hemiballism in multiple sclerosis

Movement Disorders Vol. 3, No. 1, 1988, pp. 88-94 8 1988 Movement Disorder Society

Case Report

Hemiballism in Multiple Sclerosis’

David Riley and Anthony E. Lang

Movement Disorders Clinic, Toronto Western Hospital, Toronto, Ontario, Canada

Summary: Hemiballism has rarely been reported in the setting of multiple sclerosis. Review of clinical descriptions cast some doubt on the accuracy of the diagnosis of the movement disorder in several previous cases. We report a patient with definite multiple sclerosis, with videotape documentation of hemiballism, and demonstration of a plaque in the contralateral subthalamic nucleus on a magnetic resonance image. Key Words: Hemiballism-Multiple sclerosis.

Ballism is an unusual movement disorder “characterized by the fairly abrupt onset of more or less extensive, vigorous, rapidly executed, poorly patterned, nonadaptive and seemingly purposeless activities of appendicular, truncal andor faciocephalic striated muscles” (1). Emphasis has been placed on the “violent and flinging” appearance of the movements and the involvement of proximal limb musculature (2). Clinically, it most often presents in a unilateral fashion, known as hemiballism, as a consequence of cerebrovascular disease (hemorrhage or in- farction). Other causes of focal intracerebral lesions have produced hemiballism, including trauma and metastatic brain tumors. Multiple sclerosis is an extremely rare cause; the actual incidence is difficult to determine because of lack of unifor- mity in the diagnosis of ballism. We report a case of a young woman with hemiballism complicating multiple sclerosis, with videotape presentation of the movement disorder, and magnetic resonance imaging of the likely responsible lesion.

CASE REPORT The patient is a 25-year-old woman who had suffered six episodes of remitting

variable neurologic disturbances since October 1983. Previous symptoms and signs included a right internuclear ophthalmoplegia, ocular dysmetria, bilateral complete external ophthalmoplegia, a right Homer’s syndrome, positional ver-

Videotape segments accompany this article. Address correspondence and reprint requests to Dr. A. E. Lang at 25 Leonard Ave. #301, Toronto,

Ontario, MST 2R2, Canada.

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HEMIBALLISM IN MULTIPLE SCLEROSIS 89

tigo, hyperreflexia in the lower limbs (later generalized), bilateral limb ataxia (worse on the left), numbness and paraesthesias on the left side, a broad-based gait, nocturia, a i d fatigue. Oligoclonal bands were present in the cerebrospinal fluid. A computed tomography (CT) scan of the head with routine contrast had been normal on two occasions. By December 1986, her examination disclosed no abnormalities except for difficulty performing tandem gait.

In March 1987, she developed dysarthria and persistent involuntary movements of her left side. On examination in April 1987 there was a mild slurring of speech. Her extraocular movements showed mildly saccadic pursuit to the left. At rest there were only occasional abnormal involuntary movements in the left limbs and face (Videotape Segment 1). With any action, and particularly when attempting to maintain a posture, there were uncontrollable, large-amplitude, random movements of the left arm, mainly about the shoulder joint. Similar but less frequent movements of the left leg were noted. Muscle bulk, tone, strength, and coordination (when not impaired by the abnormal movements) were normal. Tendon reflexes were uniformly brisker on the left than the right, but the plantar responses were flexor bilaterally. There was a mild reduction in light touch sensation on the left side; proprioception, graphaesthesia and vibration and pinprick sensation were normal. The remainder of the examination was normal.

Laboratory studies that were normal (or negative) include routine serum chem- istry, a complete blood count, VDRL, thyroid function tests, and anti-nuclear antibody. Visual evoked potentials (EP) were normal. Brainstem auditory EP were of low amplitude, and the interpeak latencies could not be calculated. So- matosensory EP from the median nerves were normal; those from the tibia1 nerves showed bilateral slowing of conduction between the lumbothoracic region and the vertex, with the latency significantly slower from left-sided stimulation than from right. A T2-weighted magnetic resonance scan demonstrated numerous high-intensity lesions typical of plaques of demyelination scattered throughout the intracerebral white matter, most prominent in the corona radiata and periven- tricular white matter (Fig. IA). The largest plaque was located in the region of the subthalamic nucleus on the right side (Fig. 1B).

She was treated with tetrabenazine 75 mg/day and prednisone 45 mg/day. The following day her movements had begun to diminish in frequency and intensity, but within 3 days she had developed a feeling of “heaviness” in her right arm and leg. Examination disclosed moderate right-sided bradykinesia and rigidity. The tetrabenazine was discontinued with reversal of the parkinsonism but at the cost of a resurgence of her hemiballism to its previous level. On prednisone 40 mg/day (then tapering) alone, the hemiballism gradually subsided over 2 weeks. How- ever, reduction of the dose from 20 to 15 mg/day resulted in the return of her involuntary movements. A trial of valproate has yielded no benefit (Videotape Segment 2).

