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Continuous muscle fiber activity associated with thymoma.

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4. Voiculescu V, Alexianu M, Popescu-Tismana G, et al. Polyneuropathy with lipid deposits in Schwann cells and axonal degeneranon in cerebrotendinous xanthomatosis. J Neurol Sci 1987;82:
5. Salen G, Shefer S, Berginer VM. Familial diseases with storage
of sterols other than cholesterol: cerebrotendinous xanthomatosis
and sitosterolemia with xanrhomatosis. In: Stanbury JB, Wyngaarden JB, Fredrickson DS, et al, eds. The
basis of inherited disease, ed 5. New York: McGraw Hill, 1983:713-738
*Department of Nezlrology
Hospital 2Elx
Cerebrospinal Fluid
Taurine GI Alzheimer’s
Un&wPty of Alacant
J. Alom,* J. N. Mahy,? N. Brandi,? and E. Tolosat
The article by Kowall and Beal [l}describes how glutamate-,
glutaminase-, and taurine-immunoreactive neurons develop
neurofibrillary tangles in Alzheimer’s Disease (AD). Although glutamate 123,among other neurotransmitters E31,
has been involved in the neurochemical degenerative process
in AD, less is known about taurine. Their interesting report
suggests that taurine-containing neurons could also be involved in this pathological process. We would like to communicate our results about recent measurements of cerebrospinal fluid (CSF) taurine, among other amino acids, in the same
group of A D patients in which we previously found CSF
neuropeptide Y decrements [4].
Twenty consecutive patients (7 men and 13 women with
a mean age of 64.4 yr, range 52-75 yr) were entered in
the study. All met the diagnostic criteria for probable AD
according to the NINCDS-ADRDA Work Group. Symptomatic causes of dementia were excluded by laboratory examination, electroencephalography, and computed tomography. All patiencs included in the study had a Hachinski
ischemic score of <4. Cognitive evaluation was carried out
with the Mini Mental State Examination. The patients were
also evaluated with the Blessed Dementia Scale. The controls
were 19 hospitalized subjects (8 men and 11 women, mean
age 67.7 yr, range 50-80 yr) with no known central nervous
system disorder. The samples were collected between 9:OO
AM and 11:OO AM, after the patient had spent 8 hours in a
recumbent position. The samples were frozen and stored at
-20°C until assayed. The amino acids were measured by
high pressure liquid chromatography. Comparison between
subject groups was performed using Student’s t test and
Pearson’s correlation coefficient.
CSF taurine levels were lower in patients with AD (4.8 ?
1.4 FM) than in controls (6.4 1.7 kM) (p = 0.005). The
other CSF amino acid levels did not show any statistical difference between patients and controls: aspartic acid (5.9 k
1.3 vs 5.8 t 1.4 pM, p = 0.76); serine (14.2 k 4.8 vs 15.1
5.9 p M , p = 0.16); glutamine (398.2 f 87.1 vs 432.1
111.3,p = 0.30); alanine (19.4 5.5 vs 24.6 ? 1 2 . 1 , ~=
0.13); and homocysteic acid (2.5 2 1.1 vs 2.4 t 0.8, p =
0.13). We found a correlation between the disease duration
(yr) and the levels of serine (r = 0.64, p = 0.002) and
glutamine (r = 0.46, p = 0.02). We also found a negative
correlation between the levels of alanine and the Blessed
scores (r = 0.50,p = 0.01).
Mainly, we are describing CSF taurine decrements in A D
patients. As far as we know, there are no previous studies in
the literature about CSF taurine levels in AD, and we consider that our findings agree with the report of Kowall and
We do not know if these CSF taurine decrements are
a reflection of changes in an astrocytic versus a neuronal
taurine pool. It would be desirable in future to investigate
the role of taurine-containing neurons in the neurochemical
pathology of AD.
f Department of Biochemistry
University of Bari-elona
$Department of Neurology
Hospital Clinic i Provincial
University of Barcelona
Barcelona, Spain
1. Kowall NW, Beal MF. Glutamate-, glutaminase-, and taurineimmunoreactive neurons develop neurofibrillary tangles in Alzheimer’s disease. Ann Neurol 1991;29:162-167
2. Hyman BT, Van Hoesen GW, Damasio AR. Alzheimer’s disease:
glutamate depletion in the hippocampal perforant pathway zone.
