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Postpartal onset of Amyotrophic Lateral Sclerosis (ALS): A case report

Nina Kuppelich, Barbara Tettenborn & Ansgar Felbecker

abstract Introduction: ALS is a rare neurological disorder leading to
degeneration of upper and lower motor neurons. Mean age of
onset ranges from 45 to 65 years and males are affected more
often than females. There are only a few reports of ALS
presenting during pregnancy. We report the case of a 25-year-old
woman who developed first symptoms of ALS in childbed
immediately after delivery of her second child.
Case presentation: One day after uncomplicated spontaneous
vaginal delivery of her second child the 25-year-old woman
noticed muscle weakness of her right hand. The weakness
eventually spread to the right foot and finally to the left hand
within four months. Moreover, the patient developed dysphagia,
dysarthria and forced laughing and crying.
Results: Neurological examination five months after symptom
onset revealed tetraspasticity with hyperreflexia in all limbs,
atrophy of the small hand and foot muscles, fibrillation of the
tongue as well as severe dysarthrophonia. Electromyography
showed signs of widespread denervation in three out of four
regions of the body. Motor evoked potentials (MEP) showed
signs of pyramidal tract lesions to all limbs, whereas
somatosensory evoked potentials (SEP) and sensory nerve
conduction studies revealed normal results. Further examinations
including MRI of brain and spine and extensive laboratory and
CSF testing were performed to rule out other causes of motor
neuron degeneration, all with negative results. Particular
attention was given to exclude immune-mediated diseases like
paraneoplastic disorders. At last, the diagnosis of ALS was
definite according to El Escorial and Awaji criteria. Treatment with
riluzole was started.
Conclusion: To our knowledge, this is the first report of ALS
onset in childbed. Whether this coincidence is a matter of chance
or does reflect a causal link remains unclear to date. It might be
possible that the disease already began during pregnancy
without overt clinical signs. As mentioned above, some reports of
ALS onset in pregnancy exist. Thus, a relationship between
hormonal changes during pregnancy and an increased
susceptibility to ALS may be discussed. Finally, the number of
patients with ALS onset during pregnancy or in childbed is too
small to draw definite conclusions.
   
citation Kuppelich N, Tettenborn B, & Felbecker A (2011). Postpartal onset of Amyotrophic Lateral Sclerosis (ALS): A case report. In SANP (pp. supplement 4 ad 2011;162(4), 12 S).
   
type conference paper/poster (English)
date of conference 4-11-2011
title of proceedings SANP
pages supplement 4 ad 2011;162(4), 12 S
contact Ansgar Felbecker