- Email: [email protected]
Sequential observation of movement disorders and brain images in the case of central pontine myelinolysis and extrapontine myelinolysis
ARTICLE IN PRESS
Parkinsonism and Related Disorders 12 (2006) 462–464 www.elsevier.com/locate/parkreldis
Case report
Sequential observation of movement disorders and brain images in the case of central pontine myelinolysis and extrapontine myelinolysis Jung Im Seok, Jinyoung Youn, Eun Joo Chung, Won Yong Lee Movement Disorder Division, Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Received 24 October 2005; received in revised form 2 February 2006; accepted 3 February 2006
Abstract Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are well recognized syndromes related to rapid correction of hyponatremia and have been reported to show a variety of movement disorders. However, sequential observation of movement disorders as well as brain images has seldom been reported. We report a case of CPM and EPM presenting with various sequential changes in movement disorders including delayed choreic movement over 11 months; we present sequential brain magnetic resonance images showing increased T1 and decreased fat-suppression T1 signal intensity. We suggest that delayed low signal intensity, in the fat-suppression T1-weighted images, is a result of the destruction of myelin and by products. Damage to the myelin may cause various movement disorders in a delayed manner. r 2006 Elsevier Ltd. All rights reserved. Keywords: Extrapontine myelinolysis; Movement disorders; MRI
1. Introduction
2. Case report
Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are distinctive clinical syndromes with characteristic magnetic resonance features and demyelination is frequently related to rapid correction of electrolyte disturbance [1,2]. However, the association of delayed high signal intensity in T1weighted magnetic resonance imaging (MRI) and evolving movement manifestations have not been well described [3]. We describe a case of CPM and EPM presenting with a variety of sequential movement disorders and brain MRI findings presenting increased T1 and decreased fat-suppression T1 signal intensity on followup MRI.
A 43-year-old chronic alcoholic patient presented with involuntary movements in the neck and right arm. Eleven months prior to presentation, he developed dysarthria, generalized weakness and confusion after treatment for delirium tremens and rapid correction of hyponatremia. At that time, there was postural tremor in both limbs combined with irregular flexion movements of the trunk. Several days after treatment, the tremor and myoclonus resolved. However, dystonia with abnormal extensor posture in both arms developed. Brain MRI showed high signal intensities in the pons and the basal ganglia on T2-weighted images suggesting the characteristic findings of CPM and EPM (Fig. 1A–C). Five months later, he developed choreic movement of the neck and right arm causing severe functional impairment. Risk factors for stroke such as hypertension, diabetes mellitus, heart disease, smoking and hyperlipidemia were not present. The patient had slightly impaired cognitive function. Cranial nerve examination revealed a slow saccade with
Corresponding author. Tel.: +82 2 3410 3593; fax: +82 2 3410 0052. E-mail address: [email protected] (W.Y. Lee).
1353-8020/$ - see front matter r 2006 Elsevier Ltd. All rights reserved. doi:10.1016/j.parkreldis.2006.02.002
ARTICLE IN PRESS J. Im Seok et al. / Parkinsonism and Related Disorders 12 (2006) 462–464
463
Fig. 1. Sequential brain magnetic resonance imaging (MRI) scans and brain computed tomography (CT) and fat-suppression MRI scans. Initial axial T2-weighted brain MR images revealed characteristic high signal intensities in the pons (A) and the basal ganglia (B). T1-weighted images do not show any signal abnormality in the basal ganglia (C). Follow-up axial brain MRI revealed that the pontine lesion was reduced in size and the basal ganglia lesion showed normal signal intensity on T2-weighted images (D and E). New hyperintense lesions in the basal ganglia appeared on T1weighted images (F). Brain CT does not show any abnormality (G). Fat-suppression MRI shows decreased signal in the basal ganglia compared with T1-weighted images (H).
gaze evoked nytagmus and severe spastic dysarthria. Increased reflexes and spasticity was observed in all four extremities. Choreic movement was detected on the face, neck and right arm interfering with every motion. Dystonia on both hands and truncal ataxia was also observed. Laboratory studies investigating the causes of chorea were performed: a complete blood cell count with differential, general biochemistry, liver enzymes, thyroid hormone with thyroid autoantibodies, tumor marker, serum ceruloplasmin level, serum vitamin B12 and folic acid levels were all normal. Serological tests for hepatitis, VDRL and human immunodeficiency virus were negative. Evaluations for vasculitis including antinuclear antibodies, anti-dsDNA antibodies, anti-neutrophil cytoplasmic antibody (ANCA), circulating lupus anticoagulants, anti-cardiolipin antibodies, elevated sedimentation rate (ESR), C-reactive protein (CRP), anti-Ro and anti-La antibodies were normal. Genetic abnormalities such as Huntington’s disease and dentatorubral pallidoluysian atrophy were not detected. Cerebrospinal fluid study was normal. On esophagogastroduodenoscopy (EGD) and abdominal ultrasonography (USG), there was no evidence of liver cirrhosis, splenomegaly or esophageal varices.
