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Progressive multifocal leukoencephalopathy and magnetic resonance imaging.

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Progressive Mu1tifocal
Leukoencephalopathy
and Magnetic
Resonance Imagrng
Jody D. Levy, MD," Karen L. Cottingham, MD,"
Robert J. Campbell, M D , t Gregory K. Moore, MD,"
Ference Gyorkey, MD,$ Tetsuo Ashizawa, MD,T
and Arnold M. Goldman, MDS
~~
~
Progressive multifocal leukoencephalopathy developed
in a homosexual man with underlying Hodgkin's disease. Computed tomography and magnetic resonance
imaging of the brain demonstrated multiple lesions,
more in gray than white matter. Brain biopsy established the diagnosis of progressive multifocal leukoencephalopathy. Magnetic resonance imaging was found
useful for detecting brain lesions and for localizing an
accessible lesion for biopsy.
Levy JD, Cottingham KL., Campbell RJ, Moore GK,
Gyorkey F, Ashizawa T , Goldman AM:
Progressive multifocal leukoencephalopathy
and magnetic resonance imaging.
Ann Neurol 19:399-401, 1986
Progressive multifocal leukoencephalopathy (PML) is
an uncommon papovavirus infection that usually afFrom the Departments of "Medicine, TNeurology, and $Pathology,
Veterans Administration Medical Center, Baylor College of
Medicine, and PNMR Associates-Kirby, Houston, TX.
Received Feb 6, 1985, and in revised form June 12 and Aug 28.
Accepted for publication Aug 28, 1985.
Address reprint requests to Dr Levy, Department of Medicine,
White Memorial Medical Center, 1720 Brooklyn Ave, Los Angeles,
CA 90033.
fects persons with immune deficiency states. We present the clinical and pathological features of an atypical
case of PML in which disease foci were confined predominantly to structures of t h e gray matter. The abnormalities on computed tomography (CT) and magnetic resonance imaging (MRI) corresponded well
anatomically to t h e lesions found at postmortem examination. To OUT knowledge, this is the first reported
case of PML in which MRI was used to detect lesions.
Patient History
A 48-year-old homosexual man was admitted to a Houston
hospital in April 1984 with a 4-month history of a 13.5-kg
weight loss and progressive neurological deficits consisting of
right hand weakness, right facial numbness, slurred speech,
and gait disturbance. Findings of physical examination were
remarkable for right tonsillar hypertrophy and cervical
lymphadenopathy. O n neurological examination he was
found to be oriented and dysarthric, and had right wrist
drop. There were diminished pinprick and light-touch sensations on the right face and leg, right-sided hyperreflexia, and
severe cerebellar ataxia.
Results of routine laboratory tests were normal except for
a mild leukopenia. Skin tests did not evoke responses. Peripheral lymphocyte marker studies showed an inverted
helper/suppressor T-cell ratio. Tonsillar and cervical lymph
node biopsies revealed Hodgkin's disease of the mixed cellularity type, Stage IIB. A C T scan on admission showed a
normal cranium and an electroencephalographic recording
was normal. Lumbar puncture with routine cultures and tests
was unremarkable.
The patient's neurological deficits worsened over the subsequent weeks with further impairment of cerebellar functions, development of right ankle clonus, and lethargy. Four
weeks after admission, MRI was performed at 0.35 Tesla,
showing bilateral lesions of the thalamus and parietal lobes
(Fig 1A,B) and of the brainstem (midbrain and pons). Cranial
CT scanning was repeated and revealed areas of low attenuation in both thalami and parietal lobes, without contrast enhancement and without mass effect. Histological findings of
brain biopsy tissue from the left parietal cortical lesion
showed multifocal demyelination, lipid-laden macrophages,
and oligodendroglial cells with enlarged hyperchromatic nuclei, some containing intranuclear inclusions. Examination
with the electron microscope demonstrated the presence of
intranuclear virions with the ultrastructural morphology of
papovaviruses (diameter, 28 to 30 nm) (Fig 2A), establishing
the diagnosis of PML.
Postoperatively the patient's neurological condition deteriorated further, with the development of severe bilateral
hemiparesis. Pneumonia and sepsis developed and he died at
the end of the sixth month of illness.
Postmortem examination of the brain revealed extensive
destructive lesions of PML, involving the medulla, pons, tegmentum of the midbrain, the cerebellum, and both cerebral
hemispheres. The lesions were confined predominantly to
the gray matter, with lack of relationship to cerebral blood
vessel distribution. Necrotic lesions were found in both the
right and left thalamus (Fig 2B). The anatomical location of
the lesions in the cerebral hemispheres and brainstem corresponded well with the abnormalities seen on MRI. Micro-
399
A
B
scopically, both white and gray matter lesions exhibited the
characteristics of advanced PML. No inflammatory cells or
neoplastic infiltrates were seen. Cytomegalovirus (CMV) was
demonstrated post mortem in the lung and kidney; however,
F ig 1 . (A)Magnetic resonance image of the brain demonstrating
a right thalamic lesion (arrow). The repetition (TRI time is 2
seconds and echo (TE) time is 28 msec. Slice thickness is 7 mm.
