BACKGROUND: Quantitative susceptibility mapping (QSM) has been used to measure iron accumulation in the deep nuclei of patients with Parkinson's disease (PD). This study examined the relationship between non-motor symptoms (NMSs) and iron accumulation in the deep nuclei of patients with PD. METHODS: The QSM data were acquired from 3-Tesla magnetic resonance imaging (MRI) in 29 patients with early PD and 19 normal controls. The Korean version of the NMS scale (K-NMSS) was used for evaluation of NMSs in patients. The patients were divided into high NMS and low NMS groups. The region-of-interest analyses were performed in the following deep nuclei: red nucleus, substantia nigra pars compacta, substantia nigra pars reticulata, dentate nucleus, globus pallidus, putamen, and head of the caudate nucleus. RESULTS: Thirteen patients had high NMS scores (total K-NMSS score, mean = 32.1), and 16 had low NMS scores (10.6). The QSM values in the deep were not different among the patients with high NMS scores, low NMS scores, and controls. The QSM values were not correlated linearly with K-NMSS total score after adjusting the age at acquisition of brain MRI. CONCLUSION: The study demonstrated that the NMS burdens are not associated with iron accumulation in the deep nuclei of patients with PD. These results suggest that future neuroimaging studies on the pathology of NMSs in PD should use more specific and detailed clinical tools and recruit PD patients with severe NMSs.
Basal Ganglia ; Brain ; Caudate Nucleus ; Cerebellar Nuclei ; Globus Pallidus ; Head ; Humans ; Iron* ; Magnetic Resonance Imaging ; Neuroimaging ; Parkinson Disease* ; Pars Compacta ; Pars Reticulata ; Pathology ; Putamen ; Red Nucleus

OBJECTIVES: To present a case of childhood primary dystonia with good response to zolpidem.
INTRODUCTION: Dystonia is a syndrome of sustained muscle contractions causing twisting and repetitive movements or abnormal postures. It is not uncommon in children. Although there are several options to treat dystonia,its medical treatment is notoriously difficult and often unsuccessful. Zolpidem, an imidazopyridine agonist with a high affinity to benzodiazepine subtype receptor BZ1, is reported to improve basal ganglia disease including Parkinson'sdisease and various types of dystonia.
THE CASE: 11 year old female with torticollis starting the 7years old. At q10years old symptoms progressed with her back hyperexteded. Her cranial MRI was normal. She was started on Levodopa and carbidopa however no improvement of symptoms was noted. She was started on zolpidem and there was a marked improvement in speech, writing, swallowing, walking and posture observed.
DISCUSSION: Primary dystonia is defined as syndromes in which dystonia is the sole phenotypic manifestation. There is no cognitive decline and cranial MRI is normal. Management of childhood dystonia differs in certain respects from that of adult dystonia. Dopamine responsive dystonia in children is rare, but a trial of L-dopa should be performed on all patients rith childhood-onset dystonia. Dystonia is associated with hypoactivity of the globus pallidus interna, and widespread brain alterations in GABAA/benzodiazepine receptors. Zolpidem binding sites are abundant in the basal ganglia output structure the globus pallidus interna and substantia nigra pars reticulata. It is thought that by binding to these sites, zolpidem help restore Ie ganglionic output influence to the thalamus and motor cortex.
CONCLUSION: There are several options to treat dystonia,its medical treatment is notoriously difficult and often unsuccessful. Zolpidem an imidazopyridine agonist can be given in childhood dystonia unresponsive to dopamine.

Human ; Female ; Child ; Basal Ganglia ; Benzodiazepines ; Carbidopa ; Deglutition ; Dopamine ; Dystonic Disorders ; Globus Pallidus ; Levodopa ; Motor Cortex ; Parkinson Disease ; Pars Reticulata ; Dystonia