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Between January 1, 1993 and December 31, 1997, 32 patients (representing 7.3% of 437 referrals) were rediagnosed as having a condition other than ALS. The median age at onset for these 32 patients was 56.0 years (range, 19.5-85.8 years) fo r men and 53.5 years (range, 39.5-70.4 years) for women. Twenty-nine patients (91%) presented with symptoms referable to the limbs, and the remainder presented with symptoms involving the bulbar musculature. Multifocal motor neuropathy was the most comm on condition mistaken for ALS, accounting for 7 cases (22%), followed closely by Kennedy disease (4 cases [13&]). Factors leading to diagnostic revision included evolution of atypical symptoms, results of specific investigations, and failure of symptoms to progress. Twenty-seven (84%) of the patients with an ALS mimic syndrome fulfilled the EL Escorial criteria for either "suspected" or "possible" ALS, 4 (13%) met the criteria for probable ALS, and 1 (3%) had definite ALS. The application of the El Esc orial diagnostic criteria may facilitate early recognition of non-ALS cases. Misdiagnosis of ALS remains a common clinical problem despite the increased availability of investigations and a greater awareness among neurologists of potential diagnostic pit falls. |
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amyotrophic lateral sclerosis
amyotrophic lateral sclerosis,differential diagnosis
amyotrophic lateral sclerosis,misdiagnosis
misdiagnosis
motor neuron disease
neurologic disease,diagnoses of
neuropathy,motor,multifocal
review article
X-linked bulbospinal neuronopathy
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