1. Purpose Movement disorders represent a subset of neurological disorders that involve an error within the highly complex central nervous system resulting in an end organ malfunction. The complexity, multiplicity, and symptomatic overlap of these disorders have made their diagnosis and treatment a challenge for neurologist. The relative recent introduction of high resolution MR and advancement in MR functional imaging has played an integral role in diagnosing and understanding the pathophysiology of many of these disorders. However, this topic remains a challenging and perplexing one for a lot of non-neuroradiologists; in particular to radiology residents and fellows. Throughout this educational exhibit we will introduce some of the most common movement disorders while presenting their pathognomonic radiological findings, pathophysiology, and review pertinent anatomy. Approach/Methods 1) We will present a pictorial review of pertinent normal anatomy of the brain stem, cerebellum, and medulla via interactive magnetic resonance imaging (MR) and cartoon diagrams. 2) A summary table will display the disorders, pathophysiology, CT imaging findings, MR imaging findings, extra modalities findings if available, and respective differential diagnosis. 3) Finally, real clinical/radiographic cases of different movement disorders will be used to interactively reinforce the educational exhibit highlights. Findings/Discussion Movement disorders diagnosis often is elusive secondary to rarity, clinical overlap, and extensive list of such diseases. Also, most share common clinical symptoms that might be hard to distinguish in the hands of an unexperienced clinician. Furthermore most share similar imagining and clinical findings including atrophy, signal abnormality, and metabolic hypofunction. Therefore solid knowledge of gross and functional anatomy is imperative to provide a helpful differential diagnosis. In this selective review we presented some of the more common movement disorders based on anatomical location with discussion of pathophysiology, pertinent clinical findings, and radiological findings. Also, we included a succinct differential diagnosis for each case touching on the more common disorders in each anatomical location. This presentation by no means contains all of the movement disorders nor is it a sufficient source on the topic. However, we do believe that it represents a good starting point for understanding these disorders as it touches on the ones that more likely are to be encountered by residents and general radiologists Summary/Conclusion Movement disorders encompass a wide variety of complex disorders involving the central nervous system that result in debilitating long term symptoms for patients. Imaging plays a central role in diagnosis and sometimes prognosis of these disorders. Through understanding of the central nervous system involved regions normal anatomy and pathophysiology help the radiologist reach reliable and prompt diagnosis. DiseasePathophysiologyMiscellaneousNECT findingsMR findingsAdditional imaging FindingsDifferential Diagnosis Parkinson’s Disease Degeneration of dopaminergic neurons in substantia niagra para compacta Age most significant risk factor Most common clinical staging system is Braak typically shows non- specific mid brain volume loss Mostly used for validation of stimulating electrodes position 3 telsa delinate anatomy better T1 mid brain volume loss with butterfly configuration T2 indistinctness/loss of the hyperintense substantia niagra MRS normal or increase lactate/NAA ratio PET/SPECT depending on radiotracer evaluate dopamine neurons or receptors exhibiting decrease uptake Dementia of lewy body with differentiation made on clinical ground Multiple system atrophy Multiple System Atrophy (MSA) (3 subtypes) Depend on subtype Striatonigral degeneration with Parkinson like symptoms (MSA-P) Olivopontocerebellar atrophy (MSA-C) Shy-Drager Syndrome (MSA-A) with autonomic dysregulation Considered a subtype of Parkinson plus syndromes MSA-P has clinical symptoms similar to Parkinson’s MSA-C cerebellar symptoms MSA-A autonomic dysregulation All subtypes can have cortical brain atrophy MSA-P atrophic putamen with flatten margins MSA-C cerebellar atrophy, most pronounced within hemispheres with pons flattening and atrophy MSA-A is a combination of the other two MSA-C T1 cerebellar hemispheres atrophy, pons atrophy, small concave middle cerebellar peduncles, and enlarged fourth ventricles T2/FLAIR atrophy with a cruciform hyperintensity in pons “hot cross bun” DTI decreased volume of fibers in pons MSA-P T1 putamne atrophy T2 atrophy and hypointensity of putamen with lateral hyperintensity of 1.5 tesla T2* increase blooming secondary to iron deposition MRS MAS-C: decrease pontine and cerebellar NAA/CR and Col/Cr PET FDG MAS-P: decrease uptake in putamen Cerebelloolivary atrophy Friedreich ataxia Parkinson’s Parkinson’s plus syndrome Progressive supranuclear palsy (Steele-Richardson-Olszewski) Tau protein formation with deposit mainly in globus pallidus, subtantia niagra and brain stem Most common Parkinson plus syndrome Present 60-70s, no case reported under 40 Two subtypes Richardson subtype: Present with lurching gate, dystonia, early ocular symptoms. Late but definitve clinical feature is vertical supranuclear gaze palsy. Parkinsonian type (less common) present with Parkinson like symptoms midbrain volume loss with prominent interpeduncular and ambient cisterns. Mild ventriculomegaly T1 sagittal midbrain atrophy with concave upper border (penguin sign) T2 similar finding on sagittal enlarged third ventricle and prominent perimesencephalic