This article appeared in a journal published by Elsevier. The attached copy is furnished to the author for internal non-commercial research and education use, including for instruction at the authors institution and sharing with colleagues. Other uses, including reproduction and distribution, or selling or licensing copies, or posting to personal, institutional or third party websites are prohibited. In most cases authors are permitted to post their version of the article (e.g. in Word or Tex form) to their personal website or institutional repository. Authors requiring further information regarding Elsevier’s archiving and manuscript policies are encouraged to visit: http://www.elsevier.com/copyright
Author's personal copy
Journal of Clinical Neuroscience 19 (2012) 1165–1167
Contents lists available at SciVerse ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case Reports
Delayed chorea after recovery from a symmetric parkinsonian syndrome due to striatal myelinolysis Aaron de Souza a,⇑, Paresh K. Desai b a b
Department of Neurology, Goa Medical College, Bambolim, Goa 403 202, India Department of Radiology, Goa Medical College, Bambolim, Goa, India
a r t i c l e
i n f o
Article history: Received 3 August 2011 Accepted 14 November 2011
Keywords: Chorea Extrapontine myelinolysis Parkinsonism Striatum
a b s t r a c t Extrapontine myelinolysis (EPM) is rare and usually results from osmotic stress due to inappropriate correction of electrolyte disturbances. Parkinsonism is a relatively common manifestation of EPM, seen in 60% of patients. Dopaminergic therapy leads to good recovery in many patients. Delayed extrapyramidal manifestations, including hyperkinetic movement disorders such as chorea or dystonia, are postulated to result from aberrant neuronal regeneration or denervation supersensitivity following the initial osmotic insult. We present a patient with a symmetric parkinsonian syndrome due to EPM involving the striatum who made a good recovery with levodopa therapy, only to develop chorea five months later. The possible mechanisms underlying the genesis of these movement disorders in EPM is discussed. Ó 2012 Elsevier Ltd. All rights reserved.
1. Case Report Osmotic demyelination syndrome (ODS) is increasingly recognised to involve extrapontine locations in addition to the betterknown central pontine myelinolysis. Extrapontine myelinolysis (EPM) is now well recognised as the manifestation of ODS occurring in the brain in locations other than the pons, and may present with extrapyramidal symptoms due to involvement of the basal ganglia: specifically, parkinsonism is seen in up to 56% of patients.1 After recovery from the initial illness, a subset of patients develop delayed movement disorders such as dystonia, choreoathetosis, tremor, rigidity or bradykinesia, up to 150 days from the onset of ODS.2 We present a patient who recovered well from symmetric parkinsonism due to ODS involving the striatum and later manifested with delayed chorea. A 62-year-old woman was admitted to hospital for drowsiness and confusion following vomiting and loose stools. She was moderately dehydrated at admission and serum sodium was low (101.4 mmol/L), with a serum potassium level of 1.89 mmol/L. This was thought to be due to acute gastroenteritis, on a background of hydrochlorothiazide use and salt restriction advised previously for hypertension. She was treated with intravenous 0.9% saline, oral potassium chloride, fluids by mouth and a high-salt diet. Parenteral hypertonic saline solutions were not given. Serum potassium levels remained low till the seventh day of admission, but serum sodium rose steadily to reach near-normal values by the fourth day (Fig. 1). Sodium levels did not increase by more than 11 mmol/L per day at any time. Her confusion improved by 48 hours after admission. She remained well for the next three days, but then found herself un⇑ Corresponding author. Tel.: +91 832 249 5085; +91 942 015 9422. E-mail address:
[email protected] (A. de Souza).