Ann Neurol 1987;22:37-40
3. Beal MF, Martin JB. Neuropeptides in neurological disease. Ann
Neurol 1986;20:547-565
4. Alom J, Galard R, Catalan R, er al. Cerebrospinal fluid neuropepride Y in Alzheimer’s disease. Eur Neurol 1990;30:207-210
Continuous Muscle Fiber
Activity Associated with
V. Homberg, MD,* and H.-J. Freund, MDI‘
We read with interest the article by Garcia-Merino and colleagues (February 1991 issue of Annab), concerning an association of Continuous Muscle Fiber Activity, Peripheral Neuropathy, and Thymoma [l). The authors claim that this
association has not previously been recognized. We published two similar cases 4 years ago and discussed possible
autoimmune mechanisms underlying this condition t2). Associations of malignancy and neuromyotonia have been described previously [ 3 , 41. In the French literature, cases labeled “choree fibrillaire de Morvan” closely resemble this
type of neuromuscular hyperactivity [ 5 ] . This condition had
also been reported to be associated with thymoma 161 in a
case which took a fatal course. Both of our patients showed a
proliferative thymoma with high serum titers of acetylcholine
receptor (AchR) antibodies without signs of myasthenia gravis. Unfortunately, Garcia-Merino and colleagues did not
look at AchR antibodies. We discussed the possibility in our
cases that AchR antibodies may facilitate rather than inhibit
neuromuscular cholinergic transmission. Electrophysiological
studies showed multiple discharges on needle EMG recordings and some minor neurographic abnormalities, including
F-wave failures, which resemble the mild signs of peripheral
Annals of Neurology
Vol 30 N o 5 November 1991 735
neuropathy in the cases presented by Garcia-Merino and colleagues.
We agree wtih Garcia-Merino and colleagues that the syndrome is a serious one; one of our 2 patients died. The
surviving patient, however, responded well to immunosuppressive treatment. We therefore suggest that in patients
presenting with neuromuscular hyperactivity syndromes a
search for thymorna and AchR antibodies is necessary in order not to miss the opportunity for possible immunosuppressive therapy.
‘Neurological Therapy Center
?Department of Neurology
Heinrich-Heine University
stability). Antiacetylcholine receptor (AchR) antibodies were
detected in our second patient, who also had myasthenia
gravis and an AchR titer of 6 nmole/ 1 (normal: < 2). The
possible mediation of the syndrome through anti-AchR antibodies is an interesting possibility. However, the presence of
other specific antibodies cannot be ruled out.
I t is clear that CMFAS may be the presenting manifestation of a serious neuromuscular complication of thymoma,
sometimes associated with autonomic and mental disturbances. We agree with Drs Homberg and Freund that thymoma should be ruled out in CMFAS. Their good results
with thyrnectomy and immunosuppressive drugs further emphasize this recommendation.
