Of 40 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 28 completed a controlled three-month trial of prednisone. Prednisone was shown to cause a small but significant improvement over no treatment in scored neurological disability, some measures of computer-assisted sensory detection threshold, graded muscle strength, and some attributes of nerve conduction. No subset of patients was more likely than another to be responsive to prednisone; those with a progressive course were as likely to be responsive as recurrent cases. This finding provides further justification for classifying progressive with recurrent cases as CIDP and demonstrates that prednisone treatment should not be withheld from patients with progressive disease.

Intensive evaluation of 205 cases of undiagnosed neuropathy in a center with special approaches and facilities permitted classification of 76% of the patients. Inherited disorders accounted for 42% of the series, 21% of the patients were shown to have inflammatory-demyelinating polyradiculoneuropathy, and 13% had neuropathies associated with other disorders. A considerable improvement in diagnosis was possible from evaluation of the kin of the patients with undiagnosed neuropathy. Analysis of the frequency and type of various sensory symptoms also was helpful in distinguishing between acquired and inherited neuropathies.

No abstract

No abstract

Some patients with radiologic findings of neurogenic arthropathy or multiple fractures do not exhibit overt neurologic signs. Results of nerve conduction velocity, computer-assisted sensory examination, periosteal nociception, and morphometric and graded teased-fiber evaluation of cutaneous nerves allowed us to recognize a mild neuropathic abnormality. Neurogenic arthropathy and subclinical neuropathy were also found in relatives. In three kinships, the underlying disorder was probably hereditary sensory neuropathy type 1 and in several others, it was recessively inherited sensory neuropathy. These arthropathies were often painful, and overt loss of superficial and deep pain sensation was not a prominent or necessary condition. An interplay of multiple factors including insensitivity, trauma, obesity, activity, abuse, personality, mental subnormality, and metabolic joint and bone disease are probably involved in the development of the bony lesions and thus provide further evidence that environmental factors affect expression of human mutant genes for inherited neuropathy.