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Disseminated thymoma and myasthenia gravis Dramatic rsponde to prednisone.

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Disseminated Thymoma
and Myasthenia Gravis:
Dramatic Response
to Prednisone
R. Taylor, MD,' R. Tandan, MD, MRCP,*
J. Roberts, MD,t D. P. DiCostanzo, MD,S
K. Sharma, MD,' and T. Fries, MD"
Myasthenia gravis (MG) occurs in up to 44% of patients with
thymoma; one-third of neoplasms are invasive at surgery {I},
and 1 to 15% show extrathoracic metastases [2}. The prognosis in patients having MG with invasive thymoma is worse.
Thymectomy and radiotherapy are the mainstays of treatment for invasive thymoma (i],although few cases respond
to glucocorticoids { 37 or combination chemotherapy 141.
The treatment of choice for metastatic thymoma is chemotherapy {47. Glucocorticoids have produced improvement of
MG and remission of thymoma metastatic to the lungs after
failed chemotherapy in one previous patient {5}. We report
the first patient with extrathoracic spread of thymoma and
severe MG, whose tumor and MG responded dramatically to
therapy with daily prednisone, after failure of radiotherapy
and combination chemotherapy.
Investigation of chest pain at age 39 in this woman disclosed an anterior mediastinal mass, which at surgery proved
to be a thymoma invading the adjacent lung, pleura, pericardium, and chest wall. Histologically this tumor showed
mixed epithelial and lymphoid elements. Thymectomy was
followed by 4,000 rads of irradiation to the mediastinum.
Two years later three asymptomatic right lung nodules were
resected, and within another year local recurrence in the left
hemithorax was treated with two courses of cyclophosphamide, doxorubicin, and cisplatin without effect. Tumor adherent to the left diaphragm was removed surgically, followed by 3,240 rads of local irradiation. At age 46 recurrent
pleural masses were treated with percutaneous radium implants. Investigation of abdominal pain by computed tomography (CT) at age 47 showed several hepatic masses; laparotomy revealed multiple irregular mesenteric tumors with foci
of hemorrhage in one and microscopic featyres of mixed
thymoma. Soon after surgery the patient developed progressive fatigue, weakness of neck and limb muscles, and became
wheelchair bound. Examination showed hepatomegaly, other
abdorninal masses, dyspnea, dysarthria, ptosis, neck and limb
girdle weakness, and fatigue. Intravenous edrophonium produced some improvement. Acetylcholine receptor antibody
titer was 7.76 nM/L (normal < 0.1).MG was diagnosed clinically; she was given assisted ventilation, prednisone 100 mg
daily, and a course of five-alternate-day plasmapheresis. Cyclophosphamide begun simultaneously was discontinued after four days because of hematuria. She was weaned off the
respirator after five days. Over the next few months prednisone was slowly tapered. Ten months after discharge she has
returned to her full-time desk job with continued improvement on pyridostigrnine, 60 mg four times a day, and prednisone, 40 mg daily. No further plasmapheresis has been performed. Serial abdominal CT scans and chest x-ray films have
dociimenterl nimor reeresqion (Fieure).
Abdominal computed tomographic (CT)scans through tbe liver.
(A) Prior to twatmnt there was a large hypodense tumor mass
in the lejl lobe and a smaller right subcapsular hypodensity. (Bj
Tbis C T scan was taken ten weekc afer the initiation of dzih
prednisone tveatment and shows dramatic reduction in tumor
Our patient with invasive thymoma developed MG and
extrathoracic metastasis after 8 years of treatment with multiple modalities. Therapy with daily prednisone initiated during a myasthenic crisis produced dramatic clinical improvement of both her MG and tumor burden. Spontaneous
remission seems unlikely, since progressive thoracic disease
had been present for 8 years. Experience with this case and
the one previously reported 153 indicates that in metastatic
thymoma with MG aggressive treatment with ventilatory
support, plasmapheresis, and daily glucocorticoids may be
successful in patients who have failed combination chemotherapy.
Departments of #Neurology and fMedicine
University of Vermont College of Medicine
Burlington, V T
SDepartment of Pathology
Beth Israel Medica1 Center
New York. N Y
inaccuracy, or slowing) in response to specified stimuli seems
preferable to either “apraxia” or “paresis.”
1. Bernatz PE, Khausari S, Harrison EG, Taylor WF. Thymoma:
factors influencing prognosis. Surg Clin North Am 1973;53:
2. Nickels J, Fransiila K. Thymoma metastasizing to extrathoracic
sites. Acta Pathol Microbi01 Immunol Scand [A] 1776;84:331334
3. Shellito J, Khandekar JD, McKeever WP, Vick NA. Invasive
thymoma responsive to oral corticosteroids. Cancer Treat Rep
4. Donovan PJ, Foley JF. Chemotherapy in invasive thymomas: five
case reports. J Surg Oncol 1986;33:14-17
5. H u E, Levine J. Chemotherapy of malignant thymoma. Case report and review of the literature. Cancer 1986;57:1101-1104
Tbe Toronto Hospital
Toronto, Canadz
Ocular Motor Paresis
Versus Apraxia
James A. Sharpe, MD, and Janine L. Johnston, MD
The designation acquired ocular motor apraxia was used in a
recent article in the Annals [i] to describe the eye movements of a patient who could not make saccades to command, to visual targets, or with head movement. Even with
head movement, saccades were delayed after head motion
and were abnormally slow and occasionally hypompic. Saccades to visual targets were delayed and slow. The patient,
who had bilateral cerebral ischemic damage, had no smooth
pursuit or optokinetic nystagmus. Even the quick phases of
caioric nystagmus were small and slow.