able to move her limbs freely or get out of bed. She was conscious and alert but with hypophonic dysarthric speech and markedly limited output. Severe rigidity and bradykinesia of all limbs with brisk stretch reflexes and flexor plantars were seen. Postural balance was very poor. She had a bilateral grasp reflex. No seizures, oculomotor abnormalities, dystonia, tremors or other involuntary movements, sensory disturbances or incoordination were seen. A 1.5-Tesla MRI of the brain (Siemens AG, Erlangen, Germany) conducted four days after onset of neurologic symptoms showed symmetric T2-weighted and fluid attenuated inversion recovery hyperintensities in both caudate nuclei and putamina with restricted diffusion. The pons, thalamus, globus pallidus and cerebellum were normal (Fig. 2). A diagnosis of isolated EPM following correction of dyselectrolytaemia was made. She was given supportive care and physiotherapy, and started on levodopa-carbidopa and amantadine. Doses of levodopa-carbidopa were gradually escalated to a maximum of six tablets of 250/25 mg daily, with amantadine at 200 mg/day. She made a rapid recovery, and had only mild postural dysfunction and minimal axial and appendicular rigidity at discharge 26 days later. Amantadine and levodopa were successively tapered and withdrawn without incident over two months, and she thereafter took only telmisartan for hypertension. Five months after onset of her illness, she presented with rapid asymmetric asynchronous flowing or jerky involuntary movements involving the proximal and distal limb muscles, tending to move unpredictably from one limb region to another (Supplementary video). These increased gradually during a period of 14 days, but responded well to sodium valproate (600 mg/day) and risperidone (1.5 mg/day). Thyroid function, serum calcium, serum sodium and serum caeruloplasmin were normal and antinuclear antibodies were absent.
Author's personal copy
1166
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1165–1167
Fig. 1. Graphs of rates of serum sodium and potassium correction in a patient with delayed chorea after recovery from a symmetric parkinsonian syndrome due to striatal myelinolysis. Upper and lower limits of normal values are indicated by dotted lines.
Fig. 2. Axial (A) fluid-attenuated inversion recovery MRI showing a normal-appearing pons; and (B) T2-weighted MRI showing bilateral symmetric hyperintensities involving the caudate nuclei and putamina. Globus pallidi and thalami are spared.
2. Discussion The pathogenesis of parkinsonism due to EPM is not completely elucidated yet, but is thought to result from a relative dopamine deficiency due to reduction of both the presynaptic striatal dopamine transporter and dopamine receptors on myelinated fibres in the striatum. Increased levels of dopamine metabolites such as homovanillic acid in the cerebrospinal fluid of patients with acute parkinsonism due to EPM suggest that the disease causes a reduction of dopamine receptors on myelinated fibres in the striatum. This leads to decreased function of the striatonigral negative feedback loop and of inhibitory autoreceptors, thereby enhancing dopamine and serotonin secretion.3–5 Due to the combination of hypokinetic and hyperkinetic movement disorders seen in EPM it has been postulated that striatal lesions result in variable disruption of both direct and indirect pathways. Both types of movement disorders can be seen due to alterations in the rate or pattern of activity in thalamic, pallidal, subthalamic or cortical neurons.6 Dopaminergic therapy, principally levodopa but also pramipexole, ameliorates the presynaptic dopaminergic deficit by activating the remaining dopamine receptors and leads to clinical improvement similar to that seen in idiopathic Parkinson’s disease.3–5
Delayed extrapyramidal manifestations, including hyperkinetic movement disorders, may follow weeks to months later, due to ineffective neuronal reorganisation or repair, and may be progressive and refractory to treatment.6 These movement disorders may be analogous to delayed dystonia seen with static encephalopathy, due to neuronal reorganisation with new synaptic connections, delayed death of affected neurons, denervation supersensitivity, transsynaptic degeneration of neural structures or ongoing myelinolysis.2 In this patient chorea may be attributed to denervation supesensitivity or aberrant neuronal reorganisation after recovery from a severe akinetic-rigid state. In contrast to earlier reports,6 she improved well with therapy.
Appendix A. Supplementary material Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2011.11.024.
References 1. Kallakatta RN, Radhakrishnan A, Fayaz RK, et al. Clinical and functional outcome and factors predicting prognosis in osmotic demyelination syndrome (central
Author's personal copy
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1167–1169 pontine and/or extrapontine myelinolysis) in 25 patients. J Neurol Neurosurg Psychiatry 2011;82:326–31. 2. Maraganore DM, Folger WN, Swanson JW, et al. Movement disorders as sequelae of central pontine myelinolysis: report of three cases. Mov Disord 1992;7:142–8. 3. Post B, van Gool WA, Tijssen MA. Transient parkinsonism in isolated extrapontine myelinolysis. J Neurol Sci 2009;30:325–8. 4. Kim JS, Lee KS, Han SR, et al. Decreased striatal dopamine transporter binding in a patient with extrapontine myelinolysis. Mov Disord 2003;18:342–5.
