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Anesthesiology, Marmara University Institute of Neurological Sciences, Istanbul, Turkey E-mail: [email protected]
References 1.
2. 3. 4.
Saura H, Beppu T, Matsuura H, Asahi S, Uesugi N, Sasaki M, et al. Intractable yawning associated with mature teratoma of the supramedial cerebellum. J Neurosurg 2014;121:387-9. Argiolas A, Melis MR. The neuropharmacology of yawning. Eur J Pharmacol 1998;343:1-16. Askenasy JJ, Askenasy N. Inhibition of muscle sympathetic nerve activity during yawning. Clin Auton Res 1996;6:237-9. Arai K, Kita K, Komiyama A, Hirayama K, Saeki N, Nagao K. Progressive dysautonomia in hemangioblastoma in the region of the fourth ventricle. No To Shinkei 1986;38:195-200. Access this article online
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Examination revealed bradykinesia, generalized dystonia, prominent orofacial-dyskinesias, rigidity in all four limbs (most marked in the left upper limb) and preserved muscle stretch reflexes. Bilateral ocular fundii were normal with no Kayser-Fleischer ring. Investigations revealed a normal hemogram, liver and kidney function tests, normal serum creatine kinase, and normal arterial blood gas, lactate, and blood ammonia. Magnetic resonance imaging of the brain was normal. Serum copper and ceruloplasmin along with 24-hour urinary copper were normal. Urinary gas chromatography-mass spectroscopy and plasma acylcarnitine profile were normal. There was no response to oral L-Dopa. The child showed some improvement on transient midazolam infusion, trihexyphenidyl, oral diazepam, and physical rehabilitation. In view of rapid-onset dystonia with cranio-caudal gradient of progression, preserved cognition and normal magnetic resonance imaging of the brain, a possibility of rapid-onset dystonia Parkinsonism (Dystonia 12) were kept and genetic studies were planned.
DOI: 10.4103/0028-3886.152675 PMID: xxxxx
Subacute sclerosing panencephalitis masquerading as rapid-onset dystonia-Parkinsonism in a child
After 1-month of onset of symptoms, the child developed generalized myoclonic jerks with deterioration in cognition. The electroencephalography (EEG) showed generalized periodic epileptiform discharges [Figure 1]. Immunoglobulin G (IgG) antibody titer against measles was 104.0 in serum (normal
Anesthesiology, Marmara University Institute of Neurological Sciences, Istanbul, Turkey E-mail: [email protected]
References 1.
2. 3. 4.
Saura H, Beppu T, Matsuura H, Asahi S, Uesugi N, Sasaki M, et al. Intractable yawning associated with mature teratoma of the supramedial cerebellum. J Neurosurg 2014;121:387-9. Argiolas A, Melis MR. The neuropharmacology of yawning. Eur J Pharmacol 1998;343:1-16. Askenasy JJ, Askenasy N. Inhibition of muscle sympathetic nerve activity during yawning. Clin Auton Res 1996;6:237-9. Arai K, Kita K, Komiyama A, Hirayama K, Saeki N, Nagao K. Progressive dysautonomia in hemangioblastoma in the region of the fourth ventricle. No To Shinkei 1986;38:195-200. Access this article online
Website:
Quick Response Code
www.neurologyindia.com
Examination revealed bradykinesia, generalized dystonia, prominent orofacial-dyskinesias, rigidity in all four limbs (most marked in the left upper limb) and preserved muscle stretch reflexes. Bilateral ocular fundii were normal with no Kayser-Fleischer ring. Investigations revealed a normal hemogram, liver and kidney function tests, normal serum creatine kinase, and normal arterial blood gas, lactate, and blood ammonia. Magnetic resonance imaging of the brain was normal. Serum copper and ceruloplasmin along with 24-hour urinary copper were normal. Urinary gas chromatography-mass spectroscopy and plasma acylcarnitine profile were normal. There was no response to oral L-Dopa. The child showed some improvement on transient midazolam infusion, trihexyphenidyl, oral diazepam, and physical rehabilitation. In view of rapid-onset dystonia with cranio-caudal gradient of progression, preserved cognition and normal magnetic resonance imaging of the brain, a possibility of rapid-onset dystonia Parkinsonism (Dystonia 12) were kept and genetic studies were planned.
DOI: 10.4103/0028-3886.152675 PMID: xxxxx
Subacute sclerosing panencephalitis masquerading as rapid-onset dystonia-Parkinsonism in a child
After 1-month of onset of symptoms, the child developed generalized myoclonic jerks with deterioration in cognition. The electroencephalography (EEG) showed generalized periodic epileptiform discharges [Figure 1]. Immunoglobulin G (IgG) antibody titer against measles was 104.0 in serum (normal
