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Chap 28 The reader may turn to books on genetics or teratology for an account of such oddities as hereditary unilateral ptosis, hereditary Horner syndrome (see page 464), pupillary inequalities, jaw winking, absence of a particular muscle, etc Bifacial and Abducens Palsies (Mobius Syndrome) The syn drome of congenital facial diplegia with convergent strabismus is generally referred to as Mobius syndrome, although it had been described earlier by von Graefe Its presence at birth is disclosed by the lack of facial movements and of full eye closure The most complete review of the subject in the English literature is that of Henderson, and a recent analysis of 37 affected individuals has been given by Harriette and colleagues.

At rst the condition was thought to be a manifestation of hysteria, and only later was it recognized as a neurologic entity with a predilection for Russian and Polish Jews Soon thereafter, a second hereditary form of torsion dystonia, affecting non-Jews, was observed The epidemiologic study of Eldridge, who analyzed all reported primary cases up to 1970, revealed two patterns of inheritance, one autosomal recessive, the other dominant The recessive form begins in early childhood, is progressive over a few years, and is restricted to Jewish patients with normal and often even superior intelligence The dominant form begins later, usually in late childhood and adolescence, progresses more slowly, and is not limited to any ethnic group As indicated in Chap.

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position, either in cash or margin, have an interest cost over time that is also part of the time value. As time becomes shorter, the interest collectible on those funds over the period before expiration is lower. If the option represents a futures contract on a physical commodity, it is also likely that there will be storage costs and interest on the physical item that can also make up a portion of the time value.

In Henderson s study of 61 cases of the congenital facial diplegia syndrome, there were 45 instances of an associated abducens palsy, 15 of complete external ophthalmoplegia, 18 of lingual palsy, 17 of clubfeet, 13 of a brachial disorder, 6 of mental defect, and 8 of an absent pectoral muscle Thus the overlap with other neuromuscular and CNS abnormalities is evident Harriette and coworkers empha size the frequency of hypoplastic or dysplastic tongue, palatal involvement, and general motor clumsiness They suggest that the disorder represents a widespread form of brainstem maldevelopment Early in life the mouth hangs open, the lower lip is everted, and there is dif culty in sucking Usually this syndrome can be distinguished from the facial palsy of forceps or birth injury by its bilaterality and the other associated weaknesses Occasionally more than one family member is affected (usually autosomal dominant inheritance).

6, most instances of idiopathic (primary) dystonias that come to our attention, particularly the segmental or restricted types, do not conform to the classic hereditary disorders as de ned above, although some may represent limited variants In general, these more restricted types have a later onset and a relatively milder, more slowly progressive course, with a tendency to involve the axial or the distal musculature alone Only the paravertebral, cervical, or cranial muscles may be involved (focal dystonia including torticollis and writer s cramp), with little change from year to year The clinical classi cation of the predominantly adult-onset dystonias is made more complex by the fact that both the restricted and generalized forms may be sporadic or familial Bressman and colleagues, for example, have described a restricted (cervicocranial) adult-onset form of dystonia in four generations of a non-Jewish family The symptomatology included cervical dystonia, facial grimacing, dysarthria, and dysphonia.

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