DISCUSSION This patient meets the diagnostic criteria for “laboratory-supported definite

multiple sclerosis,” proposed by Poser et al. (3). During an exacerbation of her

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90 D. RILEY AND A . E. LANG

FIG. 1. Magnetic resonance images demonstrating (A) multiple plaques of demyelination in both hemispheres, and (B) a large plaque involving the right subthalamic nucleus. (General Electric 1.5 Tesla, TR = 2000, TE = 35).

condition, she developed typical hemiballism. When anatomic correlation is available, the vast majority of cases of hemiballism demonstrate a lesion involving the contralateral subthalamic nucleus. However, well-documented in- stances of hemiballism with sparing of subthalamic nuclei have been associated with lesions of subthalamic afferent and efferent fibers, the striatum, the globus pallidus, the thalamus, and-in one case-the postcentral gyrus (1,4-6). Al- though our patient had multiple plaques seen on magnetic resonance imaging, the large striking lesion in the region of the right subthalamic nucleus (Fig. lb) was almost certainly the cause of her involuntary movements.

There have been several previously reported cases of ballism in patients with multiple sclerosis (Table 1). Mouren et al. (7), in an exhaustive review of 177 reports of hemiballism, claimed a total of six cases, including one of their own, Closer scrutiny of these case reports reveals that their “critical study” used loose criteria for the diagnosis of ballism. Only one of these cases described the typical movements of ballism in sufficient detail to make the diagnosis readily acceptable. Hyland and Forman (8) (case 14) described “extensive, flinging, involuntary movements of the [right] arm and leg” in a 42-year-old man with a 17-year history of multiple sclerosis. Anatomic correlation was not available.

Touche (9) described a 32-year-old man with an 8-year history of multiple sclerosis who developed “choreiform” movements about the left wrist, elbow, and shoulder. At autopsy, a plaque involving the right subthalamic nucleus was found. A similar case was reported by Guillain and Mollaret (10) of a 32-year-old man with a 4-year history of multiple sclerosis who demonstrated “large movements

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TABLE 1. Ballism in multiple sclerosis

Reference Syndrome Subthalamic lesion

Touche (9)

Herman (12)

Guillain and Mollaret (10, I I ) Grigoresco (14, see case H.M.)

Hyland and Forman (8, see

Mouren et al. (7)

Thiery and de Reuck (IS)

Riley and Lang (present case)

case 14)

“Choreiform” left arm movements

Bilateral upper limb “hemiballism”

Left arm monoballism Action-induced oscillations

head and upper limbs Right hemiballism

Stereotyped ballistic

Stereotyped ballistic

Left hemiballism

movements of right arm

movements of right arm

Right STN plaque at autopsy

“Plaques in basal ganglia” at


Unknown

autopsy

of Negative


Negative

Right STN plaque on MR scan

STN, subthalamic nucleus; MR, magnetic resonance.

reminiscent of hemiballismus” in the left arm. Pathologic correlation with a lesion in the contralateral subthalamic nucleus was provided in a later publication ( 1 1).

The three remaining case descriptions are less convincing. Herman (12) reported a patient with bilateral upper extremity “involuntary movements of variable character: hemiballismus (sic), parkinsonian tremor and choreiform.” The pathologic description mentions only plaques “in the basal ganglia” without further qualification. Although the lower extremities were not said to be involved, this case may represent an instance of “bilateral ballism” which may be due to diffuse bilateral basal ganglia rather than specific subthalamic nucleus lesions (13). Grigoresco’s (14) patient (case H.M.) suffered from “disordered, choreiform” oscillating movements of the upper extremities, as well as of the head, occurring only briefly following movement of the body in bed or changing from a supine to a sitting position. The description suggests titubation and ataxia much more than ballism. At autopsy there were numerous plaques, but none involving the subthalamic nuclei. Mouren et al.’s (7) own patient, a 25-year-old woman with a 10-year history of multiple sclerosis, demonstrated bursts of stereotyped “violent shakes” affecting the right arm. The authors admitted that their case was “a little atypical” for monoballism; however, a plaque involving the left subthalamic nucleus was found at autopsy.

Since Mouren et al.’s (7) review appeared, there has been one further report of “monoballism” in multiple sclerosis. Thiery and de Reuck (15) described a patient remarkably similar to the previous case (7). A 62-year-old woman with a 15-year history of multiple sclerosis demonstrated a movement disorder that had been present from the age of 50 years. It consisted of bursts of stereotyped movements of the right arm, “hitting the trunk always in the same way. Adduction of the shoulder, flexion of the elbow, and internal rotation of the entire arm made the back of the hand slap the side of the chest. After several of these movements,

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92 D. RILEY AND A . E. LANG

the phenomenon disappeared abruptly.” At autopsy, there were no lesions in the subthalamic nuclei, but an old plaque was found in the decussation of the left superior cerebellar peduncle.

