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Intern. J . Neuroscience, 1992, Vol. 62, p. 213-215 Reprints available directly from the publisher Photocopying permitted by license only
Letter to the Editor DYSTONIA AS THE PRESENTING MANIFESTATION OF MULTIPLE SCLEROSIS
Int J Neurosci Downloaded from informahealthcare.com by UB Giessen on 01/21/15 For personal use only.
(Received April 1 2 , 1991)
To the Editor: Involuntary movement disorders are an infrequent presentation of multiple sclerosis (MS). Despite the rather frequent presence of plaques in the area of the basal ganglia (e.g., thalamus, subthalamic nucleus) (McAlpine et al., 1972) and the occurrence of demyelinating lesions in the basal ganglia in 6.8% of patients with multiple sclerosis (MS) (Lumsden, 1970), only a few cases of resting tremor, hemi- or monoballism, athetosis, or choreoathetosis have been reported in MS patients during the course of their illness (Sarkari, 1968; Thiery and De Reuck, 1974; Bachman et al., 1976; Taff et al., 1985; Riley and Lang, 1988). Most of the movement disorders seen in MS such as intention tremor, myokymia, myoclonus, and tonic spasms have pathologic substrates outside the basal ganglia (Berger et al., 1984; Riley and Lang, 1988). We present a patient in whom segmental dystonia was the initial presentation of MS. A 38-year old right-handed woman was seen in the emergency department of Bay City Medical Center for the evaluation of speech disturbances and muscle spasms of the left arm and hand of two days duration. Initially, she noted that intermittently her hand assumed a “claw-like” deformity, but reported no functional loss of the left arm or hand. However, over the following 24 hours her symptoms appeared to worsen as she developed dysarthria and, in addition, her hand assumed a constant “claw-like’’ position. On the morning prior to examination, she experienced numbness of the left side of her body. Her past medical history was negative for cerebrovascular disease, seizures, syncope, and head trauma. In addition, there was no history of hypertension, diabetes, or thyroid disease and she denied alcohol or drug use. The patient had a history of a unervous breakdown,” but had not been on any type of psychotrophic drugs. Family history was negative for psychiatric or neurological disorders. On examination the patient was alert and cooperative. Intermittent dystonic facial grimaces were observed during her interview. Her mood was somewhat euphoric. She was afebrile with normal vital signs, and her neck was supple with no meningeal signs or carotid bruits. Her speech was markedly dysarthric and spastic, but there was no aphasia or anomia. She was oriented to person, place, and time. Examination of the cranial nerves showed normal optic nerves with no visual field defects. Her extraocular movements were full and there was no diplopia or blepharospasm. Facial sensation was decreased on the left. Tongue movements were clumsy and she was unable to move the tongue past the midline. Motor examination showed mild weakness in the left upper extremity, mostly in grip strength. Her left hand was fixed in a continuous “claw-like” deformity with more prominent flexion Correspondence to: Gavin I. Awerbuch, M.D. 200 S. We n o n a Avenue, Suite 298, B a y City, Michigan
48706, U.S.A. 273
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posturing of the ring and little fingers. The patient was unable to extend the fingers at the MP joints past about 30" to 40" of flexion and she experienced pain with movement in the distal interphalangeal joint of the left hand. There was mild drift with left arm extension. Muscle tone was increased in the left arm and leg. Sensory examination revealed loss of pinprick, vibration, and propioception over the entire left side of the body. There was no ataxia or dysmetria within the limitations of the spasms. Reflexes were symmetrical and plantar responses were flexor bilaterally. The patient's signs and symptoms of an acute onset progressive dystonia of the left arm and hand, dysarthria, and spasticity, raised the diagnostic possibility of acute cerebrovascular disease in the basal ganglia or brainstem. Laboratory studies including routine serum chemistries, a complete blood count, VDRL, thyroid function tests, liver