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Brainstem tuberculoma Value of sequential computed tomography.

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Vacuoles in Lymphocytes
in Adult Type
Mucolipidosis
Siuliduse and Sialic Arid in Leukocytej
Takuro Kobayashi, MD, Michiya Ohta, MD,
Ikuo Goto, MD, and Yoshigoro Kuroiwa, M D
Patient
Controls
( N = 5)
Total
Leukocytes
Subject
SIALIDASE
ND
0.224
ND
0.220 ~t0.106
(nmol/mg proteinlhr)
SIALIC ACID
Since Goldberg et a1 [ 11reported a patient with adult type
mucolipidosis and b-galactosidase deficiency, several cases
involving similar manifestations have been published. The
presence of vacuolated lymphocytes is one of the common!
features of this disorder. Although sialidase deficiency with
increased urinary excretion of sialyloligosaccharide or
sialylglycopeptide has been reported [2, 51, the accumulated substance in these vacuoles has not been identified.
Moreover, it is not clear why these vacuoles are seen only
in lymphocytes but not in polymorphonuclear leukocytes.
Accordingly, we compared sialidase activity and sialic acid
content in the lymphocytes and polymorphonuclear leukocytes of a patient with adult type mucolipidosis.
The patient is a 20-year-old Japanese woman with macular cherry-red spots, gargoyle-like face, corneal clouding, ataxia, generalized convulsions, and myoclonus. pGalactosidase in leukocytes was about 10% of normal.
Vacuoles were seen in 30 to 40% of lymphocytes but never
in polymorphonuclear leukocytes; this was confirmed by
electron microscopic examination. The vacuoles were
negative on periodic acid-Schiff, toluidine blue, and Sudan
black B staining but were positive with acid phosphatase.
Heparinized venous blood was drawn from the patient
and normal controls, and leukocytes were prepared with
5% dextran. Lymphocytes and polymorphonuclear leukocytes were separated by the Ficoll-Conray method.
Sialidase was assayed by the method of Thomas et a1 [3]
using fetuin as a substrate. Sialic acid content was determined by Warren's method [4].Protein was measured by
the phenol method.
In normal subjects, no difference in sialidase activity
between lymphocytes and polymorphonuclear leukocytes
was seen (Table). No sialidase activity was detected in the
leukocyte extract from the patient, indicating a deficiency
in both lymphocytes and polymorphonuclear leukocytes.
Sialic acid content was increased in whole leukocytes, lymphocytes, and polymorphonuclear leukocytes in this patient.
The histochemical and direct results indicate that there
may be no accumulation of sialyloligosaccharide or glycoprotein in the vacuoles of leukocytes in adult mucolipidosis.
Lymphocytes
Polymorphonuclear
Leukocvtes
Patient
Controls
( N = 5)
0
0.322
f 0.138
?
0.184
(nmol/mg protein)
29.8
8.2 f 6.1
(2.8-19.6)
30.9
10.2 c 4.9
(5.1-18.1)
29.7
9.2 f 3.1
(5.9-13.6)
Control values are shown as mean f SD.
ND
=
not determined.
References
1. Goldberg MF, Cotlier E, Fichenscher LG, et al: Macular
cherry-red spot, corneal clouding, and P-galactosidase deficiency. Arch Intern Med 128:387-398, 1971
2. Orii T, Minami R, Sukegawa K, et al: A new type of
mucolipidosis with P-galactosidase deficiency and glycopeptiduria. Tohoku J Exp Med 107:303-315, 1972
3. Thomas G H , Tiller GE Jr, Reynolds LW, et al: Increased levels
of sialic acid associated with a sialidase deficiency in I-cell disease (mucolipidosis 11) fibroblasts. Biochem Biophys Res
Commun 71:188-195, 1976
4. Warren L The thiobarbituric acid assay of sialic acids. J Biol
Chem 234:1971-1975, 1959
5 . Wenger DA, Tarby TJ, Wharton C: Macular cherry-red spots
and myoclonus with dementia: coexistent neuraminidase and
P-galactosidase deficiencies. Biochem Biophys Res Commun
82:589-595, 1978
Brainstem Tuberculoma:
Value of Sequential
Computed Tomography
Joseph R. Haskett, Jr, Capt, MC, USA,
Clinton E. Branch, Jr, Maj, MC, USAR,
and Jon H . Buscemi, Lt Col, MC, USA
Intracranial tuberculoma is, in the United States, a relatively rare but potentially curable lesion. This report presents the first reported case of brainstem tuberculoma with
resolution documented by serial CT scans.