Several months after the development of the choreic movement, follow-up brain MRI showed persistent T2 high signal intensity in the pons, but T2 high signal intensities in the basal ganglia disappeared. Rather, T1weighted images showed high signal intensities in the basal ganglia (Fig. 1D–F). Brain computed tomography (CT) did not reveal significant abnormality in the basal ganglia (Fig. 1G). Fat-suppression T1-weighted MR images showed decreased signal in the basal ganglia, which was not detected in T1-weighted images (Fig. 1(H).
3. Discussion While there have been some reports of parkinsonism and/or dystonia associated with EPM and CPM [4], focal and delayed onset choreic movement as observed in our patient has rarely been reported [3]. Because this manifestation is so unusual, we performed careful investigations to exclude other causes of the chorea, and all results were negative. We strongly suspected hepatocerebral degeneration as a cause of the patient’s involuntary movement because he was a chronic alcoholic and the findings of brain
ARTICLE IN PRESS 464
J. Im Seok et al. / Parkinsonism and Related Disorders 12 (2006) 462–464
MRI showing T1 high signals in the basal ganglia were compatible with hepatocerebral degeneration. However, hepatocerebral degeneration was ruled out because blood liver enzymes, serum ammonia and other hematologic tests were normal, and there was no evidence of a porto-systemic shunt such as splenomegaly and esophageal varices [5]. The possibility that sequential movement disorders may arise in a delayed manner in EPM due to ineffective or faulty reorganization in the basal ganglia has been considered in an earlier report [3]. However, the mechanism by which this may occur remains unclear. Only three reports have described delayed T1 high signal intensities in the basal ganglia of patients with EPM [6–8]. One report proposed that this change might reflect subacute microhemorrhagic lesions caused by vascular endothelial injury associated with EPM because the T1 high signal intensities were observed in the subacute stage [8]. This explanation is inappropriate for application to other cases where MRI was performed during the chronic stage of the disorder. Additional explanations for the T1 high signal in the basal ganglia include transient calcification or fat deposition [7]. However, the precise mechanisms of delayed T1 high signal intensities have not been determined. In our case, high signal intensities of the basal ganglia in T1-weighted images decreased in the fat-suppression T1-weighted MR images. Absence of calcification was confirmed by brain CT. We suggest that the high signal intensity in the T1-weighted images may be due to lipid accumulation in the basal ganglia. This phenomenon may result from the destruction of myelin, and the
regional release of myelin by-products, which are principally composed of cholesterol, cerebroside and to a lesser degree lecithin and sphingolipid [9]. In conclusion, we propose that delayed high T1 signal intensity and low fat-suppression T1 signal intensity in the basal ganglia may be a marker for the regional release of myelin by-products and may cause sequential movement disorders in a delayed manner.
References [1] Laureno R, Karp BI. Myelinolysis after correction of hyponatremia. Ann Intern Med 1997;126:57–62. [2] Wright DG, Laureno R, Victor M. Pontine and extrapontine myelinolysis. Brain 1979;102:361–85. [3] Seah ABH, Chan LL, Wong MC, Tan EK. Evolving spectrum of movement disorders in extrapontine and central pontine myelinolysis. Parkinsonism Relat Disord 2002;9:117–9. [4] Seiser A, Schwarz S, Aichinger-Steiner M, Funk G, Schnider P, Brainin M. Parkinsonism and dystonia in central pontine and extrapontine myelinolysis. J Neurol Neurosurg Psychiatry 1998; 65:119–21. [5] Jog MS, Lang AE. Chronic acquired hepatocerebral degeneration: case reports and new insights. Mov Disord 1995;10:714–22. [6] Choe WJ, Cho BK, Kim IO, Shin HY, Wang KC. Extrapontine myelinolysis caused by electrolyte imbalance during the management of suprasellar germ cell tumors. Report of two cases. Childs Nerv Syst 1998;14:155–8. [7] Ho VB, Fitz CR, Yoder CC, Ceyer CA. Resolving MR features in osmotic myelinolysis (general pontine and extrapontine myelinolysis). AJNR 1993;14:163–7. [8] Waragai M, Satoh T. Serial MRI of extrapontine myelinolysis of the basal ganglia: a case report. J Neurol Sci 1998;161:173–5. [9] Koenig SH. Cholesterol of myelin in the determinant of gray–white contrast in MRI of brain. Magn Reson Med 1991;20:285–91.
