- Email: [email protected]
Rapidly deteriorating parkinsonism and dysautonomia in patient with central pontine and extrapontine myelinolysis
Clinical Neurology and Neurosurgery 113 (2011) 513–514
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
Rapidly deteriorating parkinsonism and dysautonomia in patient with central pontine and extrapontine myelinolysis Do-Young Kwon a , Woo-Keun Seo a , Moon Ho Park a,∗ , Young Sun Kang b , Seong-Beom Koh a , Dae Ryoung Cha b , Kun Woo Park a a b
Department of Neurology, Korea University College of Medicine, Republic of Korea Division of Nephrology, Department of Internal Medicine, Korea University College of Medicine, Republic of Korea
a r t i c l e
i n f o
Article history: Received 4 September 2009 Received in revised form 31 January 2011 Accepted 1 February 2011 Available online 26 February 2011 Keywords: Osmotic demyelination Hyponatremia Parkinsonism Dysautonomia
1. Introduction Extrapyramidal symptoms are rarely the predominant sequelae of osmotic myelinolysis because the pyramidal manifestations usually dominate [1,2]. Secondary parkinsonism in osmotic demyelination may develop late in the disease course, and usually responds well to conventional treatment. We report an unusual case of mixed parkinsonism and dysautonomia that showed a rapidly deteriorating course without any evidence of pyramidal tract signs in central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) after aggressive treatment of hyponatremia, which did not respond to levodopa treatment and led to a devastating outcome. Although CPM and EPM have the same pathophysiology, serial brain MRIs disclosed that the signal changes associated with CPM and EPM were not simultaneous and were not correlated with changes in neurological manifestations. 2. Case report A 51-year-old man was admitted to the hospital complaining of general weakness. The patient had a seven-day history of poor oral intake, but denied exposure to drugs. On admission, his neu-
∗ Corresponding author at: Department of Neurology, Korea University Ansan Hospital, 516 Gojan-1-dong, Danwon-gu, Ansan-City, Gyeonggi-do 425-707, Republic of Korea. Tel.: +82 31 412 5150; fax: +82 31 412 5154. E-mail addresses: [email protected], [email protected] (M.H. Park). 0303-8467/$ – see front matter © 2011 Elsevier B.V. All rights reserved. doi:10.1016/j.clineuro.2011.02.002
rologic examination was unremarkable, except for mild lethargy and disorientation. His initial blood chemistry was as follows: serum sodium concentration 100 mmol/L, chloride 62.9 mmol/L, and potassium 2.6 mmol/L. Diffusion-weighted imaging (DWI) of the brain was unremarkable (Fig. 1A-1 and A-2). We made an initial diagnosis of hyponatremia and treated the patient with hypertonic saline. After the infusion, his serum sodium increased to 124 mmol/L over 24 h, and he was alert and fully oriented by the next day. On the fifth day of hospitalization, although there was no weakness on neurologic examination, the patient complained of gait instability, slowness of movement with stiffness and tremor of all four limbs in both acting and resting states. His blood chemistry was unremarkable, but MRI revealed high signal intensity in the pons on fluid attenuated inversion recovery (FLAIR) sequences with indefinite slightly increased signals in the caudate and putamen (Fig. 1B-1 and B-2). The patient deteriorated rapidly and became hypophonic, agitated, and unable to walk due to severe postural instability without weakness of limbs. His reflexes were brisk, and his plantar responses were flexor. The patient exhibited only extrapyramidal symptoms, including bradykinesia and rigidity, which caused him to become bedridden. His mental status was alert, and he was able to communicate and answer questions appropriately using his eyelids and visual pursuit, although he could not speak due to pseudobulbar palsy. The patient’s parkinsonism progressed very rapidly on the Hoehn and Yahr scale for parkinsonism from 0 (no signs of disease) to 5 (bedridden unless assisted) within five days. He also demonstrated autonomic nervous system (ANS) dys-
514
D.-Y. Kwon et al. / Clinical Neurology and Neurosurgery 113 (2011) 513–514
Fig. 1. Diffusion-weighted MRI showing no signal abnormality on b-1000 (A-1) or ADC images (A-2). FLAIR sequences on the sixth day of hospitalization, one day after the onset of parkinsonism, revealed a signal change in the caudate and putamen but not the thalamus (B-1) and the pons (B-2). MRIs taken on the tenth day of hospitalization showed extended lesions to the thalamus (C-1), along with a more expanded lesion in the pons (C-2).
function with excessive sweating and fluctuating blood pressure. Treatments with levodopa, anticholinergics, and benzodiazepines were not effective. An MRI taken on the tenth day of hospitalization revealed a more extended area of pontine lesion, and broadened previous extrapontine lesions to the caudate, putamen, and thalamus (Fig. 1C-1 and C-2). The patient died of sudden cardiac arrest 10 days after admission while sleeping.
