Movement Disorders Val. 6 , No. 4, 1991, pp. 379-383 0 1991 Movement Disorder Society
Communications ogy of blepharospasm, probably by a reduction of their direct control on the brainstem nuclei.
Blepharospasm Associated with Pseudohypoparathyroidismand Bilateral Basal Ganglia Calcifications
0. Blin G. Masson G. Serratrice Clinique des maladies du systeme nerveux et de l’appareil locomoteur CHU Timone 13385 Marseille, France
To the Editor: Blepharospasm is a focal dystonia (I), most of the time idiopathic, that can be connected with many neurological, ophthalmological, or metabolic conditions, and sometimes with a focal lesion of the central nervous system (2,3). Its pathophysiology remains poorly understood. Marsden (4) attributed it to a basal ganglia dysfunction, whereas Jankovic (3) considered it a disorder.of rostralbrainstem-diencephalon. We report here a blepharospasm case associated with pseudohypoparathyroidism; computed tomography (CT) scan showed bilateral calcifications of basal ganglia. In 1980, a 60-year-old man presented with a hypocalcemia (1.65 mM) and a high serum phosphorus (1.39 mM). Serum parathyroid hormone was normal. Results of an osseous biopsy confirmed the pseudohypoparathyroidism. Since 1985 the patient had suffered from an irregular closing of the eyelids, leading to important functional discomfort. This symptom appeared at any moment of the day, particularly during or after an effort. Treatment with pyridostigmine (360 mg/day) brought only a temporary result, as did right and left eyelid surgery, performed respectively in September 1986 and January 1987. In May 1987 the patient pointed out that the blepharospasm particularly started with voluntary blinking, bright light, effort, and car driving. There was an uninhibited glabellar reflex. No other dystonic symptom or involuntary movement was associated. At this time, laboratory exams showed normal serum calcium levels (2.16 mM). Radiography showed a dystrophic syndrome concerning the hands and feet, with calcifications of the tendinous inserts and soft tissues. CT scan showed voluminous bilateral calcifications involving the basal ganglia. The brainstem was normal. Blink reflexes studied on the two trigeminal nerves (VI) were normal, as was the brainstem evoked potential. Treatment with high doses of trihexyphenidyl (16 &day) improved his condition. The present report calls attention to an association between blepharospasm and pseudohypoparathyroidism, which probably resulted in calcification of the central nervous system. Association between blepharospasm and basal ganglia calcifications was noted before (5). In our patient, whereas blink reflexes were normal, there was an uninhibited glabellar reflex with deficient supranuclear control on trigeminal reflex arc (6). Moreover, brainstem was normal and only basal ganglia were affected by calcifications. Basal ganglia have been involved in the control of brainstem reflexes (7) and in movement disorders in animals (S), as well as in cranial dystonia in humans (1,3,6). This report lends credence to the assumption of an involvement of the basal ganglia in the pathophysiol-
References 1 . Fahn S . Blepharospasm: a form of focal dystonia. Adv Neurol 1988;49:125-1 34. 2. Jankovic J , Ford J. Blepharospasm and orofacial-cervical dystonia: clinical and pharmacological findings in 100 patients. Ann Neurol 1983;13:402-41l . 3. Jankovic J, Patel SC. Blepharospasm associated with brainstem lesions. Neurology 1983;33:1237-1240. 4. Marsden CD. Blepharospasm-oromandibular dystonia syndrome (Brueguel’s syndrome). A variant of adult-onset torsion dystonia? J Neurol Neurosurg Psych 1976;39:12041209. 5. Herraiz J, Roquer J, Escudero D, Maso E. Meige’s syndrome and bilateral pallidal calcifications. J Neurol 1988; 235:384. 6 . Berardelli A, Rothwell JC, Day BL, Marsden CD. Pathophysiology of blepharospasm and oromandibular dystonia. Bruin 1985;108:593-608. 7. Schneider JS. Interactions between the basal ganglia, the pontine parabrachial region, and the trigeminal system in cat. Neuroscience 1986;19:411425. 8. Spooren WPJM, Groenewegen HJ, Cools AR. Subregions of the caudate nucleus and their in- and output channels in orofacial dyskinesia. Brain Res 1991;539:85-93.
Thalamic Demyelination and Paroxysmal Dystonia in Multide Sclerosis
m
To the Editor: We describe a patient with paroxysmal right hemidystonia as the initial manifestation of multiple sclerosis in whom the magnetic resonance scans showed a lesion involving the left thalamus. The phenomenon referred to as paroxysmal dystonia, tonic spasms, or tonic seizures is an uncommon but wellestablished feature of multiple sclerosis (MS). Although a brainstem or spinal origin has been postulated, the site of the demyelination remains unproved (1). Here we report a patient with MS and paroxysmal right hemidystonia as the initial symptom in whom brain magnetic resonance imaging (MRI) showed a lesion involving the left thalamus.