A 28-year-old American Indian man was found on examination to have gait ataxia, internuclear ophthalmoplegia,
From the Departments of Neurology and Neuropathology, Neurological Institute, Faculty of Medicine, Kyushu University 60,
Fukuoka, Japan.
From the Department of Medicine, Neurology Service, Fitzsimons
Army Medical Center, Denver, CO.
Accepted for publication May 5, 1979.
Address reprint requests to D r Goto, Department of Neurology, Neurological Institute, Faculty of Medicine, Kyushu University, 3-1-1 Maedashi, Higashi-ku, Fukuoka 812, Japan.
Address reprint requests to Dr Branch, Neurological Group of
Northeast Georgia, 1220 Sherwood Park Dr NE, Gainesville, G A
30501.
Accepted for publication May 7, 1979.
Notes and Letters 275
right peripheral facial paralysis, bilateral Babinski signs,
and a drainiiig scrota1 a b s c i s . A n interme~liate-strt-ngth
purifie-d protein clcrivative skin test was positive t o 2 0 m m
o f induration. (.crebrospinal fluid examination was consis~tous
(:T Scan clemonstrated
tent with g r a ~ l ~ i ~ m ~inenirigitis.
a brainstern ni:iss with surrounding e d e m a and contrast etihancemcnr (Pigurct) Subsequently, culturcs wcre positive
for Mjc-uhui-terium tuhrrr.ulo.ri.i. Antituberculosis therapy
was begun, anti t h c patient’s condition returned t o normal
over t h e next six inoriths. Serial CT scans over this period
revealed rcscilurion of the pontine mass.
IJntil recently, the diagnosis o f terltral nervous system
ruberculomas has clcpen~ledo n autopsy, surgical biopsy, o r
roentgenographic p r o t c d u r e s that lack spec-ifitity and sensivitity a n J rvquire t h e patient t o uridcrgo numerous UCIconiforlablc anti poreiitially h a m d o u s procedures. Several
recent reports have demonstrated t h e value of computerized axial tomography in identifying t h e n u m b e r and
location o f intrlicranial ruberculomas as well as helping to
identify the i i e ~ e l o p m e n of
t communicating hydrocephalus
12-41. To ilatc, two reports have dealt with sequenrial CT
scanning in t u l w r i u l o u s meningitis and its usefulness in
folhwIrig the rc.solutron o f a supratentorial tuberculoma I I ,
3 I.
Our patierit h a J a pic cure o f progressive focal neurological clcfizir. Tht- CT scan provideJ a noninvasive, sensitive
m c r h o J o f follow in^ his response t o antituberculosis thetapy until total rcsolution occurred. We believe computerized axial toniography is a safe, simple, noninvasive
method of fi)lloa,ing the progress of supratentorial and infratentorial tuberculonias during treatment while also allowing the opportunity t o assess ventricle size t o detect the
presence o r prcgrt-ssion o f hydrocc-phalus.
276
Aiiids o f Neurolog)
Vol 6
No 3
References
I . Arimirsu ’I’
Jahbari
,
B, Buckler RE, et al: Computed tomography in a verified case of tuberculous meningitis. Neurology
29:384-386, 1979
2. Claveria LE, du Boulay GH, Moseley IF: Intracranial infec-
tions: investigation by computerized axial tomography.
Neuroradiology 12:59-7 1, 1976
3. Enzmann DE, Norman D, Mani J, et al: Computed tomography
of granulomatous basal arachnoiditis. Radiology 120:341-344,
1976
4. Price HI, Danziber A: Computed tomography in cranial tuberculosis. Am J Roentgenol Radium Ther Nucl Med 130:769771, 1078
5 . Srevens DL, Everett DE: Sequential computerized axial tomog-
raphy i n tuberculous meningitis. JAMA 239:642, 1978
Sample and Test
Considerations
in Evaluating SDatial
Deficits in Left-Ha.nders
U
I
Sheri A. Berenbaum. P h D
In their paper, “ W A I S performance in brain-damaged leftand right-handers” ( A n n N e u r o l 2:422- 424, 1977), Todd
and Satz purport to test Levy’s [ 1-31 m o d e l of t h e effects of
From the Dight Institute for Human Genetics, University o f Minnesota, Minneapolis, M N 55455.
S e p t e m b e r 1079
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