hypothyroidism, rheumatoid arthritis, or, in men, sleep apnea.

Clinical Findings

The patient complains of chronic aching pain and stiffness, frequently involving the entire body but with prominence of pain around the neck, shoulders, low back, and hips. Fatigue, sleep disorders, subjective numbness, chronic headaches, and irritable bowel symptoms are common. The patient feels incapable of performing normal activities, and even minor exertion aggravates pain and increases fatigue. Patients occasionally trace the onset of symptoms to an acute event or viral-like illness. Physical examination is normal except for “trigger points” of pain produced by palpation of various areas such as the trapezius, the medial fat pad of the knee, and the lateral epicondyle of the elbow. Read the rest of this entry »

Multiple Sclerosis

Multiple sclerosis is initially characterized by weakness, numbness, tingling, or unsteadiness in a limb; spastic paraparesis may follow. This common neurologic disorder of unknown cause has its greatest incidence in young adults.
Epidemiologic studies indicate that multiple sclerosis is much more common in persons of western European lineage who live in temperate zones. No population with a high risk for multiple sclerosis exists between latitudes 40 °N and 40 °S.
Genetic, dietary, and climatic factors cannot account for these differences. There may be a familial incidence of the disease, since affected relatives are sometimes reported. The strong association between multiple sclerosis and specific HLA antigens (HLA-DR2) provides support for a theory of genetic predisposition. Many believe that the disease has an immunologic basis. Pathologically, Focal-often perivenular-areas of demyelination with reactive gliosis are found scattered in the white matter of brain and spinal cord and in the optic nerves.

Clinical Findings
A. Symptoms and Signs: The common initial presentation is weakness, numbness, tingling, or unsteadiness in a limb; spastic paraparesis; retrobulbar neuritis; diplopia; disequilibrium; or a sphincter disturbance such as urinary urgency or hesitancy. Symptoms may disappear after a few days or weeks, although examination often reveals a residual deficit.
In most patients, there is an interval of months or years after the initial episode before new symptoms develop or the original ones recur. Eventually, however, relapses and usually incomplete remissions lead to increasing disability, with weakness, spasticity, and ataxia of the limbs, impaired vision, and urinary incontinence. The findings on examination at this stage commonly include optic atrophy, nystagmus, dysarthria, and pyramidal, sensory, or cerebellar deficits in some or all of the limbs. Read the rest of this entry »

Benign Essential (Familial) Tremor

The cause of benign essential tremor is uncertain, but it is sometimes inherited in an autosomal dominant manner. Tremor may begin at any age and is enhanced by emotional stress. The tremor usually involves one or both hands, the head, or the hands and head, while the legs tend to be spared. Examination reveals no other abnormalities. Ingestion of a small quantity of alcohol commonly provides remarkable but short-lived relief by an unknown mechanism.

Although the tremor may become more conspicuous with time, it generally leads to little disability, and treatment is often unnecessary. Occasionally, it interferes with manual skills and leads to impairment of handwriting. Speech may also be affected if the laryngeal muscles are involved. In such circumstances, propranolol may be helpful but will need to be continued indefinitely in daily doses of 60–240 mg. However, intermittent therapy is sometimes useful in patients whose tremor becomes exacerbated in specific predictable situations. Primidone may be helpful when propranolol is ineffective, but patients with essential tremor are often very sensitive to it. They are therefore started on 50 mg daily, and the daily dose is increased by 50 mg every 2 weeks depending on the response; a maintenance dose of 125 mg three times daily is commonly effective. Occasional patients fail to respond to these measures but are helped by alprazolam (up to 3 mg daily in divided doses).

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.