and presented with an unusual clinical course as evaluated through serial MRIs. It is important to recognize that the rapid correction of unbalanced electrolytes might cause devastating outcomes. Clinicians should keep in mind that the clinical manifestations of osmotic demyelination syndrome can be diverse, and that MRI findings may not correlate with neurological manifestations in acute states. Financial support
3. Discussion Extrapyramidal symptoms are rare in cases of osmotic demyelination syndrome, but if present usually show delayed onset and respond well to levodopa treatment [3,4]. However, the present case showed an unusually acute onset of parkinsonism with overlapping dysautonomia, and rapid deterioration without response to levodopa treatment. As demyelination is the principal pathologic change in osmotic myelinolysis, it is possible that extrapyramidal manifestations may present as complex patterns because they may initially be induced by demyelination of dopamine receptors in the striatum [1]; their delayed onset, progressive, and protean nature may be due to ineffective, random compensatory reorganization of neuronal structures after osmotic myelinolysis [2]. Radiological evaluations may not reflect the relevant clinical features in a timely fashion for this type of complex pathophysiology [5]. In the present case, acute parkinsonism was not fully elucidated until the last MRI revealed extrapontine lesions. In summary, we present a case of devastating osmotic demyelination syndrome that exhibited exclusive extrapyramidal features
None. Conflict of interest None. References [1] Nagamitsu S, Matsuishi T, Yamashita Y, Yamada S, Kato H. Extrapontine myelinolysis with parkinsonism after rapid correction of hyponatremia: high cerebrospinal fluid level of homovanillic acid and successful dopaminergic treatment. J Neural Transm 1999;106:949–53. [2] Seah AB, Chan LL, Wong MC, Tan EK. Evolving spectrum of movement disorders in extrapontine and central pontine myelinolysis. Parkinsonism Relat Disord 2002;9:117–9. [3] Koussa S, Nasnas R. Catatonia and Parkinsonism due to extrapontine myelinolysis following rapid correction of hyponatremia: a case report. J Neurol 2003;250:103–5. [4] Sullivan AA, Chervin RD, Albin RL. Parkinsonism after correction of hyponatremia with radiological central pontine myelinolysis and changes in the basal ganglia. J Clin Neurosci 2000;7:256–9. [5] Kumar SR, Mone AP, Gray LC, Troost BT. Central pontine myelinolysis: delayed changes on neuroimaging. J Neuroimaging 2000;10:169–72.
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
Rapidly deteriorating parkinsonism and dysautonomia in patient with central pontine and extrapontine myelinolysis Do-Young Kwon a , Woo-Keun Seo a , Moon Ho Park a,∗ , Young Sun Kang b , Seong-Beom Koh a , Dae Ryoung Cha b , Kun Woo Park a a b
Department of Neurology, Korea University College of Medicine, Republic of Korea Division of Nephrology, Department of Internal Medicine, Korea University College of Medicine, Republic of Korea
a r t i c l e
i n f o
Article history: Received 4 September 2009 Received in revised form 31 January 2011 Accepted 1 February 2011 Available online 26 February 2011 Keywords: Osmotic demyelination Hyponatremia Parkinsonism Dysautonomia
1. Introduction Extrapyramidal symptoms are rarely the predominant sequelae of osmotic myelinolysis because the pyramidal manifestations usually dominate [1,2]. Secondary parkinsonism in osmotic demyelination may develop late in the disease course, and usually responds well to conventional treatment. We report an unusual case of mixed parkinsonism and dysautonomia that showed a rapidly deteriorating course without any evidence of pyramidal tract signs in central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) after aggressive treatment of hyponatremia, which did not respond to levodopa treatment and led to a devastating outcome. Although CPM and EPM have the same pathophysiology, serial brain MRIs disclosed that the signal changes associated with CPM and EPM were not simultaneous and were not correlated with changes in neurological manifestations. 2. Case report A 51-year-old man was admitted to the hospital complaining of general weakness. The patient had a seven-day history of poor oral intake, but denied exposure to drugs. On admission, his neu-
∗ Corresponding author at: Department of Neurology, Korea University Ansan Hospital, 516 Gojan-1-dong, Danwon-gu, Ansan-City, Gyeonggi-do 425-707, Republic of Korea. Tel.: +82 31 412 5150; fax: +82 31 412 5154. E-mail addresses: [email protected], [email protected] (M.H. Park). 0303-8467/$ – see front matter © 2011 Elsevier B.V. All rights reserved. doi:10.1016/j.clineuro.2011.02.002