enzymes (GPT, GOT, LDH), antinuclear antibody, EEG, visual evoked responses, somatosensory evoked responses in the upper and lower extremities, and auditory brainstem evoked responses were negative or normal, respectively. Serum IgG was 1575 mg/dl (normal: 540-1480 mg/dl) and oligoclonal (IgG) bands were absent. Serum albumin was low (3.1 g/dl) (normal: 3.5-5.5 g/dl). Lumbar cerebrospinal fluid (CSF) analysis revealed absence of oligoclonal bands, normal IgG level (2.0 mg/dl), an IgG index of 0.4 (normal 0-.7), an IgG/albumin ratio of .22 (normal: 0-.25), and albumin concentration of 9 mg/dl (normal: 1148 mg/dl). Myelin basic protein level was normal (< .5 ng/ml) (normal: < 4.0 ng/ ml). A noncontrast CT scan of the brain was normal. A TZweighted MRI scan revealed numerous high-intensity lesions typical of plaques of demyelination scattered throughout the intracerebral white matter, most prominent in the corona radiata and periventricular white matter. Several lesions were noted also in the basal ganglia region with predominance on the right side. These lesions were characteristic of and compatible with the diagnosis of MS. The patient was placed on baclofen (15 rng/day) and her dystonic movements gradually resolved over the following two weeks. Early symptoms and signs of MS usually include weakness and/or numbness in one or more limbs. In about 25% of patients, the initial manifestation of MS is an episode of retrobulbar neuritis. Not infrequently the disease may present with ataxia and nystagmus, with or without weakness and spasticity of the limbs (Adams and Victor, 1985). Dystonia, an abnormal involuntary movement defined by the presence of sustained posturing as well as tonic and clonic spasms of different parts of the body (Oppenheimer, 191l ) , is a distinctly uncommon feature of MS. Most patients with dystonia have primary torsion dystonia, which is either sporadic or hereditary (Fahn, 1984). The primary dystonias, by and large, are slowly progressive disorders that can plateau anywhere in the course of the illness. Characteristically, they begin insidiously and almost always with action dystonia. Only with progression of time do dystonic postures develop (Fahn and Jankovic, 1984). In contrast, most secondary dystonias frequently present with dystonia at rest, have an obvious sudden beginning, and tend to have a course that stabilizes and does not progress (Fahn and Jankovic, 1984). Secondary dystonia, which may be either focal when only a single body part is involved, or segmental when more than one body part is involved (Fahn, 1984), may result from structural brain lesions (i.e., cerebrovascular disease, head trauma, encephalitis), metabolic diseases (e.g., Wilson's disease), toxic exposure (e.g. , carbon monoxide), or degenerative neurological disorder (e.g., progressive supranuclear palsy) (Fahn and Jankovic, 1984; Pettigrew and Jankovic, 1985). Dystonic postures of the hands and feet are frequently seen in pa-
Int J Neurosci Downloaded from informahealthcare.com by UB Giessen on 01/21/15 For personal use only.
LETTER TO THE EDITOR
275
tients with advanced stages of Parkinson’s disease and in Parkinsonian patients receiving dopaminergic therapy (Jankovic, 1982; Fahn and Jankovic, 1984). Sparing of the corticospinal tract with disruption of the pathways between the striatum, pallidum, and thalamus is probably essential for the development of segmental dystonia (Dooling and Adams, 1975). In the series reported by Pettigrew and Jankovic (1985), all but two of the 22 cases of segmental dystonia had CT scan evidence of focal lesions in the striatum with no apparent evidence of corticospinal involvement in the internal capsule. This patient had multiple periventricular and basal ganglia lesions, the latter accounting for the development of segmental dystonia. However, unlike previous reports in which involuntary movements in MS occurred during the course of the disease, this case is remarkable in that dystonia was the presenting features of the disease. In view of the fact that in 6.8% of MS patients demyelinating lesions have been demonstrated in the basal ganglia (Lumsden, 1970), we suggest that MS be considered in the differential diagnosis of young adults presenting with an acute-onset focal or segmental dystonia.
