Physiopathogeny of Moebius syndrome and arthrogriposis due to in utero misoprostrol exposure

Fisiopatogenia da síndrome de Moebius e da artrogripose decorrentes da exposição in utero ao misoprostol

THESES

PHYSIOPATHOGENY OF MOEBIUS SYNDROME AND ARTHROGRIPOSIS DUE TO IN UTERO MISOPROSTROL EXPOSURE (ABSTRACT)^*. THESIS. SÃO PAULO, 1999.

MARIA JOAQUINA MARQUES-DIAS^**

Misoprostol, a PGE1 marketed as Cytotec, has been misused as the most popular abortifacient in Brazil. The drug may determine congenital malformations mainly of the extremities and central nervous system when it fails to cause the abortion.

Neuroclinic, neurophysiologic, imaging and neuropathologic studies of 42 children exposed to the drug in the 1^st trimester of pregnancy, with Moebius syndrome (MS, 31) or arthrogriposis multiplex (AMC, 11) revealed suggestive anomalies that support an embryonic vascular disruption as the main causative mechanism of these abnormalities.

In the MS group, 4 patients presented the syndrome as the sole congenital anomaly; in 19 MS was associated with abnormalities of the extremities; and 8 also had associated congenital hydrocephalus.

All patients with AMC presented other anomalies of the extremities, and 4 associated to congenital hydrocephalus.

Cranial CT revealed brainstem calcifications in 2 patients. Neuropathologic study in 3 patients disclosed bilateral old foci of gliosis, necrosis with calcifications, in the brain stem, bilaterally, situated very close to some cranial nerve nuclei. In 9 patients with AMC electroneuromyographic study revealed a neurogenic pattern and in 8 muscular CT scanning showed marked atrophy and replacement of muscle by adipose tissue. CT in patients with hydrocephalus revealed supratentorial dilatation of ventricles with relative preservation of IV ventricle volume.

The clinical aspects studied allow us to suggest that MS and AMC in these patients would be caused by a transient arterial disturbance related to misoprostol in the territories of the subclavian artery between the 4^th and 6^th or of the spinal arteries until the 12^th weeks, respectively.

KEY-WORDS: misoprostol, Moebius syndrome, congenital arthrogriposis multiplex.

**Address: Rua Frei Caneca 972 / 34, 01307-002 São Paulo SP, Brasil.

*

Fisiopatogenia da síndrome de Moebius e da artrogripose decorrentes da exposição

in utero ao misoprostol (Resumo). Tese de Livre-Docência, Faculdade de Medicina da Universidade de São Paulo (Disciplina de Neurologia Infantil).

Publication Dates

• Publication in this collection
  06 Dec 2000
• Date of issue
  June 2000