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For example, when cerebellar ataxia, a unilateral Horner syndrome, paralysis of a vocal cord, and analgesia of the face of acute onset are combined with loss of pain and temperature sensation in the opposite arm, trunk, and leg, the most likely cause is an occlusion of the vertebral artery, because all the involved structures can be localized to the lateral medulla, within the territory of this artery Thus, the anatomic diagnosis determines and limits the etiologic possibilities If the signs point to disease of the peripheral nerves, it is usually not necessary to consider the causes of disease of the spinal cord Some signs themselves are almost speci c eg, opsoclonus for paraneoplastic cerebellar degeneration and Argyll Robertson pupils for neurosyphilitic or diabetic oculomotor neuropathy.

Restated, the topography or distribution of involvement tends to be alike in all patients with the same disease Thus, the pattern of weakness is a central diagnostic attribute of muscular disease as it is for the various diseases of the peripheral nervous system discussed in Chap 46, but the con gurations differ in important ways between disease of the muscles and of the nerves The following patterns of muscle involvement constitute a core of essential clinical knowledge in this eld: 1 Ocular palsies presenting more or less exclusively as ptosis, diplopia, and strabismus As a rule, primary diseases of muscle do not involve the pupil, and in most instances their effects are bilateral In lesions of the third, fourth, or sixth cranial nerves, the neural origin is disclosed by the pattern of ocular muscle palsies, abnormalities of the pupil, or both.

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When weakness of the orbicularis oculi (muscles of eye closure) is added to weakness of eye opening (levator palpebrae; ptosis), it nearly always signi es myasthenia gravis and occasionally, a rare primary disease of muscle (progressive external ophthalmopelgia) The most frequent causes of subacute and chronic development of relatively pure affection of the muscles of eye movement are myasthenia gravis, progressive external ophthalmoplegia, oculopharyngeal muscular dystrophy, and exophthalmic (hyperthyroid) ophthalmopathy In progressive external ophthalmoplegia, the muscles including the levators of the eyelids become paralyzed almost symmetrically over a period of years This disorder has been shown in most cases to be a form of mitochondrial myopathy at times coupled with the Kearns-Sayre syndrome noted further on Oculopharyngeal dystrophy involves primarily the levators of the eyelids and, to a somewhat lesser extent, other eye muscles and pharyngeal-upper esophageal striated muscles.

It begins in middle or late adult life and later, and like progressive external ophthalmoplegia tends later to involve girdle and proximal limb muscles There are several other less common chronic myopathies in which external ophthalmoplegia is associated with involvement of other muscles or organs, namely, the congenital ophthalmoplegia of the Goldenhar-Gorlin syndrome (see Aleksic et al); the Kearns-Sayre syndrome (retinitis pigmentosa, heart block, short stature, generalized weakness, and ovarian hypoplasia); other congenital myotubular and mitochondrial myopathies; nuclear ophthalmoplegia with bifacial weakness (Mobius syndrome); and the myo tonic dystrophy of Steinert Rarely, eye muscle weakness may occur at a late stage in other dystrophies, such as the facioscapulohumeral type Although not a regular feature of the disease, ophthalmoparesis can occur in the reverse myasthenic syndrome of Lambert-Eaton Acute bilateral ophthalmoplegia raises an entirely different set of diagnostic considerations.

It may be an expression of the Fisher syndrome that consists of ophthalmoplegia, are exia, and ataxia and of other variants of the Guillain-Barre syndrome, or of botulism, diphtheria, rarely tick paralysis, and of occlusion of the basilar artery or its branches Ptosis is variable in all of these conditions When present in infantile myopathic disease, it is frequently a marker of the congenital myasthenic syndromes Trichinosis is a rare cause, associated also with periorbital edema..

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Irrespective of the intellectual process that one utilizes in solving a particular clinical problem, the fundamental steps in diagnosis always involve the accurate elicitation of symptoms and signs and their correct interpretation in terms of disordered function of the nervous system Most often when there is uncertainty or disagreement as to diagnosis, it will be found later that the symptoms of disordered function were incorrectly interpreted in the rst place Thus, if a complaint of dizziness is identi ed as vertigo instead of light-headedness or if partial continuous epilepsy is mistaken for.

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