Parkinsonism and Related Disorders 12 (2006) 462–464 www.elsevier.com/locate/parkreldis
Case report
Sequential observation of movement disorders and brain images in the case of central pontine myelinolysis and extrapontine myelinolysis Jung Im Seok, Jinyoung Youn, Eun Joo Chung, Won Yong Lee Movement Disorder Division, Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Received 24 October 2005; received in revised form 2 February 2006; accepted 3 February 2006
Abstract Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are well recognized syndromes related to rapid correction of hyponatremia and have been reported to show a variety of movement disorders. However, sequential observation of movement disorders as well as brain images has seldom been reported. We report a case of CPM and EPM presenting with various sequential changes in movement disorders including delayed choreic movement over 11 months; we present sequential brain magnetic resonance images showing increased T1 and decreased fat-suppression T1 signal intensity. We suggest that delayed low signal intensity, in the fat-suppression T1-weighted images, is a result of the destruction of myelin and by products. Damage to the myelin may cause various movement disorders in a delayed manner. r 2006 Elsevier Ltd. All rights reserved. Keywords: Extrapontine myelinolysis; Movement disorders; MRI
1. Introduction
2. Case report
Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are distinctive clinical syndromes with characteristic magnetic resonance features and demyelination is frequently related to rapid correction of electrolyte disturbance [1,2]. However, the association of delayed high signal intensity in T1weighted magnetic resonance imaging (MRI) and evolving movement manifestations have not been well described [3]. We describe a case of CPM and EPM presenting with a variety of sequential movement disorders and brain MRI findings presenting increased T1 and decreased fat-suppression T1 signal intensity on followup MRI.
A 43-year-old chronic alcoholic patient presented with involuntary movements in the neck and right arm. Eleven months prior to presentation, he developed dysarthria, generalized weakness and confusion after treatment for delirium tremens and rapid correction of hyponatremia. At that time, there was postural tremor in both limbs combined with irregular flexion movements of the trunk. Several days after treatment, the tremor and myoclonus resolved. However, dystonia with abnormal extensor posture in both arms developed. Brain MRI showed high signal intensities in the pons and the basal ganglia on T2-weighted images suggesting the characteristic findings of CPM and EPM (Fig. 1A–C). Five months later, he developed choreic movement of the neck and right arm causing severe functional impairment. Risk factors for stroke such as hypertension, diabetes mellitus, heart disease, smoking and hyperlipidemia were not present. The patient had slightly impaired cognitive function. Cranial nerve examination revealed a slow saccade with
Corresponding author. Tel.: +82 2 3410 3593; fax: +82 2 3410 0052. E-mail address: [email protected] (W.Y. Lee).
1353-8020/$ - see front matter r 2006 Elsevier Ltd. All rights reserved. doi:10.1016/j.parkreldis.2006.02.002
ARTICLE IN PRESS J. Im Seok et al. / Parkinsonism and Related Disorders 12 (2006) 462–464
463
Fig. 1. Sequential brain magnetic resonance imaging (MRI) scans and brain computed tomography (CT) and fat-suppression MRI scans. Initial axial T2-weighted brain MR images revealed characteristic high signal intensities in the pons (A) and the basal ganglia (B). T1-weighted images do not show any signal abnormality in the basal ganglia (C). Follow-up axial brain MRI revealed that the pontine lesion was reduced in size and the basal ganglia lesion showed normal signal intensity on T2-weighted images (D and E). New hyperintense lesions in the basal ganglia appeared on T1weighted images (F). Brain CT does not show any abnormality (G). Fat-suppression MRI shows decreased signal in the basal ganglia compared with T1-weighted images (H).
gaze evoked nytagmus and severe spastic dysarthria. Increased reflexes and spasticity was observed in all four extremities. Choreic movement was detected on the face, neck and right arm interfering with every motion. Dystonia on both hands and truncal ataxia was also observed. Laboratory studies investigating the causes of chorea were performed: a complete blood cell count with differential, general biochemistry, liver enzymes, thyroid hormone with thyroid autoantibodies, tumor marker, serum ceruloplasmin level, serum vitamin B12 and folic acid levels were all normal. Serological tests for hepatitis, VDRL and human immunodeficiency virus were negative. Evaluations for vasculitis including antinuclear antibodies, anti-dsDNA antibodies, anti-neutrophil cytoplasmic antibody (ANCA), circulating lupus anticoagulants, anti-cardiolipin antibodies, elevated sedimentation rate (ESR), C-reactive protein (CRP), anti-Ro and anti-La antibodies were normal. Genetic abnormalities such as Huntington’s disease and dentatorubral pallidoluysian atrophy were not detected. Cerebrospinal fluid study was normal. On esophagogastroduodenoscopy (EGD) and abdominal ultrasonography (USG), there was no evidence of liver cirrhosis, splenomegaly or esophageal varices.