Massachusetts General Hospital
Boston. M A
1. Garcia-Merino A, Cabello A, Mora JS, Liaho H. Continuous muscle fiber activity, peripheral neuropathy, and thymoma. Ann Neurol 1991;29:215-218
2. Halbach M, Hornberg V, Freund HJ. Neurornuscular, autonomic
and central cholinergic hyperactivity associated with thymorna
and acetylcholine receptor-binding antibody. J Neurol 1987;234:
3. Walsh JC. Neuromyotonia: an unusual presentation of intrathoracic malignancy. J Neurol Neurosurg Psychiatry 1976;39:
1086- 1091
4. Wearness E. Neurornyotonia and bronchial carcinoma. Electromyogr C h Neurophysiol 1974;14:527-535
5. Morvan A. De la chorCe fibrillaire. Gaz Hebd Med Chir 1890;
27: 173-200
6. Laterre EC, Bergmanns J, Ferriere G. ChorCe fibrillaire de Morvan et thymome (etude clinique et neurophysiologique). Schweiz
Arch Neurol Neurochir Psychiatr 1973;111:60
A. Garcia-Merino, MD
We thank Drs Homberg and Freund for their comments on
the association of continuous muscle fiber activity syndrome
(CMFAS) and thymoma. We were aware of the association
of thymoma with overt peripheral neuropathy in the absence
of CMFAS [I], and with CMFAS without neuropathy [2].
We were not aware of the simultaneous association of both
conditions with thymoma. The first patient mentioned by
Hornberg and Freund 133 lacked conclusive evidence of peripheral nerve damage. The second patient did have pathological changes indicative of mild axonal neuropathy at
autopsy, although clinical expression was minimal. In this
patient, nerve conduction study was normal, except for afterdischarges that obscured the identification of the F waves.
Our patients did have a clear clinical neuropathy: They presented with areflexia and sensory losses, their conduction
velocities were abnormal, denervation atrophy was found on
muscle biopsy, and abundant signs of axonal and myelin damage were present in the nerve biopsy.
Mention of the “chorke fibrillaire de Morvan” syndrome
poses important nosologic considerations. This condition
seems to be a CMFAS with prominent autonomic and mental
manifestations and has a presumed immunological basis. It
should possibly be included generically as a CMFAS. Our
second patient had some traits reminiscent of that particular
variety of CMFAS (e.g., confusion, drowsiness, vascular in-
1. Bogousslavsky J, Regli F, Doret AM, et al. Encephalopathy, peripheral neuropathy, dysautonomia, myasthenkd gravis, malignant
thymoma, and antiacetylcholine receptor antibodies in the CSF.
Eur Neurol 1983;22:301-306
2. McComas AJ. Neurornuscular function and disorders. London,
Buttenvoahs, 1977, pp 315-317
3. Halbach M, Homberg V, Freund HJ. Neuromuscular, autonomic, and central cholinergic hyperactivity associated with thymnma and acetylcholine receptor-binding antibody. J Neurol
Brain and Retinal
Microemboli During
Cardiac Surgery
Isla M. Williams, MD,” J. F. Stephens, MSc,?
E. P. Kchardson, Jr, MD,* G. Stirling, FRACS, FACS,S
and P. Robinson, PhDll
We noted with interest, in the article by Moody and colleagues [l}, the photographs of microemboli in cerebral capillaries of human brains after cardiopulmonary bypass, and
we also noted the comments in the accompanying editorial
by Gilman {2}. We call the attention of the authors to our
reports from 1973 to 1975, which show emboli in the brains
and retinas of patients and in the retinas of dogs after cardiopulmonary bypass [3-6]. The colorless, transparent emboli
were stained faintly pink with oil red 0 (ORO) and had starlike pink clusters (crystals of ORO). The emboli, however, failed to stain with hernatoxylin and eosin, periodic
acid-Schiff‘s reagent counterstained with hematoxylin, or
Schultz’s stain for cholesterol. Phase-contrast microscopy
showed that in some areas the colorless material was coarsely
granulated. The vessels containing such occlusions were distended. The finely granular material was sometimes extruded
from the vessels in the presence of a histiocyte [5].
Electron rnicroprobe analysis identified a high content of
silicon in the embolic foreign material within the retinal capillary network from two patients prepared with trypsin digestion { S , 7, 8). The defoaming of blood with silicone (Simethicone A) is still used in cardiopulmonary bypass surgery.
although the amount used has been reduced to a minimum
736 Annals of Neurology Vol 30 N o 5 November 1991
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fiber, muscle, associates, thymoma, activity, continuous
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