Hypokinetic Movement Disorders

Pathological findings of Parkinson's disease (PD) include depigmentation and neuronal loss in the substantia nigra (SN) and the presence of Lewy bodies and pale bodies( Fig 34-1). Because the degenerating cells in the SN normally synthesize the neurochemical dopamine, the pathophysiological hallmark of PD is dopaminergic underactivity at the site of these cells' axonal projection--that is, the striatum (caudate nucleus and putamen). Lewy bodies are eosinophilic...

Other Forms of Chorea

Senile chorea (essential chorea) begins after age 60 and is unaccompanied by any particular neurobehavioral symptoms or a family history of chorea. Whereas some patients have caudate atrophy similar to that seen in HD, others have predominant putaminal degeneration. In occasional cases, genetic detection studies in these patients reveal that the correct diagnosis is actually HD. Treatment of the chorea is similar to that used for HD. Paroxysmal dyskinesia is a chronic condition that becomes...

Compressive Neuropathies

COMPRESSIVE DISORDERS OF THE MEDIAN NERVE Pathogenesis and Pathophysiology. The median nerve is subject to compression at the wrist, where it accompanies the flexor tendons as they pass beneath the volar carpal ligament. This region, the carpal tunnel, is a closed space within which pressure may rise. Studies with recording wicks placed in the canal show that flexion or extension elevate the canal pressure and that thickening of tendon sheaths or encroachment by other structures leads to a...

Other Disorders of the Skull and Spine Dwarfism

Although dwarfism is primarily a defect of the musculoskeletal system, central and peripheral nervous system manifestations are encountered in a variety of these disorders. The most frequent neurological manifestations are a result of impingement of the nervous system as a result of narrowing of bony foramina. For example, in some dwarfism syndromes, such as achondroplasia, narrowing of the foramen magnum can obstruct CSF flow and result in hydrocephalus. In achondroplasia, the development of...

Neurological Applications in Diagnosis and Treatment

Renal osteodystrophy, hyperparathyroidism, and acromegaly represent some of the metabolic and endocrine disturbances that the skull responds to. However, the importance of radiographic findings has decreased with the routine laboratory determination of certain diseases. The skull characteristically demonstrates mottling of the vault, manifesting a salt and pepper appearance. Occasionally, cystlike lesions, or brown tumors, may occur. The findings in renal...

Ankylosing Spondylitis

Ankylosing spondylitis (AS) is one of the group of seronegative forms of arthritis, which also includes Reiter's syndrome and psoriatic arthritis. There is a close association with the HLA-B27 type, and the disease is more than three times more common in men than in women. In many patients, the disease seems to progress slowly for years, primarily with back pain, and then stabilizes without much disability. In some cases the disease progresses to total spinal fusion, producing a typical bamboo...

Degenerative Disc Disease

The vertebral disc is composed of the inner gelatinous nucleus pulposus and the surrounding annulus fibrosus. Vertebral disc herniation refers to rupture of the annulus with displacement of the central nucleus. In youth, the disc is highly elastic. With the passage of time, the direct vascular supply to the vertebrae and discs decreases, and they undergo the accumulated effects of axial loading. The resulting decreases in water and oxygen content, and metabolic...

Episodic Weakness Syndrome

There are a number of unusual syndromes in which weakness is intermittent or fleeting. These are listed in Table 15-15 . Because of the phenomenon of fatigability and recovery after rest the patient with myasthenia gravis may complain of episodic weakness. Myasthenia gravis is seldom confused with these other disorders because of the features mentioned earlier and the predilection of the weakness for the ocular and cranial muscles. An episodic hemiparesis is more apt to lead to confusion. The...