REFERENCES Adams, R. D. & Victor, M. (1985). Principles of Neurology. New York: McGraw-Hill Book Company, pp. 699-717. Bachman, D. S . , Lao-Velez, C. & Estanol, B. (1976). Dystonia and choreoathetosis in multiple sclerosis. Archives of Neurology, 33, 590. Berger, J . R., Sheremata, W. A. & Melamed, E. (1984). Paroxysmal dystonia as the initial manifestation of multiple sclerosis. Archives of Neurology, 4 1 , 747-750. Dooling, E. C. & Adams, R. D. (1975). The pathological anatomy of posthemiplegic athetosis. Brain, 98, 29-48. Fahn, S . (1984). The varied clinical expression of dystonia. Neurologic Clinics, 2, 541-554. Fahn, S . & Jankovic, J. (1984). Practical management of dystonia. Neurologic Clinics, 2, 555-569. Jankovic, I. (1982). Management of motor side effects of chronic levodopa therapy. Clinical Neuropharmacology, 5, 519-528. Lumsden, C. E. (1970). The neuropathology of multiple sclerosis. In P. J . Vinken & G. W. Bruyn (Eds.). Handbook of clinical neurology, vol. 9, Amsterdam: North Holland, pp. 217-309. McAlpine, D., Lumsden, C . E. & Acheson, E. D. (1972). Multiple sclerosis: a reappruisel. Baltimore: Williams & Wilkins Co., pp. 177-178. Oppenheimer, H. (191 1). “Uber eine eigenartige Krampfkrankheit des kindlichen und jugendlichen Alters (dysbasia lordotica progressiva, dystonia musculorum deformans). Neurologische Zentralblatt, 30, 1090. Pettigrew, L. C . & Jankovic, J. (1985). Hemidystonia: a report of 22 patients and a review of the literature. Journal of Neurology, Neurosurgery, and Psychiatry, 48, 650-657. Riley, D. & Lang, A. E. (1988). Hemiaballism in multiple sclerosis. Movement Disorders, 3 , 88-94. Sarkari, N.B.S. (1968). Involuntary movements in multiple sclerosis. British Medical Journal, 2, 738740. Taff, I . , Sabato, U. C. & Lehrer, G . (1985). Choreoathetosis in multiple sclerosis. Clinical Neurology and Neurosurgery, 87, 41-43. Thiery, E. & De Reuck, J . (1974). Monoballism and multiple sclerosis. Acta Neurologica Belgica, 74, 24 1-249. G . I. AWERBUCH and R. SANDYK
Intern. J . Neuroscience, 1992, Vol. 62, p. 213-215 Reprints available directly from the publisher Photocopying permitted by license only
Letter to the Editor DYSTONIA AS THE PRESENTING MANIFESTATION OF MULTIPLE SCLEROSIS
Int J Neurosci Downloaded from informahealthcare.com by UB Giessen on 01/21/15 For personal use only.
(Received April 1 2 , 1991)
To the Editor: Involuntary movement disorders are an infrequent presentation of multiple sclerosis (MS). Despite the rather frequent presence of plaques in the area of the basal ganglia (e.g., thalamus, subthalamic nucleus) (McAlpine et al., 1972) and the occurrence of demyelinating lesions in the basal ganglia in 6.8% of patients with multiple sclerosis (MS) (Lumsden, 1970), only a few cases of resting tremor, hemi- or monoballism, athetosis, or choreoathetosis have been reported in MS patients during the course of their illness (Sarkari, 1968; Thiery and De Reuck, 1974; Bachman et al., 1976; Taff et al., 1985; Riley and Lang, 1988). Most of the movement disorders seen in MS such as intention tremor, myokymia, myoclonus, and tonic spasms have pathologic substrates outside the basal ganglia (Berger et al., 1984; Riley and Lang, 1988). We present a patient in whom segmental dystonia was the initial presentation of MS. A 38-year old right-handed woman was seen in the emergency department of Bay City Medical Center for the evaluation of speech disturbances and muscle spasms of the left arm and hand of two days duration. Initially, she noted that intermittently her hand assumed a “claw-like” deformity, but reported no functional loss of the left arm or hand. However, over the following 24 hours her symptoms appeared to worsen as she developed dysarthria and, in addition, her hand assumed a constant “claw-like’’ position. On the morning prior to examination, she experienced numbness of the left side of her body. Her past medical history was negative for cerebrovascular disease, seizures, syncope, and head trauma. In addition, there was no history of hypertension, diabetes, or thyroid disease and she denied alcohol or drug use. The patient had a history of a unervous breakdown,” but had not been on any type of psychotrophic drugs. Family history was negative for psychiatric or neurological disorders. On examination the patient was alert and cooperative. Intermittent dystonic facial grimaces were observed during her interview. Her mood was somewhat euphoric. She was afebrile with normal vital signs, and her neck was supple with no meningeal signs or carotid bruits. Her speech was markedly dysarthric and spastic, but there was no aphasia or anomia. She was oriented to person, place, and time. Examination of the cranial nerves showed normal optic nerves with no visual field defects. Her extraocular movements were full and there was no diplopia or blepharospasm. Facial sensation was decreased on the left. Tongue movements were clumsy and she was unable to move the tongue past the midline. Motor examination showed mild weakness in the left upper extremity, mostly in grip strength. Her left hand was fixed in a continuous “claw-like” deformity with more prominent flexion Correspondence to: Gavin I. Awerbuch, M.D. 200 S. We n o n a Avenue, Suite 298, B a y City, Michigan
48706, U.S.A. 273
Int J Neurosci Downloaded from informahealthcare.com by UB Giessen on 01/21/15 For personal use only.
