A Great Conversation With Dr. James Corbett.

• The prognosis for vision in most patients with pseudotumor cerebri is excellent; however, visual loss, which is the only serious complication, may occur either early or late in the course of the disease. A group of 57 patients was followed up five to 41 years with visual fields, visual acuity, and fundus photographs. Blinding visual loss or severe visual impairment in one or both eyes occurred in 14 patients, and in seven patients, this occurred months to years after the initial symptoms. Systemic hypertension was a significant risk factor for visual loss in patients with pseudotumor cerebri, and blindness occurred in eight of 13 patients who were hypertensive. Despite suggestions that blind spot measurement is useful for following up patients with this condition, we believe that sequential quantitative perimetry gives more complete information and is essential to rational decision making in the treatment of pseudotumor cerebri.

Visual Loss in Pseudotumor Cerebri: Follow-up of 57 Patients From Five to 41 Years and a Profile of 14 Patients With Permanent Severe Visual Loss

It is an honor to be asked to deliver the Silversides Lecture. Dr. Silversides’ clear understanding of the need for the collaboration of the basic and clinical sciences is a measure of the good sense for which he is admired. For a clinician, the greatest challenge to his clinical judgment lies in formulation of strategies to deal with vexing and serious conditions. Often we are called upon to treat conditions for which no satisfactory pathophysiologic mechanism has been elucidated. It is said that the clinician must frequently “get on top of the problem before he gets to the bottom of it.” In the spirit of all clinicians who seek better clinical approaches to poorly understood disease, I will devote this discussion to the diagnosis and treatment of pseudotumor cerebri.

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The 1982 Silversides lecture. Problems in the diagnosis and treatment of pseudotumor cerebri.

THE UNUSUAL SYNDROME of congenital facial diplegia with restriction of horizontal eye movements has been the subject of speculation and interest which has continued to be active since the first description by A. von Graefe in 1880.' Although this syndrome has been described under such headings as congenital facial diplegia, oculofacial paralysis, congenital paralysis of the sixth and seventh nerves, and nuclear agenesis, it does not lend itself to a brief accurately descriptive term. The designation of MObius2.3 as the eponym is justified, since he first suggested that congenital bi€ateral abducens-facial paralysis might be an independent pathologic entity. This he did after comparing his original case with that of von Graefe and that of Chisholm.4 Later he described other cases of cranial nerve palsies under the presumptive term of i n f a n t h Kentschwund. He did not clearly distinguish between congenital and acquired conditions, and his concept of pathogenesis, that is nuclear degeneration, has not been supported by the meager pathologic studies thus far available. Most recent authors would agree with the following description: The Mobius syndrome involves a variable degree of facial paralysis with inability to abduct the eyes beyond the midpoint. Partial atrophy of the tongue and other deformities about the face and head are often present. Paralysis of the soft palate and muscles of mastication may occur. Absence of pectoral muscles, talipes, syndactyly, and other extremital deformities are common. Mental deficiency occasionally accompanies the physical defects, but signs related to long-fiber tract involvement are seldom seen. The condition is congenital and nonprogressive. Henderson6 and Danise have tabulated the various deformities and the relative frequence of their occurrence and association. A description is not complete, however, without elaboration of the peculiar nature of both the facial paralysis and the restriction of eye movements. The paralysis of the face is nearly always bilateral, often asymmetric, and tending when incomplete to spare the lower face and platysma. Many authors have commented not only on this peculiar distribution of paralysis but on the usual lack of sagging of the face and the absence of contractures which are seen in acquired facial paralysis. When electrical stimulation has been employed the response of the areas of preserved activity has been roughly proportional to the degree of voluntary movement possible. Reaction of degeneration has not been reported. The facial paralysis seen in cases of congenital facial diplegia without other defects is characterized by the same features.5.7 Inability to abduct either eye beyond the midpoint is the other cardinal feature of the syndrome. This is particularly striking since conjugate movements in the vertical plane are often normal or nearly so. Convergence is, as a rule, present to varying degrees, even though adduction may be absent or much weaker in lateral gaze. Pupillary responses are normal. Bell's phenomenon is commonly present. Nystagmus is seldom seen, and horizontal nystagmus has not been elicited by vestibular stimu-

Neurologic aspects of the Mobius syndrome. A case study with electromyography of the extraocular and facial muscles.

Enlargement of the blind spot caused by papilledema.

The neuromuscular disorder known as myasthenia gravis was first described by Willis in the 17th century. The clinical features were more distinctly outlined by Erb in 1878, and the periods of remission were emphasized by Goldflam, so that the condition frequently has been referred to as Erb-Goldflam's disease. Since this article is designed to focus attention on the present status of therapy in myasthenia gravis, there will be no effort made to consider in detail the pathology and symptomatology of this disease. For a comprehensive survey of these aspects, reference may be made to the excellent review by Keschner and Strauss. 1 The chief clinical feature of myasthenia gravis is muscle fatigability on sustained effort. The initial symptoms most often appear in the extraocular muscles, producing ptosis and a variable diplopia. The extraocular muscle involvement may be so severe as to produce a complete external ophthalmoplegia (Fig. 1 A ). Weaknesses

A Great Conversation With Dr. James Corbett.

References

Visual Loss in Pseudotumor Cerebri: Follow-up of 57 Patients From Five to 41 Years and a Profile of 14 Patients With Permanent Severe Visual Loss

The 1982 Silversides lecture. Problems in the diagnosis and treatment of pseudotumor cerebri.

Neurologic aspects of the Mobius syndrome. A case study with electromyography of the extraocular and facial muscles.

Enlargement of the blind spot caused by papilledema.

Present status of therapy in myasthenia gravis.