(B) Coronal magnetic resonance image showing a ldt parietal
cortical lesion in the area of the postcentral gyrus extending t o
the superior parietal lobule (arrow).The T R time is 1.5 seconds
and TE time is 56 msec. Slice thickness is 7 mm.
no central nervous system involvement was detected micro-
scopically.
Discussion
PML usually occurs in patients with lymphoma or
leukemia, particularly Hodgkin’s disease and chronic
lymphocytic leukemia 12). Other associated disorders
have included carcinomatous, granulomatous, inflammatory, and autoimmune diseases, as well as the innate
and acquired immune deficiency syndrome (AIDS)
121. Our patient’s history of homosexuality, inverted
T 4 E 8 ratio, and type of opportunistic infections suggest that he may also have had AIDS. It would also
seem more likely that the background immunosuppression was caused by AIDS rather than Hodgkin’s
disease, as PML is a relatively late clinical complication
of lymphoproliferative disease 16). The diagnosis of
AIDS cannot be confirmed here because Hodgkin’s
disease was the initial diagnosis, which prevents
classification of this patient under the current Centers
for Disease Control Surveillance definition of AIDS
151. A few reported cases of Hodgkin’s disease in association with AIDS are now under consideration [ 3 , 41.
The unusual aspect of this case is the extensive
amount of gray matter involvement in comparison to
that found in previously reported cases. The explana400 Annals of Neurology Vol 19 No 4
April 1986
tion for the extension of papovavirus beyond its usual
confines is uncertain. Perhaps our patient’s immune
system was so severely depressed that he developed a
more aggressive form of the disease.
Although this man cannot be classified as having
typical AIDS, he had multiple opportunistic infections,
including the demonstration of disseminated CMV
post mortem. Although no central nervous system involvement was detected microscopically, immunocytochemical studies for CMV were not performed and
therefore accompanying brain infection with CMV
cannot be ruled out.
We believe that this is the first reported case of PML
in which MRI was used. We found it to be valuable in
identifying the multiple brain lesions in our patient.
The location of lesions indicated by MRI corresponded well to those found at postmortem examination. Using cranial CT, only the late necrotic PML
lesions are reportedly visualized and not the early foci
of demyelination [l]. This could explain why the initial
CT scan of our patient appeared normal. Whether
A
serological studies for papovavirus; Gabriele M. ZuRhein, MD (University of Wisconsin Medical Center), and Joel Kirkparrick, MD
(Baylor College of Medicine), for review of rhe neuropathological
specimens; James J. Butler, MD, and Barbara M. Osborne, MD
(M. D. Anderson Hospital and Tumor Institute, Houston, TX), for
review of the tonsillar and lymph node specimens; Dorothy E.
Lewis, PhD (Baylor College of Medicine), for the cell surface
phenoryping studies; and Nelda P. Wray, MD (Baylor College of
Medicine), for her support.
References
B
Fig 2. (A) Electron micrograph of the left parietal lesion showing a portion of a glial nucleus containing numerous crystalline
aggregates of papovavirus particles replacing nuclear chromatin.
Portion of cell cytoplasm at right upper corner and below nuclear
membrane at bottom.(Uranyl acetate and lead citrate; x 37,500,
inset x 62,500 before 22% reduction.) (B) Extensive cavitary
necrosis of the right and left thalamus (arrows).
MRI is more sensitive than cranial CT and is able to
detect earlier lesions remains to be determined. We
are unable to reach conclusions on this point because
too much time elapsed between the two studies. Our
experience indicates, however, that MRI of the brain is
an accurate method for outlining areas of disease.
1. Bosch EP, Cancilla PA, Cornell SH: Computerized tomography
in progressive multifocal leukoencephalopathy. Arch Neurol
33:216, 1976
2. Brooks BR, Walker D L Progressive multifocal leukoencephalopathy. Neurol Clin 2:299-313, 1984
3. Robert NJ, Schneiderman H: Hodgkin’s disease and the acquired
immunodeficiency syndrome. Ann Intern Med 101:142-143,
1984
4. Schoeppel SL, Hoppe RT, Dorfman RF, er al: Hodgkin’s disease
in homosexual men with generalized lymphadenopathy. Ann Intern Med 102:68-70, 1985
5. Selik RM, Haverkos HW, Curran JW: Acquired immune
deficiency syndrome (AIDS) trends in the United States, 19781982. Am J Med 76:493-500, 1984
6. ZuRhein GM: Association of papova-virions with a human demyelinating disease (progressive multifocal leukoencephalopathy).
Prog Med Virol 11:185-247, 1969
The authors thank Duard L. Walker, MD, and his laboratory (University of Wisconsin School of Medicine) for the immunocytochemical studies of biopsy and posrmorrem specimens and for the
Brief Communication: Levy et al: PML and MRI 401
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