274
G. I. AWERBUCH AND R. SANDYK
posturing of the ring and little fingers. The patient was unable to extend the fingers at the MP joints past about 30" to 40" of flexion and she experienced pain with movement in the distal interphalangeal joint of the left hand. There was mild drift with left arm extension. Muscle tone was increased in the left arm and leg. Sensory examination revealed loss of pinprick, vibration, and propioception over the entire left side of the body. There was no ataxia or dysmetria within the limitations of the spasms. Reflexes were symmetrical and plantar responses were flexor bilaterally. The patient's signs and symptoms of an acute onset progressive dystonia of the left arm and hand, dysarthria, and spasticity, raised the diagnostic possibility of acute cerebrovascular disease in the basal ganglia or brainstem. Laboratory studies including routine serum chemistries, a complete blood count, VDRL, thyroid function tests, liver enzymes (GPT, GOT, LDH), antinuclear antibody, EEG, visual evoked responses, somatosensory evoked responses in the upper and lower extremities, and auditory brainstem evoked responses were negative or normal, respectively. Serum IgG was 1575 mg/dl (normal: 540-1480 mg/dl) and oligoclonal (IgG) bands were absent. Serum albumin was low (3.1 g/dl) (normal: 3.5-5.5 g/dl). Lumbar cerebrospinal fluid (CSF) analysis revealed absence of oligoclonal bands, normal IgG level (2.0 mg/dl), an IgG index of 0.4 (normal 0-.7), an IgG/albumin ratio of .22 (normal: 0-.25), and albumin concentration of 9 mg/dl (normal: 1148 mg/dl). Myelin basic protein level was normal (< .5 ng/ml) (normal: < 4.0 ng/ ml). A noncontrast CT scan of the brain was normal. A TZweighted MRI scan revealed numerous high-intensity lesions typical of plaques of demyelination scattered throughout the intracerebral white matter, most prominent in the corona radiata and periventricular white matter. Several lesions were noted also in the basal ganglia region with predominance on the right side. These lesions were characteristic of and compatible with the diagnosis of MS. The patient was placed on baclofen (15 rng/day) and her dystonic movements gradually resolved over the following two weeks. Early symptoms and signs of MS usually include weakness and/or numbness in one or more limbs. In about 25% of patients, the initial manifestation of MS is an episode of retrobulbar neuritis. Not infrequently the disease may present with ataxia and nystagmus, with or without weakness and spasticity of the limbs (Adams and Victor, 1985). Dystonia, an abnormal involuntary movement defined by the presence of sustained posturing as well as tonic and clonic spasms of different parts of the body (Oppenheimer, 191l ) , is a distinctly uncommon feature of MS. Most patients with dystonia have primary torsion dystonia, which is either sporadic or hereditary (Fahn, 1984). The primary dystonias, by and large, are slowly progressive disorders that can plateau anywhere in the course of the illness. Characteristically, they begin insidiously and almost always with action dystonia. Only with progression of time do dystonic postures develop (Fahn and Jankovic, 1984). In contrast, most secondary dystonias frequently present with dystonia at rest, have an obvious sudden beginning, and tend to have a course that stabilizes and does not progress (Fahn and Jankovic, 1984). Secondary dystonia, which may be either focal when only a single body part is involved, or segmental when more than one body part is involved (Fahn, 1984), may result from structural brain lesions (i.e., cerebrovascular disease, head trauma, encephalitis), metabolic diseases (e.g., Wilson's disease), toxic exposure (e.g. , carbon monoxide), or degenerative neurological disorder (e.g., progressive supranuclear palsy) (Fahn and Jankovic, 1984; Pettigrew and Jankovic, 1985). Dystonic postures of the hands and feet are frequently seen in pa-
Int J Neurosci Downloaded from informahealthcare.com by UB Giessen on 01/21/15 For personal use only.
