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Focal Limb Dystonia in a Patient With a Cerebellar Mass
Fernando Alarcón, MD;
Eduardo Tolosa, MD;
Esteban Muñoz, MD
Arch Neurol. 2001;58:1125-1127.
ABSTRACT
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Background Focal dystonia of acute onset is indicative of a structural lesion in
the nervous system. Cerebellar lesions have rarely been associated with dystonia.
Case Description A 42-year-old woman was admitted to the neurology ward because of fever,
confusion, and gait unsteadiness. She was diagnosed as having tuberculous
meningitis, and, after a few days of antituberculous treatment, she developed
prominent dystonia of the left upper limb. Cranial nuclear magnetic resonance
imaging showed an isolated lesion compatible with a tuberculoma in the left
cerebellar hemisphere. Both the limb dystonia and the tuberculoma resolved
with maintained antituberculous treatment.
Conclusions In the patient described, the presence of upper-limb dystonia ipsilateral
to a focal cerebellar lesion and the resolution of the dystonia and the mass
lesion following treatment suggest that the cerebellum or its connections
to the thalamus and/or basal ganglia could be involved in the pathophysiology
of the dystonia.
INTRODUCTION
DYSTONIA IS characterized by inappropriately prolonged muscle contractions
that forcefully distort the body into abnormal postures. Most cases of dystonia
are idiopathic. Acute onset of dystonia, particularly when it begins focally
in a limb and remains unilateral, suggests the possibility of a focal central
nervous system structural disease. The central nervous system sites that have
been most frequently implicated as causing secondary dystonias are the thalamus
and the striatopallidal complex.1, 2
Other sites include, more rarely, the spinal cord, the brainstem, and the
cerebral cortex.1, 3, 4, 5, 6, 7
Lesions that primarily involve the cerebellum or its pathways have not been
classically associated with dystonia. We report a case of a patient with left
upper-limb dystonia of acute onset associated with a cerebellar tuberculoma.
REPORT OF A CASE
A 42-year-old woman with a history of pulmonary tuberculosis in her
husband and 4 children was admitted to the hospital because of signs of apathy,
irritability, anorexia, and restlessness at night. Four weeks before admission,
she developed headaches, nausea, vomiting, abdominal pain, urinary incontinence,
and generalized tonic-clonic seizures. Two weeks later, she displayed daytime
sleepiness, fever, and gait instability. On admission, she had a fever and
was confused, with a tendency toward sleepiness. Her speech was slow. Funduscopic
examination revealed absence of venous pulsations, but the papilla boundaries
were well delineated. Pupils were normal and reactive to light. Nystagmus
at the right lateral gaze was observed. She had mild right facial and limb
paresis. Tendon reflexes were normal and symmetrical. The right plantar response
was equivocal and the left was flexor. She had a left cerebellar syndrome
with upper-limb intention tremor and with finger-to-nose and heel-knee-shin
ataxia. Her gait was unsteady. Findings of sensory examination, including
proprioception, were normal.
Results of routine laboratory tests, chest x-rays, and electrocardiogram
were normal. A cranial computed tomographic scan showed hydrocephalus. Cerebrospinal
fluid examination showed 170 cells/mL, with 95% of them mononuclear; proteins,
300 mg/dL; and glucose; 55 mg/dL (3.0 mmol/L). The result of a Mantoux test
was positive. A diagnosis of tuberculous meningitis was made, and antituberculous
treatment was started with ethambutol, isoniazid, rifampicin, and pyrazinamide.
The result of a Lowenstein-Jensen culture and an enzyme-linked immunosorbent
assay for tuberculosis confirmed the diagnosis.
After 2 weeks of antituberculous treatment, the patient had improved
but still had mild intermittent confusion. She then developed involuntary
movements and postures in the left arm. At rest, she kept a dystonic posture
in her left arm with wrist flexion and slight internal rotation in the arm
and hand, which tended to become more intense with action. When she was asked
to keep her arms outstretched, marked dystonic posture of the left arm developed,
with marked internal rotation of the arm and hand and downward wrist flexion
accompanied by adduction of the thumb and hyperextension of the fingers. When
standing up, she also showed an abnormal limb posture, which was more evident
when walking, showing internal rotation and bringing backward the upper left
limb with flexion of the wrist and metacarpophalangeal joints and adduction
of the thumb. The patient also displayed a postural and intentional tremor
of the upper limbs that was more pronounced on the left side. There was a
mild right hemiparesis with ataxia. No sensory deficits could be demonstrated.
Cranial nuclear magnetic resonance imaging (NMRI) showed hydrocephalus and
a 4-cm left cerebellar lesion, predominantly vermian and paravermian, that
was hyperintense on T2-weighted sequences and enhanced with gadolinium (Figure 1). This lesion was compatible with
tuberculoma.