Several months after the development of the choreic movement, follow-up brain MRI showed persistent T2 high signal intensity in the pons, but T2 high signal intensities in the basal ganglia disappeared. Rather, T1weighted images showed high signal intensities in the basal ganglia (Fig. 1D–F). Brain computed tomography (CT) did not reveal significant abnormality in the basal ganglia (Fig. 1G). Fat-suppression T1-weighted MR images showed decreased signal in the basal ganglia, which was not detected in T1-weighted images (Fig. 1(H).
3. Discussion While there have been some reports of parkinsonism and/or dystonia associated with EPM and CPM [4], focal and delayed onset choreic movement as observed in our patient has rarely been reported [3]. Because this manifestation is so unusual, we performed careful investigations to exclude other causes of the chorea, and all results were negative. We strongly suspected hepatocerebral degeneration as a cause of the patient’s involuntary movement because he was a chronic alcoholic and the findings of brain
ARTICLE IN PRESS 464
J. Im Seok et al. / Parkinsonism and Related Disorders 12 (2006) 462–464
MRI showing T1 high signals in the basal ganglia were compatible with hepatocerebral degeneration. However, hepatocerebral degeneration was ruled out because blood liver enzymes, serum ammonia and other hematologic tests were normal, and there was no evidence of a porto-systemic shunt such as splenomegaly and esophageal varices [5]. The possibility that sequential movement disorders may arise in a delayed manner in EPM due to ineffective or faulty reorganization in the basal ganglia has been considered in an earlier report [3]. However, the mechanism by which this may occur remains unclear. Only three reports have described delayed T1 high signal intensities in the basal ganglia of patients with EPM [6–8]. One report proposed that this change might reflect subacute microhemorrhagic lesions caused by vascular endothelial injury associated with EPM because the T1 high signal intensities were observed in the subacute stage [8]. This explanation is inappropriate for application to other cases where MRI was performed during the chronic stage of the disorder. Additional explanations for the T1 high signal in the basal ganglia include transient calcification or fat deposition [7]. However, the precise mechanisms of delayed T1 high signal intensities have not been determined. In our case, high signal intensities of the basal ganglia in T1-weighted images decreased in the fat-suppression T1-weighted MR images. Absence of calcification was confirmed by brain CT. We suggest that the high signal intensity in the T1-weighted images may be due to lipid accumulation in the basal ganglia. This phenomenon may result from the destruction of myelin, and the
regional release of myelin by-products, which are principally composed of cholesterol, cerebroside and to a lesser degree lecithin and sphingolipid [9]. In conclusion, we propose that delayed high T1 signal intensity and low fat-suppression T1 signal intensity in the basal ganglia may be a marker for the regional release of myelin by-products and may cause sequential movement disorders in a delayed manner.
References [1] Laureno R, Karp BI. Myelinolysis after correction of hyponatremia. Ann Intern Med 1997;126:57–62. [2] Wright DG, Laureno R, Victor M. Pontine and extrapontine myelinolysis. Brain 1979;102:361–85. [3] Seah ABH, Chan LL, Wong MC, Tan EK. Evolving spectrum of movement disorders in extrapontine and central pontine myelinolysis. Parkinsonism Relat Disord 2002;9:117–9. [4] Seiser A, Schwarz S, Aichinger-Steiner M, Funk G, Schnider P, Brainin M. Parkinsonism and dystonia in central pontine and extrapontine myelinolysis. J Neurol Neurosurg Psychiatry 1998; 65:119–21. [5] Jog MS, Lang AE. Chronic acquired hepatocerebral degeneration: case reports and new insights. Mov Disord 1995;10:714–22. [6] Choe WJ, Cho BK, Kim IO, Shin HY, Wang KC. Extrapontine myelinolysis caused by electrolyte imbalance during the management of suprasellar germ cell tumors. Report of two cases. Childs Nerv Syst 1998;14:155–8. [7] Ho VB, Fitz CR, Yoder CC, Ceyer CA. Resolving MR features in osmotic myelinolysis (general pontine and extrapontine myelinolysis). AJNR 1993;14:163–7. [8] Waragai M, Satoh T. Serial MRI of extrapontine myelinolysis of the basal ganglia: a case report. J Neurol Sci 1998;161:173–5. [9] Koenig SH. Cholesterol of myelin in the determinant of gray–white contrast in MRI of brain. Magn Reson Med 1991;20:285–91.