rologic examination was unremarkable, except for mild lethargy and disorientation. His initial blood chemistry was as follows: serum sodium concentration 100 mmol/L, chloride 62.9 mmol/L, and potassium 2.6 mmol/L. Diffusion-weighted imaging (DWI) of the brain was unremarkable (Fig. 1A-1 and A-2). We made an initial diagnosis of hyponatremia and treated the patient with hypertonic saline. After the infusion, his serum sodium increased to 124 mmol/L over 24 h, and he was alert and fully oriented by the next day. On the fifth day of hospitalization, although there was no weakness on neurologic examination, the patient complained of gait instability, slowness of movement with stiffness and tremor of all four limbs in both acting and resting states. His blood chemistry was unremarkable, but MRI revealed high signal intensity in the pons on fluid attenuated inversion recovery (FLAIR) sequences with indefinite slightly increased signals in the caudate and putamen (Fig. 1B-1 and B-2). The patient deteriorated rapidly and became hypophonic, agitated, and unable to walk due to severe postural instability without weakness of limbs. His reflexes were brisk, and his plantar responses were flexor. The patient exhibited only extrapyramidal symptoms, including bradykinesia and rigidity, which caused him to become bedridden. His mental status was alert, and he was able to communicate and answer questions appropriately using his eyelids and visual pursuit, although he could not speak due to pseudobulbar palsy. The patient’s parkinsonism progressed very rapidly on the Hoehn and Yahr scale for parkinsonism from 0 (no signs of disease) to 5 (bedridden unless assisted) within five days. He also demonstrated autonomic nervous system (ANS) dys-
514
D.-Y. Kwon et al. / Clinical Neurology and Neurosurgery 113 (2011) 513–514
Fig. 1. Diffusion-weighted MRI showing no signal abnormality on b-1000 (A-1) or ADC images (A-2). FLAIR sequences on the sixth day of hospitalization, one day after the onset of parkinsonism, revealed a signal change in the caudate and putamen but not the thalamus (B-1) and the pons (B-2). MRIs taken on the tenth day of hospitalization showed extended lesions to the thalamus (C-1), along with a more expanded lesion in the pons (C-2).
function with excessive sweating and fluctuating blood pressure. Treatments with levodopa, anticholinergics, and benzodiazepines were not effective. An MRI taken on the tenth day of hospitalization revealed a more extended area of pontine lesion, and broadened previous extrapontine lesions to the caudate, putamen, and thalamus (Fig. 1C-1 and C-2). The patient died of sudden cardiac arrest 10 days after admission while sleeping.
and presented with an unusual clinical course as evaluated through serial MRIs. It is important to recognize that the rapid correction of unbalanced electrolytes might cause devastating outcomes. Clinicians should keep in mind that the clinical manifestations of osmotic demyelination syndrome can be diverse, and that MRI findings may not correlate with neurological manifestations in acute states. Financial support
3. Discussion Extrapyramidal symptoms are rare in cases of osmotic demyelination syndrome, but if present usually show delayed onset and respond well to levodopa treatment [3,4]. However, the present case showed an unusually acute onset of parkinsonism with overlapping dysautonomia, and rapid deterioration without response to levodopa treatment. As demyelination is the principal pathologic change in osmotic myelinolysis, it is possible that extrapyramidal manifestations may present as complex patterns because they may initially be induced by demyelination of dopamine receptors in the striatum [1]; their delayed onset, progressive, and protean nature may be due to ineffective, random compensatory reorganization of neuronal structures after osmotic myelinolysis [2]. Radiological evaluations may not reflect the relevant clinical features in a timely fashion for this type of complex pathophysiology [5]. In the present case, acute parkinsonism was not fully elucidated until the last MRI revealed extrapontine lesions. In summary, we present a case of devastating osmotic demyelination syndrome that exhibited exclusive extrapyramidal features
None. Conflict of interest None. References [1] Nagamitsu S, Matsuishi T, Yamashita Y, Yamada S, Kato H. Extrapontine myelinolysis with parkinsonism after rapid correction of hyponatremia: high cerebrospinal fluid level of homovanillic acid and successful dopaminergic treatment. J Neural Transm 1999;106:949–53. [2] Seah AB, Chan LL, Wong MC, Tan EK. Evolving spectrum of movement disorders in extrapontine and central pontine myelinolysis. Parkinsonism Relat Disord 2002;9:117–9. [3] Koussa S, Nasnas R. Catatonia and Parkinsonism due to extrapontine myelinolysis following rapid correction of hyponatremia: a case report. J Neurol 2003;250:103–5. [4] Sullivan AA, Chervin RD, Albin RL. Parkinsonism after correction of hyponatremia with radiological central pontine myelinolysis and changes in the basal ganglia. J Clin Neurosci 2000;7:256–9. [5] Kumar SR, Mone AP, Gray LC, Troost BT. Central pontine myelinolysis: delayed changes on neuroimaging. J Neuroimaging 2000;10:169–72.