1167
5. Nagamitsu S, Matsuishi T, Yamashita Y, et al. Extrapontine myelinolysis with parkinsonism after rapid correction of hyponatremia: high cerebrospinal fluid levels of homovanillic acid and successful dopaminergic treatment. J Neural Transm 1999;106:949–53. 6. Seah AB, Chan LL, Wong MC, et al. Evolving spectrum of movement disorders in extrapontine and central pontine myelinolysis. Parkinsonism Relat Disord 2002;9:117–9.
doi:10.1016/j.jocn.2011.11.024
Progressive multifocal leukoencephalopathy (PML) mimicking high-grade glioma on delayed F-18 FDG PET imaging Koen Mertens a, Marjan Acou b, Caroline Van den Broecke c, Roel Nuyts d, Dirk Van Roost d, Eric Achten b, Ingeborg Goethals a,⇑ a
Department of Nuclear Medicine, Polikliniek 7, Ghent University Hospital, De Pintelaan 185, Gent 9000, Belgium Department of Radiology, Ghent University Hospital, Gent, Belgium Department of Pathology, Ghent University Hospital, Gent, Belgium d Department of Neurosurgery, Ghent University Hospital, Gent, Belgium b c
a r t i c l e
i n f o
Article history: Received 8 August 2011 Accepted 13 August 2011
Keywords: Delayed imaging F-18 fluorodeoxyglucose Positron emission tomography Progressive multifocal leukoencephalopathy
a b s t r a c t The purpose of our study was to determine the increase in F-18 fluorodeoxyglucose (FDG) uptake in a patient with progressive multifocal leukoencephalopathy (PML) between early and late scan times using positron emission tomography (PET) imaging with F-18 FDG at conventional (60 minutes [min] after injection, PET60) and delayed (300 min after injection, PET300) intervals. PET60 and PET300 imaging was performed on a pathologically proven PML lesion. The PML lesion in the posterior fossa exhibited an increase in F-18 FDG uptake of 52% between early and late times, which was in the range of that in high-grade gliomas. Thus, dual-time-point PET with F-18 FDG may not be able to differentiate between infectious and malignant brain lesions. Ó 2012 Elsevier Ltd. All rights reserved.
1. Introduction
2. Case report
Progressive multifocal leukoencephalopathy (PML) is a progressive, demyelinating disease caused by the reactivation of the John Cunningham (JC) virus in immunocompromised patients.1 Clinical features are non-specific and the diagnosis is often considered for the first time based on MRI findings. White matter involvement usually appears on MRI as asymmetric multifocal lesions without contrast enhancement. However, conventional MRI is not specific enough for the differential diagnosis of single lesions. Complementary to conventional MRI, proton magnetic resonance (MR) spectroscopy (MRS) and positron emission tomography (PET) imaging can be used for metabolic characterisation. Definite diagnosis is based on cerebrospinal fluid polymerase chain reaction for JC virus DNA and a brain biopsy.2 Here, we report a patient with PML presenting with the acute onset of focal neurological signs and a large non-enhancing lesion in the posterior fossa on MRI. The lesion was further characterised by dual-time-point F-18 fluorodeoxyglucose (FDG) PET imaging. To our knowledge this is the first reported case of PML studied by F-18 FDG PET at delayed intervals.
The patient is a 52-year-old man with a history of heart transplantation. In November 2008, he developed diplopia and a left cerebellar syndrome. Conventional MRI at the time of admission revealed hyperintense fluid-attenuated inversion recovery and hypointense T1-weighted signal changes without contrast enhancement in the left cerebellar hemisphere, extending in to the middle cerebellar peduncle. On diffusion-weighted imaging (DWI), the lesion was hyperintense, with high signal on the corresponding apparent diffusion coefficient (ADC) map images, indicating T2 shine through. The absence of diffusion restriction ruled out recent ischemia. Because the patient was immunocompromised, the diagnosis of PML was considered. MRS was performed to differentiate the lesion from a low-grade glioma. On MRS, however, there was no significant increase in choline (Cho), which is present in glial brain tumors. The metabolic profile showed a decrease in N-acetylaspartate (NAA) and high levels of lactate (Fig. 1). The large lesion provided an opportunity to determine the metabolic profile further, and we characterised the lesion with dualtime-point F-18 FDG PET studies. On the PET60 images, the most prominent finding was a high uptake in the pons; however, on conventional MRI or DWI, no signal abnormalities were observed in the brain stem. Analysis of the PET60 and PET300 images further indicated that the activity in the pons increased with time. The increase between early and late scan times was 38%. In addition, there was
⇑ Corresponding author. Tel.: +32 9 332 30 28; fax: +32 9 332 38 07. E-mail address:
[email protected] (I. Goethals).
View publication stats