LETTER TO THE EDITOR
275
tients with advanced stages of Parkinson’s disease and in Parkinsonian patients receiving dopaminergic therapy (Jankovic, 1982; Fahn and Jankovic, 1984). Sparing of the corticospinal tract with disruption of the pathways between the striatum, pallidum, and thalamus is probably essential for the development of segmental dystonia (Dooling and Adams, 1975). In the series reported by Pettigrew and Jankovic (1985), all but two of the 22 cases of segmental dystonia had CT scan evidence of focal lesions in the striatum with no apparent evidence of corticospinal involvement in the internal capsule. This patient had multiple periventricular and basal ganglia lesions, the latter accounting for the development of segmental dystonia. However, unlike previous reports in which involuntary movements in MS occurred during the course of the disease, this case is remarkable in that dystonia was the presenting features of the disease. In view of the fact that in 6.8% of MS patients demyelinating lesions have been demonstrated in the basal ganglia (Lumsden, 1970), we suggest that MS be considered in the differential diagnosis of young adults presenting with an acute-onset focal or segmental dystonia.
REFERENCES Adams, R. D. & Victor, M. (1985). Principles of Neurology. New York: McGraw-Hill Book Company, pp. 699-717. Bachman, D. S . , Lao-Velez, C. & Estanol, B. (1976). Dystonia and choreoathetosis in multiple sclerosis. Archives of Neurology, 33, 590. Berger, J . R., Sheremata, W. A. & Melamed, E. (1984). Paroxysmal dystonia as the initial manifestation of multiple sclerosis. Archives of Neurology, 4 1 , 747-750. Dooling, E. C. & Adams, R. D. (1975). The pathological anatomy of posthemiplegic athetosis. Brain, 98, 29-48. Fahn, S . (1984). The varied clinical expression of dystonia. Neurologic Clinics, 2, 541-554. Fahn, S . & Jankovic, J. (1984). Practical management of dystonia. Neurologic Clinics, 2, 555-569. Jankovic, I. (1982). Management of motor side effects of chronic levodopa therapy. Clinical Neuropharmacology, 5, 519-528. Lumsden, C. E. (1970). The neuropathology of multiple sclerosis. In P. J . Vinken & G. W. Bruyn (Eds.). Handbook of clinical neurology, vol. 9, Amsterdam: North Holland, pp. 217-309. McAlpine, D., Lumsden, C . E. & Acheson, E. D. (1972). Multiple sclerosis: a reappruisel. Baltimore: Williams & Wilkins Co., pp. 177-178. Oppenheimer, H. (191 1). “Uber eine eigenartige Krampfkrankheit des kindlichen und jugendlichen Alters (dysbasia lordotica progressiva, dystonia musculorum deformans). Neurologische Zentralblatt, 30, 1090. Pettigrew, L. C . & Jankovic, J. (1985). Hemidystonia: a report of 22 patients and a review of the literature. Journal of Neurology, Neurosurgery, and Psychiatry, 48, 650-657. Riley, D. & Lang, A. E. (1988). Hemiaballism in multiple sclerosis. Movement Disorders, 3 , 88-94. Sarkari, N.B.S. (1968). Involuntary movements in multiple sclerosis. British Medical Journal, 2, 738740. Taff, I . , Sabato, U. C. & Lehrer, G . (1985). Choreoathetosis in multiple sclerosis. Clinical Neurology and Neurosurgery, 87, 41-43. Thiery, E. & De Reuck, J . (1974). Monoballism and multiple sclerosis. Acta Neurologica Belgica, 74, 24 1-249. G . I. AWERBUCH and R. SANDYK
