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Astasia and gait failure with damage of the pontomesencephalic locomotor region.

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same solvents as the patient reported. This second patient had developed a severe cerebellar syndrome with
only minor components suggestive of parkinsonism.
Further studies of patients with neurological dysfunction secondary to solvents may lead to the identification of specific neurotoxins and genetic characteristics
that predispose individuals to a particular neurological
consequence.
Astasia and G t Failure
with Damage of the
PontomesenceDhalic
Locomotor Rggion
Joseph C. Masdeu, MD, U m a Alampur, MD,
Rossella Cavaliere, M D , and George Tavoulareas,
MD
Dr Uirri is the recipient of the 1993 RCPSC-Sandoz Clinical Research Fellowship.
Wc thank the Medical Research Council of Canada, National Parkinson Foundation (Miami), Dystonia Medical Research Foundation,
Parkinson Foundation of Canada, and Movement Disorder Institute
for their support, Dr I. J. Kopin for his useful comments, and Susan
Calne for her assistance in the preparation of this manuscript.
References
1. Ikeda 11, Markey CJ, Markey SP. Search for neurotoxins struccurally related to 1-methyl-I-phenylpyridine(MPP+ ) in the
Although there is evidence in experimental animals of
the importance of the pedunculopontine area in locomotion, clinical lesion reports are lacking. An 83-year-old
woman became unable to stand and to generate stepping
movements after a hemorrhage at the pontomesencephalic junction involving the right pedunculopontine area.
Her deficit resembled the gait failure present in some
elderly individuals.
Masdeu JC, Alampur U, Cavaliere R, Tavoulareas G .
Astasia and gait failure with damage of the
pontomesencephalic locomotor region.
A n n Neurol 1994;35;619-62 1
pathogenesis of Parkinson’s disease. Brain Res 1992;575:285298
2. Snow BJ, Okada A, Martin WRW, et al. PET scanning in doparesponsive dystonia, parkinsonism-dystonia, and young-onset
parkinsonism. In: Segawa M, ed. Hereditary progressive dystonia (with marked diurnal variation). Carnforth: Parthenon, 1993:
181-186
3. Hormes JT, Filley CM, Rosenberg NL. Neurologic sequelae of
chronic solvent vapor abuse. Neurology 1986;36:698-702
4. McLean DR, Jacobs H, Mielke BW. Methanol poisoning: a clinical and pathological study. Ann Neurol 1980;8:161-167
5. Pezzoli G, Barbieri S, Ferrante C, et al. Parkinsonism due to
n-hexdne exposure. Lancet 1989;2:874
6. Guggeuheim MA, Couch JR, Weinberg W. Motor dysfunction
as a permanent complication of methanol ingestion: presentation
of a case with a beneficial response to levodopa treatment. Arch
Neurol 1971;24:550-554
7. Benignus VA. Health effects of toluene: a review. NeurotoxiC O ~ O ~1981;2:567-588
Y
8. Keane JR. Toluene optic neuropathy. Ann Neurol 1978;4:390
9. Filley CM, Hearon RK, Rosenberg NL. White matter dementia
in chronic toluene abuse. Neurology 1990;40:512-534
10. Voog L, Eriksson T. Toluene-induced decrease in rat plasma
concentrations of tyrosine and tryptophan. Acra Pharmacol Toxicol 1984;54:15 1-1 5 3
11. Juorio AV, Yu PH. Effects of benzene and other organic
solvents on the decarboxylation of some brain aromatic-I-amino
acids. Biochem Pharmacol 198>;34:1381-1387
12. Fuxe K, Anderson K, Nilsen OG, et al. Toluene and telencephalic dopamine: selective reduction of amine turnover in discrete DA nerve terminal systems of the anterior caudate nucleus
by low concentrations of toluene. Toxic Lett 1982;12:115-123
13. Nordstrom A-L, Farde L, Halldin C . Time course of D2dopamine receptor occupancy examined by PET after single oral
doses of haloperidol. Psychopharmacology 1992;106:433-438
14. Wolters ECh, Huang C-C, Clark C, er al. Positron emission
tomography in manganese intoxication. Ann Neurol 1989;26:
647-65 1
15. Shinotoh H , Snow BJ, Huang CC, et al. Presynapric and postsynaptic striatal dopaminergic function in manganese intoxication studied by positron emission tomography. Can J Neurol
Sci 1993;20(suppl 4):S236
Laterodorsally at the pontomesencephalic junction is
the mesencephalic locomotor region, which plays an
important role in locomotion in animals El]. Stimulation of this region in the cat induces “controlled locomotion,” meaning that progressively higher current
drives up the frequency of stepping from walking to
running. Anatomically, this area corresponds to the
pedunculopontine nucleus 11). In humans, loss of neurons in the pedunculopontine nucleus has been found
in progressive supranuclear palsy and Parkinson’s disease but not in patients with Alzheimer’s disease [2}.