The uncertainty in accepting some of these case reports as true examples of ballism underscores the difficulty of accurately depicting movement disorders verbally. An additional diagnostic problem is variability in the definition of ballism; this very question undermines the validity of the conclusions regarding incidence, etiology, and pathogenesis drawn from Mouren et al.’s (7) monumental work. Although controversy in definition and classification of movement disorders will persist (i.e., “the eye of the beholder” will remain the final arbiter in diagnosing many movement disorders ), the advent of the videotape medium for medical reporting as pioneered in this journal (16) promises to end much of the diagnostic confusion which plagues the older literature.

Although demyelinating disease is logically regarded as a disease of white matter, the gray matter of the cerebral hemispheres is frequently involved. Lumsden (17) found 6.8% of multiple sclerosis plaques involved the basal ganglia. By contrast, movement disorders are generally quite rare in multiple sclerosis: those that are relatively common (Table 2) have pathologic substrates outside the basal ganglia. However, a wide spectrum of other movement disorders has been reported (Table 2).

Our patient developed disabling right hemiparkinsonism within three days of beginning tetrabenazine 75 mg/day. It is interesting to speculate that subclinical involvement of the nigrostriatal complex on the left may have predisposed her to the early manifestation of this complication, while the subthalamic lesion may have masked the possible development of similar signs on the left side.

Acknowledgment: Thanks to Somchai Jiaravuthisan, M.D. for refemng this patient. This work was supported in part by the United Parkinson Foundation (Chicago, IL, U .S .A . ) and the Parkinson’s Disease Foundation (New York, NY, U.S.A.)

TABLE 2. Movement disorders in multiple sclerosis

Common Cerebellar tremor Facial myokimia Tonic spasms Palatal myoclonus

Hemiballism (Table 1) Generalized dystonia (18) Focal dystonia (torticollis) (19, videotape segment 3) Occupational (pianist’s) dystonia (20) Chorea (21, videotape segment 3) Athetosis (21) Choreoathetosis (18,21,22) Parkinsonian (resting) tremor (10,12,14,23) Akinesia with freezing episodes (19.20)

Uncommon

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LEGENDS FOR VIDEOTAPE

SEGMENT 1: April 24, 1987. The patient demonstrates occasional spontaneous movements of the left side of the face, particularly about the eye and corner of the mouth. There are intermittent spontaneous movements of the left arm at rest. With activity there are frequent movements of sufficient amplitude and speed to cause striking of the hand against the face or a nearby wall. Similar though less pronounced movements, mainly of a rotational nature, are seen in the left leg. As the patient mentions at the beginning, the movements had been more continuous and severe earlier in the course. She had received haloperidol 0.5 mg the night before and the day of the videotaping and this had dampened the movements somewhat.

SEGMENT 2: June 22, 1987. After the patient’s movements returned during withdrawal of prednisone, she began taking valproic acid 1 g/day. There is very little change in the control of her limb movements. In this segment the leg movements actually appear worse. The brief period of rhythmic movements seen while holding the hands before the face probably relates to a voluntary attempt to over- come the involuntary movements (a phenomenon more commonly recognized in dystonia).

SEGMENT 3: Another example of movement disorders in multiple sclerosis. This 37-year-old woman had a history of waxing and waning neurologic dysfunc- tion dating back 15 years. She had a 7-year history of involuntary head turning to the right (spasmodic torticollis). In 1984, she underwent denervation of the left sternocleidomastoid muscle. In 1985, she underwent a left stereotactic thalamo- tomy for severe ataxic tremor of the right upper limb. Her family had noted gri- macing and “fidgeting” for many years. The videotape shows residual rightward torticollis, obvious limb and gait ataxia, and irregular random movements of the distal extremities, face, and trunk, consistent with chorea. In summary, this patient demonstrates both chorea and spasmodic torticollis, representing only the second known example of the latter movement disorder complicating multiple sclerosis. (Thanks to Trevor Gray, M.D. for referring this patient.)

ADDENDUM A recent review of ballism (24) proposes a report by Riechen et al. (25) (case 2) as an

example of hemiballism complicating multiple sclerosis. The case in question concerns a patient who developed a unilateral movement disorder after an encephalitic illness before the age of two years. This movement disorder is variously described as “hemiathetosis,” “hemiballistic” and “action myoclonus and shaking”, and was associated with hemiat- rophy and spastic hemiparesis. At age 43, the patient developed a “shaking palsy” of the right arm. At autopsy, there was bilateral status marmoratus, much more extensive on the right. There were several foci of demyelination in the brainstem as well. Again, as with other reports reviewed in our paper, this cannot be accepted as a case of hemiballism caused by multiple sclerosis. It may represent, however, an example of focal parkinsonism due to demyelination, since plaques were found in both substantia nigra.

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hemiballism in multiple sclerosis

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