Neurological Vignette Received: December 20, 2010 Accepted: March 8, 2011 Published online: May 13, 2011
Eur Neurol 2011;65:307–308 DOI: 10.1159/000327305
Decreased Contralateral Putamen [123I]FP-CIT SPECT Uptake in Hyperglycemic Hemichorea-Hemiballismus Vincenzo Belcastro a , Laura Pierguidi a , Nicola Tambasco c, Luigi Sironi a , Leonardo Sacco a , Angelo Corso b, Angelo Taborelli a , Marco Arnaboldi a
a
Unità Operativa di Neurologia, Dipartimento di Neuroscienze, e b Unità Operativa di Medicina Nucleare, Azienda Ospedaliera Sant’Anna, Como, e c Clinica Neurologica, Ospedale S. Maria della Misericordia, Perugia, Italia
Key Words Hemichorea-hemiballismus ⴢ Diabetes mellitus ⴢ [123I]FP-CIT SPECT ⴢ Movement disorders An 82-year-old man with no familial or personal history of neurological illness or drug abuse, being treated for hypertension, was admitted to the emergency department for sudden onset of hemichorea-hemiballismus (HCHB) movements on the left side. On admission, the neurological examination revealed no abnormal pyramidal or sensory signs. Cognitive impairment, personality, and behavioral changes were not found. Laboratory studies demonstrated high glucose values (590 mg/dl) and hemoglobin A1c concentration was 19%, whereas there was no evidence of ketosis. The patient was given intravenous insulin which normalized glucose values and, after 2 days, the patient’s HCHB move-
Discussion
HCHB is a rare presentation of severe non-ketotic hyperglycemia secondary to diabetes mellitus [1]. The pathophysiology of hyperglycemic HCHB has not been clearly established, however, biochemical and neuroimaging findings support hyperviscosity as the most probable mechanism [2, 3]. This condition usually disappears after the blood-glucose abnormality has been corrected but, as in our patient too, a persistent HCHB syndrome has been described despite the normalization of glucose concentrations [1]. Moreover, in uncommon cases, chorea triggered by a hyperglycemic state could be persistent despite the normalization of metabolic stress [4]. The neuropathological nature of the characteristic MRI alterations is still controversial. Proton MRI spectroscopy and diffu-
2 Color version available online
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ments resolved. However, 20 days after the acute episode of hyperglycemia a left HCHB occurred again. Routine laboratory investigations demonstrated normal glucose values (95 mg/dl). Magnetic resonance imaging (MRI) of the brain showed a strongly T1-weighted hyperintensity in the right lenticular nucleus, mainly in the putamen (fig. 1). [123I]FP-CIT SPECT showed a strictly unilateral reduced uptake in the region of the right putamen (fig. 2) with a right putamen/cortex ratio of 1.53 (normal agedependent range: 1.7–4.8). Initial treatment with levodopa up to 300 mg daily (for 1 month) followed by tetrabenazine (up to 150 mg daily for 1 month) and levetiracetam (up to 2,000 mg daily for 1 month) was inefficient, whereas at the present follow-up (i.e. 8 months after onset of HCHB movements) treatment with haloperidol up to 6 mg/day had only a modest effect.
Fig. 1. T1-weighted image showing a
strongly hyperintense signal in the right putamen. Fig. 2. [123I]FP-CIT SPECT showing reduced uptake within the right putamen.
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sion-weighted MRI studies in HCHB suggest that both a hyperviscosity syndrome, possibly caused by hyperglycemia, and concomitant cytotoxic edema could be the cause of the MRI changes [2, 3]. Similarly to all reported cases in the literature [1–3], the MRI of our case evidenced a high signal on T1-weighted images in the putamen contralateral to the HCHB movements. The consistent involvement of the putamen suggests that the neuronal population of the putamen (i.e. GABAergic medium spiny neurons) has a crucial role in the pathogenesis of HCHB secondary to severe hyperglycemia [2]. This interpretation is not surprising considering that, among neuronal subtypes, striatal medium spiny neurons are highly vulnerable to energy depletion. The hypothesis of a reversible metabolic impairment may explain those cases where transient MRI and clinical alterations occurred. Conversely, irreversible damage to the putamen could also explain the persistent HCHB syndrome [1]. Interestingly, in our patient, [123I]FP-CIT SPECT showed a strictly unilateral reduced uptake in the right putamen corresponding to the left side with abnormal involuntary movements. To the best of our knowledge, this is the first report describing reduced presynaptic dopaminergic function predominant in the putamen in a patient with HCHB syndrome. In a recent work, in monozygotic twins with chorea-acanthocytosis, a reduction in DAT SPECT with -CIT was found [5]. In that paper, the most important DAT reduction was found contralateral to the most affected side by hyperkinetic movements and changes in the same region were also found by diffusion tensor imaging and FDG-PET [5], confirming that the more pronounced clinical presentation could be the expression of more important putaminal changes [2]. Although SPECT findings do not demonstrate a relation between HCHC and hyperviscosity, we argue that our finding of reduced DAT uptake in the region of the putamen could be due to neuronal loss or to internalization of dopamine re-uptake sites in
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the presynaptic dopamine terminals or to other factors [2, 3]. Further longitudinal studies in patients with hyperglycemic HCHB are needed to confirm the interest of DAT-SPECT in this rare condition. Disclosure Statement
The authors have no conflicts of interest to declare. References 1 Postuma RB, Lang AE: Hemiballism: revisiting a classic disorder. Lancet Neurol 2003;2:661–668. 2 Kandiah N, Tan K, Lim CC, Venketasubramanian N: Hyperglycemic choreoathetosis: role of the putamen in pathogenesis. Mov Disord 2009;24:915–919. 3 Chu K, Kang DW, Kim DE, Park SH, Roh JK: Diffusion-weighted and gradient echo magnetic resonance findings of hemichorea-hemiballismus associated with diabetic hyperglycemia: a hyperviscosity syndrome? Arch Neurol 2002;59:448–452. 4 Ahlskog JE, Nishino H, Evidente VG, Tulloch JW, Forbes GS, Caviness JN, Gwinn-Hardy KA: Persistent chorea triggered by hyperglycemic crisis in diabetics. Mov Disord 2001;16:890–898. 5 Müller-Vahl KR, Berding G, Emrich HM, Peschel T: Chorea-acanthocytosis in monozygotic twins: clinical findings and neuropathological changes as detected by diffusion tensor imaging, FDG-PET and 123I-CIT-SPECT. J Neurol 2007;254:1081–1088.
Vincenzo Belcastro, MD Unità Operativa di Neurologia, Dipartimento di Neuroscienze Azienda Ospedaliera Sant’Anna via Ravona, IT–22100 Como (Italy) Tel. +39 031 585 9946, E-Mail vincenzobelcastro @ libero.it
Belcastro/Pierguidi/Tambasco/Sironi/ Sacco/Corso/Taborelli/Arnaboldi