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A, Nuclear magnetic resonance imaging T1-weighted spin-echo (T1wSE)
image, without contrast, shows a hyperintense cerebellar lesion adjacent to
the fourth ventricle involving predominantly the left hemisphere ventrally
and the right hemisphere dorsally. Temporal horn dilation secondary to hydrocephalus
is also present. B, Axial T1wSE image shows multiple ring and nodular contrast-enhancing
lesions at the cerebellar vermis and adjacent hemisphere, involving mostly
the left hemisphere ventrally and the right hemisphere dorsally. C, Coronal
T1wSE image shows a left paravermian nodular, contrast-enhancing lesion, with
compression of the fourth ventricle and dilation of the occipital horns of
the lateral ventricles.
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Ten days later, dystonia of the upper limb had improved, but gait ataxia
persisted for 2 months. After 90 days of treatment, the patient was free of
symptoms. A new NMRI of the brain was normal.
COMMENT
We report a case of a patient with tuberculous meningitis who, 2 weeks
after starting antituberculous therapy, developed prominent upper-limb dystonia
of acute onset. Cranial NMRI showed a hyperintense lesion on T2-weighted sequences
in the cerebellar hemisphere ipsilateral to the dystonic arm that was enhanced
with gadolinium, but no other focal structural lesions were seen. The cerebellar
lesion was compatible with a tuberculous granuloma. Both limb dystonia and
the cerebellar lesion disappeared with antituberculous treatment. Other neurological
symptoms and signs displayed by our patient, such as confusion or hemiparesis,
could be related to hydrocephalus or to extracerebellar pathologic conditions
not visualized on the cranial NMRI.
Cerebellar disorders are typically not associated with dystonic phenomena,
and the cerebellum or its pathways have not been usually considered in the
pathophysiology of dystonia. In certain neurodegenerative disorders of the
cerebellum, brainstem, and spinal cord, cerebellar ataxia and dystonia can
coexist,8, 9, 10 and
focal dystonia of the hand has been described in cases of isolated degenerative
ataxia.11 In these cases of focal dystonia
and isolated ataxia, the cerebellar syndrome was more prominent on the side
of the dystonic limb, but the authors consider this type of dystonia to be
the result of presumed abnormalities of the basal ganglia or their connections.11 Still, other evidence suggests that the cerebellum
or its projections could be involved in dystonic disorders. Human deep cerebellar
stimulation can result in abnormal tonic postures of the neck and limbs.12 Several cases of blepharospasm associated with brainstem
lesions had clinical or radiological evidence of cerebellar involvement.13, 14 A recent case report suggested that
symptomatic limb dystonia could be produced by focal brainstem lesions and
that cerebellar dysfunction could play a role in its pathophysiology.4 Another study,15 conducted
with positron emission tomography, emphasized the participation of structures
such as the cerebellothalamic pathways in the genesis of dystonic phenomena.
In experimental animals, brainstem lesions involving the medial longitudinal
fasciculus, medial reticular formation, red nucleus, and brachium conjunctivus
have produced dystonia.4, 5, 6
Finally, it has been suggested that the damage of a single subpopulation of
cerebellar neurons could also produce dystonia.16
In our patient, the coexistence of a unilateral cerebellar syndrome
in the same arm with the focal dystonia and the presence on NMRI of a cerebellar
lesion ipsilateral to the dystonic syndrome suggest a cerebellar origin for
the abnormal involuntary movement. The concomitant disappearance of the focal
dystonia and of the cerebellar mass following treatment also supports this
interpretation. Deep small or asymmetrical lesions in or around the basal
ganglia secondary to the presence of basilar exudate that were not apparent
in the imaging studies could have conditioned the abnormal movements in our
patient. However, recent studies in patients with tuberculous meningitis show
that abnormal movements are more frequently associated with the presence of
demonstrable deep infarcts on imaging studies.17
The absence of these lesions and the presence of a single cerebellar mass
suggest that the dystonia in our patient could be cerebellar in origin.
A cerebellar lesion involving the dentate nucleus or its efferent pathways
could cause abnormal dystonic movements and postures ipsilateral to the cerebellar
lesion through an abnormal functional input to the contralateral thalamus.
In our patient, the left cerebellar lesion, through edema, direct pressure
effect, or ischemia, could have compromised the cerebellar input to the contralateral
thalamus, leading to contralateral thalamofrontal disinhibition and abnormal
central sensorimotor processing,18 which could
have resulted in dystonic movements ipsilateral to the cerebellar lesion.
Secondary focal dystonias have been encountered most often with lesions
of the contralateral thalamus and of the lentiform and caudate nuclei, and
only rarely in patients with brainstem lesions. The association of arm dystonia
in a patient with an ipsilateral cerebellar tuberculoma, described in this
report, and the available evidence from the literature support the concept
that a cerebellar lesion may cause or significantly contribute to the development
of a focal dystonia in some patients.