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Communications ogy of blepharospasm, probably by a reduction of their direct control on the brainstem nuclei.
Blepharospasm Associated with Pseudohypoparathyroidismand Bilateral Basal Ganglia Calcifications
0. Blin G. Masson G. Serratrice Clinique des maladies du systeme nerveux et de l’appareil locomoteur CHU Timone 13385 Marseille, France
To the Editor: Blepharospasm is a focal dystonia (I), most of the time idiopathic, that can be connected with many neurological, ophthalmological, or metabolic conditions, and sometimes with a focal lesion of the central nervous system (2,3). Its pathophysiology remains poorly understood. Marsden (4) attributed it to a basal ganglia dysfunction, whereas Jankovic (3) considered it a disorder.of rostralbrainstem-diencephalon. We report here a blepharospasm case associated with pseudohypoparathyroidism; computed tomography (CT) scan showed bilateral calcifications of basal ganglia. In 1980, a 60-year-old man presented with a hypocalcemia (1.65 mM) and a high serum phosphorus (1.39 mM). Serum parathyroid hormone was normal. Results of an osseous biopsy confirmed the pseudohypoparathyroidism. Since 1985 the patient had suffered from an irregular closing of the eyelids, leading to important functional discomfort. This symptom appeared at any moment of the day, particularly during or after an effort. Treatment with pyridostigmine (360 mg/day) brought only a temporary result, as did right and left eyelid surgery, performed respectively in September 1986 and January 1987. In May 1987 the patient pointed out that the blepharospasm particularly started with voluntary blinking, bright light, effort, and car driving. There was an uninhibited glabellar reflex. No other dystonic symptom or involuntary movement was associated. At this time, laboratory exams showed normal serum calcium levels (2.16 mM). Radiography showed a dystrophic syndrome concerning the hands and feet, with calcifications of the tendinous inserts and soft tissues. CT scan showed voluminous bilateral calcifications involving the basal ganglia. The brainstem was normal. Blink reflexes studied on the two trigeminal nerves (VI) were normal, as was the brainstem evoked potential. Treatment with high doses of trihexyphenidyl (16 &day) improved his condition. The present report calls attention to an association between blepharospasm and pseudohypoparathyroidism, which probably resulted in calcification of the central nervous system. Association between blepharospasm and basal ganglia calcifications was noted before (5). In our patient, whereas blink reflexes were normal, there was an uninhibited glabellar reflex with deficient supranuclear control on trigeminal reflex arc (6). Moreover, brainstem was normal and only basal ganglia were affected by calcifications. Basal ganglia have been involved in the control of brainstem reflexes (7) and in movement disorders in animals (S), as well as in cranial dystonia in humans (1,3,6). This report lends credence to the assumption of an involvement of the basal ganglia in the pathophysiol-
References 1 . Fahn S . Blepharospasm: a form of focal dystonia. Adv Neurol 1988;49:125-1 34. 2. Jankovic J , Ford J. Blepharospasm and orofacial-cervical dystonia: clinical and pharmacological findings in 100 patients. Ann Neurol 1983;13:402-41l . 3. Jankovic J, Patel SC. Blepharospasm associated with brainstem lesions. Neurology 1983;33:1237-1240. 4. Marsden CD. Blepharospasm-oromandibular dystonia syndrome (Brueguel’s syndrome). A variant of adult-onset torsion dystonia? J Neurol Neurosurg Psych 1976;39:12041209. 5. Herraiz J, Roquer J, Escudero D, Maso E. Meige’s syndrome and bilateral pallidal calcifications. J Neurol 1988; 235:384. 6 . Berardelli A, Rothwell JC, Day BL, Marsden CD. Pathophysiology of blepharospasm and oromandibular dystonia. Bruin 1985;108:593-608. 7. Schneider JS. Interactions between the basal ganglia, the pontine parabrachial region, and the trigeminal system in cat. Neuroscience 1986;19:411425. 8. Spooren WPJM, Groenewegen HJ, Cools AR. Subregions of the caudate nucleus and their in- and output channels in orofacial dyskinesia. Brain Res 1991;539:85-93.
Thalamic Demyelination and Paroxysmal Dystonia in Multide Sclerosis
m
To the Editor: We describe a patient with paroxysmal right hemidystonia as the initial manifestation of multiple sclerosis in whom the magnetic resonance scans showed a lesion involving the left thalamus. The phenomenon referred to as paroxysmal dystonia, tonic spasms, or tonic seizures is an uncommon but wellestablished feature of multiple sclerosis (MS). Although a brainstem or spinal origin has been postulated, the site of the demyelination remains unproved (1). Here we report a patient with MS and paroxysmal right hemidystonia as the initial symptom in whom brain magnetic resonance imaging (MRI) showed a lesion involving the left thalamus.
379
![[Paroxysmal dystonia and multiple sclerosis].](/assets/img/hold.png)
