Nerve conduction studies in adrenomyeloneuropathy.

نویسندگان

  • V Chaudhry
  • H W Moser
  • D R Cornblath
چکیده

OBJECTIVE Adrenomyeloneuropathy (AMN) is an X linked metabolic disorder presenting with progressive spastic paraparesis in the third to fifth decade of life. Although peripheral neuropathy is also present in most patients, prominent pyramidal signs may make its clinical recognition difficult. The objective was to characterise the peripheral neuropathy in patients with AMN by nerve conduction studies. METHODS Nerve conduction studies were performed in 99 men known to have AMN and in 38 heterozygous women, all of whom had neurological disabilities. RESULTS Of the 13 variables obtained, at least one was abnormal in 82% of patients. The abnormalities were more common in men than in women (87% v 67%); in legs than in arms (77% v 38%); in motor than in sensory conduction (80% v 39%); and in latency (distal and F wave) and velocity compared with amplitude (80% v 29%). Twenty six patients had at least one nerve variable value in the demyelinating range. Four variables (sural velocity, peroneal amplitude, peroneal velocity, and peroneal F wave) were correlated with the expanded disability status scale; five variables (peroneal velocity, tibial H reflex, median distal latency, median conduction velocity, and median F wave latency) were correlated with serum very long chain fatty acids (VLCFAs); and two variables (sural amplitude and peroneal distal latency) were more likely to be abnormal in patients with normal adrenal function than in patients with Addison's disease. CONCLUSIONS Nerve conduction studies in patients with AMN are often abnormal and suggest a mixture of axonal loss and multifocal demyelination. Their correlation with disability status and serum VLCFAs suggests that measures from nerve conduction studies may be useful in evaluating future treatments.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

The Relationship between Nerve Conduction Studies and Electromyography Findings in Patients with Carpal Tunnel Syndrome

Background: Carpal tunnel syndrome is the most common neuropathy in the general population. Nerve conduction studies are among the standard methods for diagnosing carpal tunnel syndrome. Electromyography is painful and unpleasant, and if nerve conduction studies can be used to diagnose axonal injury in carpal tunnel syndrome, electromyography might be replaced. Objectives: This study aimed t...

متن کامل

Clinical and Genetic Aspects in Twelve Korean Patients with Adrenomyeloneuropathy

PURPOSE This study was designed to investigate the characteristics of Korean adrenomyeloneuropathy (AMN) patients. MATERIALS AND METHODS We retrospectively selected 12 Korean AMN patients diagnosed by clinical analysis and increased plasma content of very long chain fatty acids. RESULTS All 12 patients were men. Patient ages at symptom onset ranged from 18 to 55 years. Family history was po...

متن کامل

Evaluation of Changes in Nerve Conduction Velocity in the Lower Limbs of Football Players

Background & Aims: Lower limbs’ nerves are exposed to mechanical injuries in athletes (e.g. football players), because of the excessive physical demand of their job. The objective of the present study was to evaluate the influence of regular and intense sports, such as football, on nerves in the lower leg. This study was conducted during 2006 to 2007 in the Shiraz Rehabilitation Faculty, Univer...

متن کامل

Prevalence of Concurrent Disorders of Ulnar Nerve Entrapment at the Elbow and Carpal Tunnel Syndrome Ahvaz Imam Khomeini Hospital During 2009 to 2012

Carpal tunnel syndrome (CTS) is the most well-known and frequent form of median nerve entrapment, and accounts for 90% of all entrapment neuropathies. Entrapment neuropathies of the ulnar nerve are relatively common with ulnar neuropathy at the elbow more prevalent than ulnar neuropathy at the wrist. The diagnosis of ulnar neuropathy at the elbow is usually confirmed in a relatively straight – ...

متن کامل

Normal Values of Median, Ulnar and Radial Nerves Conduction in Healthy Adults (20-59 years)

ABSTRACT The object of this study is determination of normative values of nerve conduction studies (NCS) among the population of Guilan Province. We selected 216 at the ages of 20-59yr on the basis of their medical histories and examination , and on condition that they had no factors or diseases interfering with nerve conduction by convenience sampling method. In this cross-sectional study, t...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Journal of neurology, neurosurgery, and psychiatry

دوره 61 2  شماره 

صفحات  -

تاریخ انتشار 1996