AUTHOR INFORMATION
Accepted for publication October 30, 2000.
From the Department of Neurology, Eugenio Espejo Hospital, Quito, Ecuador
(Dr Alarcón), and Neurology Service, Institute Clínic Malaltias
del Sistema Nervós, Hospital Clínic Universitari, Universitat
de Barcelona, Barcelona, Spain (Drs Tolosa and Muñoz).
Corresponding author and reprints: Fernando Alarcón, MD, Department
of Neurology, Eugenio Espejo Hospital, PO Box 17-07-9515, Quito, Ecuador (e-mail: falarcon{at}ramt.com).
REFERENCES
| |
1. Marsden CD, Obeso JA, Zarranz JJ, Lang AE. The anatomical basis of symptomatic hemidystonia. Brain. 1985;108:463-483.
FREE FULL TEXT
2. Obeso JA, Giménez-Roldán S. Clinicopathological correlation in symptomatic dystonia [review]. Adv Neurol. 1988;50:113-122.
PUBMED
3. Berardelli A, Thompson PD, Day BL, Rothwell JC, O'Brien MD, Marsden CD. Dystonia of the legs induced by walking or passive movement of the
big toe in a patient with cerebellar ectopia and syringomyelia. Neurology. 1986;36:40-44.
FREE FULL TEXT
4. Esteban Muñoz J, Tolosa E, Saiz A, Vila N, Marti MJ, Blesa R. Upper-limb dystonia secondary to a midbrain hemorrhage. Mov Disord. 1996;11:96-99.
FULL TEXT
|
ISI
| PUBMED
5. Plant GT, Kermode AG, du Boulay EPGH, McDonald WI. Spasmodic torticollis due to a midbrain lesion in a case of multiple
sclerosis. Mov Disord. 1989;4:359-362.
FULL TEXT
|
ISI
| PUBMED
6. Zweig RM, Hedreen JC. Brainstem pathology in cranial dystonia. Adv Neurol. 1988;49:395-407.
PUBMED
7. Leenders KL, Frackowiak RSJ, Quinn N, Brooks D, Sumners D, Marsden CD. Ipsilateral blepharospasm and contralateral hemidystonia and parkinsonism
in a patient with a unilateral rostral brainstem-thalamic lesion: structural
and functional abnormalities studies with CT, MRI, and PET scanning. Mov Disord. 1986;1:51-58.
FULL TEXT
| PUBMED
8. Iizuka R, Hirayama K, Maehara K. Dentato-rubro-pallido-luysian atrophy: a clinico-pathological study. J Neurol Neurosurg Psychiatry. 1984;47:1288-1298.
ABSTRACT
9. Barbeau A, Roy M, Cunha L, et al. The natural history of Machado-Joseph disease: an analysis of 138 personally
examined cases. Can J Neurol Sci. 1984;11(suppl 4):510-525.
10. Duvoisin RC. The olivopontocerebellar atrophies. In: Marsden CD, Fahn S, eds. Movement Disorders
2. London, England: Butterworth-Heinemann; 1987:249-269.
11. Fletcher NA, Stell R, Harding AE, Marsden CD. Degenerative cerebellar ataxia and focal dystonia. Mov Disord. 1988;3:336-342.
FULL TEXT
|
ISI
| PUBMED
12. Nashold BS Jr, Slaughter G. Effects of stimulating or destroying the deep cerebellar regions in
man. J Neurosurg. 1969;31:172-186.
ISI
| PUBMED
13. Jankovic J, Patel SC. Blepharospasm associated with brainstem lesions. Neurology. 1983;33:1237-1240.
FREE FULL TEXT
14. Jankovic J. Blepharospasm associated with palatal myoclonus and communicating hydrocephalus. Neurology. 1984;34:1522-1524.
FREE FULL TEXT
15. Vidaihet M, Lehéricy S, Remy P, et al. Midbrain dystonia: anatomo-clinical study using 3D magnetic resonance
imaging and fluorodopa positron emission tomography [abstract]. Neurology. 1997;48(suppl 2):A62.
16. Hedreen JC, Zweig RM, DeLong MR, Whitehouse PJ, Price DL. Primary dystonias: a review of the pathology and suggestions for new
directions of study. Adv Neurol. 1988;50:123-130.
PUBMED
17. Alarcón F, Dueñas G, Cevallos N, Lees AJ. Movement disorders in 30 patients with tuberculous meningitis. Mov Disord. 2000;15:561-569.
FULL TEXT
|
ISI
| PUBMED
18. Hallett M. Is dystonia a sensory disorder [editorial]? Ann Neurol. 1995;38:139-140.
FULL TEXT
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ISI
| PUBMED
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