Despite the seemingly important role in locomotion
played by this region, there are no clinical reports of
gait impairment by discrete lesions in this area. Such
an occurrence is reported here. The disorder of ambulation suffered by this patient is strikingly similar to
the one suffered by many older patients with gait failure and may provide a clue to its localization.
Patient Report
A n 83-year-old woman, a chronic hypertensive, was waiting
for a bus after her weekly 6 hours of casino playing when she
felt nauseated, vomited once, and fell to the floor, becoming
unable to stand or walk. She never lost consciousness or had
any sensory complaints. When evaluated by a neurologist 3
Department of Neurology, St Vincent’s Hospital and Medical Center of New York and New York Medical College, New York, NY.
Received Sep 14, 1993, and in revised form Oct 25. Accepted for
publication Oct 27, 1993.
Address correspondence to Dr Masdeu, Department of Neurology,
St Vincent’s Hospital and Medical Center of New York, 153 West
1lth Street, New York, N Y 10011.
Copyright
0 1994 by t h e American Neurological Association 619
Magnetic resonance imging (MRI) scans obtained 3 weeh after
a right pontomesencephalic hemorrhage. Sagittal (A) and axial
(Bi T1-weighted (SE 400116 at 1.5 T I images. The high content of methemoglobin at this stage of organization makes the lesion appear hyperintense. The patient also had periventricular
white matter changes on T2-1rrighted MRI (SE 4,000190 at
1.5 TI and a lacune in the right puluinar (Ci.
hours later, she was fully oriented, had restricted up-gaze,
increased convergence tone, a skew deviation of the eyes in
primary position, the right being hypertropic by about 10
degrees, and a 20-degree restriction of right lateral gaze that
could be partially Overcome with the oculocephalic maneuver. At ambient light, both pupils were about 1.5 mm in
diameter and reacted to light.
She could sit up only by pulling herself up with both hands
holding on to the side rails of the bed. On attempts to stand
from a chair, she rose to a semierect position by pushing up
with both arms on the armrests, only to fall backwards into
the chair again while her legs failed to perform any synergistic
standing movements. However, she could draw a cross or a
circle with either leg while sitting in a chair and isometric
strength in the legs was normal. Holding on to a walker
and stooped forward, she could stand and generate short,
shuffling, irregular steps, while being propped up by the examiners. H e r base of ambulation was only minimally wide.
Regular stepping was not initiated either in attempted walking or in response to loss of her balance. The steps were of
irregular amplitude and direction, sometimes directed laterally, lacking any cadence or rhythmicity. The left foot seemed
to step less readily than the right. Often there would be two
steps with one foot while the other remained motionless.
Plantar responses were equivocal. The remainder of the neurological examination was unremarkable, including absence
of tremor and normal finger-to-nose test. The gait abnormalities persisted when examined 4 months after the acute event.
O n magnetic resonance imaging, a recent hemorrhage,
measuring 7 rnm in diameter anteroposteriorly and transversally and 12 mm in diameter rostrocaudally, occupied the
tegmentum of the brainstem on the right side at the pontomesencephalic junction (Fig). Its superior border was near
the inferior aspect of the red nucleus. In addition there were
extensive periventricular hemispheric white matter changes
on T2-weighted images, with mild changes on T1-weighted
images. A small old lacunar infarct was present in the right
620 Annals of Neurology
V O ~35
No 5
May 1994
pulvinar. Magnetic resonance angiography showed normal
vessels in the neck and circle of Willis.
Discussion
Several lesions may have contributed to the sudden
gait failure in this patient. In addition to the obvious
pontomesencephalic hemorrhage, time-locked to her
deficit, white matter changes may have interfered with
alternate compensatory pathways 131. The thalamic lacune is unlikely to have played a role because thalamic
lesions causing inability to stand are centered in the
ventrolateral region, rather than the pulvinar [4]. Although this patient did not have a classical superior
cerebellar peduncular syndrome, it is possible that
involvement of fastigial efferents, which course in the
region of the hemorrhage and project to the vestibular
nuclei, may have contributed to her pronounced postural impairment.