We suggest that the term ocular motor apraxia be restricted
to disorders of initiating voluntary saccades when reflexive
saccades to visual targets, or saccades initiated with head
movement, and vestibular quick phases are spared. These
conditions are met by the syndrome of congenital ocular
motor apraxia [2) but seldom in acquired brain disease. For
example, some patients with Huntington’s disease ’have impaired initiation of voluntary saccades to command but reflexive saccades to visual targets are intact f3}; patients are
unable to suppress reflexive glances to nove1 visual stimuli.
There are at least two major classes of cerebrally triggered
saccades: (1) internally triggered (volitional) saccades, as in
reply to the examiner’s command or in response to a predictably moving target; and (2) externally triggered (reflexive)
saccades, as in response to a sudden, unpredictable, retinal
target. Patients with genuine apraxia of saccades exhibit
deficits of the first class of saccades but not the second. The
defects can be partial, as after focal frontal lobe damage,
when saccades to predictable targets (class 1) are impaired
but saccades to random targets (class 2) are preserved 147.
Patients with Alzheimer’s disease [5] and Parkinson’s disease
{ 6 ) have elements of each type of deficit.
We disagree with the appropriateness of the designation of
apraxia in the patient described by Dr Pierrot-Deseilligny
and associates [i]. The patient had paralysis (or paresis) of
saccades and pursuit, not apraxia. We also wish to point out
that slowing of saccades after cerebral lesions, as illustrated
by their patient, had been previously quantified in our laboratory after cerebral hemispheric damage in humans [4, 7).
Finally, explicit description of the types of deficits (delay,
1. Pierrot-Deseiiligny C , Gautier J-C, Loron P. Acquired ocuiar
motor apraxia due to bilateral frontoparietai infarcts. Ann Neurol
2. Zee DS, Yee RD, Singer HS. Congenital oculomotor apraxia
Brain 1977;100:581-600
3. Lasker AG, Zee DS, Hain TC, et ai. Saccades in Huntington’s
disease: initiation defects and distractibiiity. Neurology 1987;37:
4. Shatpe JA. Adaptation to frontal lobe lesions. In: Keller EL, Zee
DS, eds. Adaptive processes in visuai and oculomotor systems.
Oxford: Pergamon Press, 1986:239-246
5. Fletcher WA, Sharpe JA. Saccadic eye movement dysfunction in
Alzheimer’s disease. Ann Neurol 1986;20:464-47 1
6. White OB, Saint-Cyr JA, Tomlinson RD, Shatpe JA. Ocular
motor deficits in Parkinson’s disease. 111. Coordination of eye and
head movements. Brain 1988;111:115-130
7. Sharpe JA, Lo AW, Rabinovitch HE. Contro1 of the saccadic and
smooth pursuit systems after cerebral hemidecortication. Brain
Charles Pierrot-Deseilligny, MD,
Jean-Claude Gautier, MD, and Philippe Loron, MD
Sharpe and Johnston prefer the term ocuiar motor paresis for
the oculomotor syndrome that we recently reported, in
which initiation of al1 cerebraiiy triggered saccades was disturbed [i]. According to them, the term ocukzr motor apraxia
should be applied only to impairment of volitional saccades.
Application of this concept, however, would mean that the
same term could also theoretically be used when impairment
selectively involves one type or several types of saccade in
either class of saccades (volitional or reflexive), Le., in a number of different oculomotor syndromes.
Therefore, we consider it preferable to limit the use of the
term ocukzr motor apraxia to cases in which initiation of al1
types of cerebrally triggered saccades is impaired coa greater
or lesser degree. This was the case in most congenital forms
of the syndrome E2-41, in which the term oruiar motor
apraxia was first used, but also in a few acquired forms [i, 5).
In our patient [i), initiation of al1 cerebrally triggered saccades was severely impaired when the head was immobilized.
Only rare visually guided saccades were then observed, but
quick phases of the vestibular nystagmus persisted. In contrast, with the head free, volitional as well as reflexive saccades were present, even though they were delayed. Thus,
there were clear dissociations of responses, essentially according to the experimentai conditions but also to the type
of saccades. Such dissociations meet the generai criteria for
apraxia. As for the syndrome called apraxia of saccades by
Sharpe and Johnston, a more appropriate term might well be
paralysis of volitional saccades.
With regard to our patient, the term paresis cannot be
applied FO the impairment of saccade initiation, leading to the
onset or the absence of saccades according to the experimen-
Annals of Neurology
Vol 25 N o 2 February 1989 203
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disseminated, prednisone, thymoma, myasthenia, rsponde, dramatic, gravis
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