The pedunculopontine area, which contains both
large cholinergic neurons and smaller noncholinergic
neurons, is thought to play a key role in activities involving rhythmic behavior and specifically in ambulation {l}.Its connectivity suggests an important role in
motor behavior. It receives projections from the deep
cerebellar nuclei [ S ] , substantia nigra [GI, and globus
pallidus [?I, as well as from the nucleus locus ceruleus
and the raphe nuclei [S}, ,and projects to the substantia
nigra, globus p&dus, subthalamic nucleus, ventrolateral nucleus of the thalamus, and motor cortex {9], as
well as to pontomedullary reticular nuclei known to
influence the pattern generators in the spinal cord {S,
lo}. In addition to the effects of electric stimulation
mentioned above, the results of injection in this area
of putative neurotransmitters suggest a role for t h e
pedunculopontine area in locomotion. Spontaneous
locomotion, which occurs in the precollicularpremammillary transected cat, can be blocked by the
injection of y-aminobutyric acid (GABA). In contrast,
by giving increasing amounts of GABA antagonists or
substance P, the step cycle frequency can be increased
from a walk to a trot to a gallop [S]. Lesions of the
pedunculopontine nucleus lead to a reduction of locomotor activity in experimental animals [l 11.
Small infarcts or hemorrhages in this area are uncommon. Patient 3 of Sand and co-workers (12) was
reported to be unable to stand on presentation, but
the patient had in addition a right-sided hemiparesis
and a fluctuating level of alertness. The lesion in the
patient reported by Felice and co-workers Cl31 was
higher, affecting the dorsomedial red nucleus and
caused gait ataxia. The third patient of Caplan and
Goodwin r14) could not walk after a small hemorrhage
limited to the dorsolateral midbrain and pons. In reviewing the literature on this topic, Caplan and Goodwin (141 make the point that several patients were
reported to have bilateral ataxla with unilateral lesions.
It is possible that some of these patients had cerebellar
ataxia and others a syndrome similar to the one described here.
The deficit of gait observed in this patient bears
striking resemblance to the gait failure experienced by
many elderly individuals, which in most cases does not
have a clear anatomical correlate (151. Neuropathological studies of the pedunculopontine area in this common disorder of aging may be warranted in view of
the clinical findings in the present case.
References
1. Garcia-hi1 E. The pedunculopontine nucleus. Prog Neurobiol
1991;36:363-389
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Elevated Serum Levels
of Endothelial Leukocyte
Adhesion Molecules
in G d a i n - B a r k Syndrome
and Chronic Inflammatory
Demyelinating
Poly neuropath y
Nobuyuki Oka, MD, Ichiro Akiguchi, MD,
Teruaki Kawasaki, MD, Katsunori Ohnishi, MD,
and Jun Kimura, MD
We determined the serum concentrations of the soluble
form of endothelial leukocyte adhesion molecule-1
(sELAM-1)in 25 patients with Guillain-Barresyndrome,
14 patients with chronic inflammatory dernyelinating
polyneuropathy, 15 patients with amyotrophic lateral
sclerosis, 15 patients with other polyneuropathies, 10 patients with acute stroke, and 12 healthy control subjects.
The patients with Guillain-Barre syndrome in the acute
stage had a higher sELAM-I serum lwel compared with
the control subjects ( p < 0.001). In 7 of the 9 patients
the elevated level returned to normal by 14 days after
the onset of neurological symptoms. In addition, a significantly elevated serum level of sELAM-1 was found
in patients with chronic inflammatory dernyelinating
polyneuropathy ( p < 0.01).These findings suggest a role
for ELAM-1 in the inflammatory process of these disorders.
Oka N , Akiguchi 1, Kawasaki T, Ohnishi K,
Kimura J. Elevated serum levels of endothelial
leukocyte adhesion molecules in Guillain-Barrk
syndrome and chronic inflammatory demyelinating
polyneuropathy. Ann Neurol 1994;35:621-624
9. Nakano K, Hasegdwd Y , Tokushige A,
et al. Topographical
projections from the thalamus, subthalamic nucleus and pedunculopontine tegmental nucleus to the striatum in the Japanese
monkey, iMucuru fuscuta. Brain Res 1990;537:54-68
10. Grofova I, Keane S. Descending brainstem projections of the
pedunculopontine tegmental nucleus in the rat. Anat Embryo1
( B e d 1991:184:275-290
11. Mogenson G, Wu M, Brudzynski S. The role of pedunculopontine nucleus in locomotor activity. Neurosci Abstr 1990;16:753
(Abstract)
12. Sand J, Biller J, Corbett J, et al. Partial dorsal mesencephalic
hemorrhages: report of three cases. Neurology 1986;36:529-
533
The immune mechanisms in Guillain-Barre syndrome
(GBS) and chronic inflammatory demyelinating polyFrom the department of Neurology, Faculty of Medicine, Kyoto
University, Sakyo-ku, Kyoto, Japan
Received Aug 13, 1993, and in revised form Nov 8 Accepted for
publication Nov 11, 1993
Address correspondence to Dr Oka, Department of Neurology, Kyoto University Hospital, Shogoin, Sakyo-ku, Kyoto 606-01, Japan
Copyright 8 1994 by the American Neurological Association 621
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