cisterns Volumetric midbrain less than 70mm 3 and midbrain to pons ratio less than 0.15 DTI wide spread white matter abnormalities Nuclear medicine PET FDG decrease glucose metabolism in midbrain Decrease nerve terminals on dopamine transporter radioligands Tauopathies Alzheimer Parkinson MSA-P Hypertrophic Olivary degeneration Secondary degeneration of dentate-rubro-olivary pathway “Guillain-Mollaret Triangle” The olivary becomes hypertrophic rather than hypotrophic Cerebellar symptoms Can be ipsilateral or contralateral depending on pathway desruption normal olivary, but can see primary insult T1 typically normal or mild enlargement T2/FLAIR: hyperintensity with enlargement, eventually my becme hypotrophic but hyperintensity typically persist T1C+: typically do not enhance PET show increase metabolism SPECT demonstrate hyperperfusion Demyelinating disease Perforating artery infarct Rarely metronidazole neurotoxicity Creutzfeldt-Jakob Disease Prion disease secondary to proteinaceous infectious particles called prions Multiple subtypes some affect cognitive ability while others affect cerebellum. Most common type movement disorder include myoclonic jerks and akinetic mutism Cortical disease, rarely involve white matter Early normal Late sulci and ventricular prominence T1 normal T2/FLAIR: hyperintensities in basal ganglia, thalami, cerebral cortex with classic pulinar sign and posteromedial thalamus “hockey stick sign” DWI hyperintnese in striatum, cortex, and thalami Alzheimer Frontotemporal lobar degeneration Huntington Disease Loss of GABAergic neurons in basal ganglia Autosomal dominant Involuntary spasm and/or jerking and later chorea Atrophy of caudate nucleus with lateral ventricle anterior horn dilatation. Also putamen and globus pallidus Generalized atrophy with some suggestion of frontal lobes predilection T1 similar to NECT with increase intracaudal distance (>20mm, normal <10 mm). T2 hyperintensity in caudate head secondary to gliosis MRS Increase lactate in occipital cortex in symptomatic patients Decrease NAA/Cr with increase Cho/Cr suggestive of gliosis PET: decrease metabolism in caudate nucleus SPECT: hypoperfusion in basal ganglia Leigh disease Wilson Disease Carbon monoxide poisoning Fragile X–associated tremor/ataxia syndrome FMR1 gene premutation Recently discovered in 2001 Affect men above 50 years May affect up to 1 of 3,000 Intentional tremors and ataxia Small/atrophic cerebellum T1 symmetrical cerebellar white matter signal decrease T2 hyperintense ceberllar white matter Cerebellar atrophy Corpous Collousm thinning Olivoponto- cerebellar atrophy Subtypes of autosomal dominant cerebellar ataxia MCA-P Wilson’s Disease Friedrich Ataxiaexpansion of an unstable GAA trinucleotide repeat located in chromosome 9q Autosomal recessive most common inherited progressive ataxia Typically diffuse cerebellar atrophy (vermis and hemispheres) with preserved pons (debatable). Early normal Late cerebellar vermis and medulla atrophy (debatable) Superior cerebellar peduncle atrophy (contain most of white matter bundles) Superior spinal cord involvement No signal abnormality on T2/FLAIR DTI: white matter bindle atrophy autosomal dominant cerebellar ataxia early onset cerebellar ataxia with retained ref lexes Summary Table of Movement Disorders Parkinson’s Disease Normal axial T2 MR. Note normal hyperintense paras compacta (yellow arrow) situated between paras reticulata (blue semi circle) and red nucleus (orange circle) Axial T2 MR in patient with Parkinson, note loss of the hyper- intense band of paras compacta (yellow arrow) between the two almost kissing hypo-intensities (paras reticulata and red nucleus). Huntington’s Disease Demographics: 74 year old male Clinical presentation: Parkinsonian symptoms MR: classical loss of paras compacta Clinical Course: Currently followed by the movement disorder clinic Normal axial T2 MR. Note normal sized caudate (light red arrow) and Putamen (yellow arrow) Huntington’s Disease: Note atrophic caudate and Putamen (light red and yellow arrows respectively). Both structures are also hypointense. Also present is widened intra- caudate distance (blue double arrowhead). Normal Coronal T2 MR. Normal Caudate (arrow) and intra-caudate space. Huntington Disease: Coronal T2 MR exhibiting atrophic caudate and increase intra-caudate space (double arrowhead). Demographic: 48 year old male Clinical presentation: Chorea MR: Caudate atrophy with increase intracaudate distance and anterior horns dilation Clinical Course: Currently patient is in the movement disorder clinic Multiple System Atrophy (MSA-C) Normal Sagittal T1 MR demonstrating normal pons (orange arrow) and cerebellum (blue arrows) MSA-C: Sagittal T1 MR demonstrating atrophic pons (orange arrow) and cerebellum (blue arrows). Normal Axial T2 MR through the lower pons. MSA-C. Axial MR through the lower pons demonstrate atrophy and classical "hot cross bun" (arrow). Note atrophic cerebellum. Demographics: 66 year old female. Clinical: cerebellar symptoms. MR: Pons and cerebellar atrophy with T2 pons “ hot cross bun sign”. Clinical Course: Currently followed by neurology. Progressive Supranuclear Palsy (PSP) Normal Sagittal T1 demonstrate normal midbrain ( blue arrow) and tectum (light orange arrow). PSP: Sagittal T1 demonstrate midbrain atrophy (blue arrow) with ("penguin" or "hummingbird" sign) and tectum atrophy (green arrow). Demographics: 55 year old male Clinical presentation: Parkinsonian symptoms MR: Midbrain and tectum atrophy with classical "penguin" or "hummingbird" sign Clinical Course: Currently patient is in the movement disorder clinic Creutzfeldt-Jakob Disease (CJD) CJD: Axil FLAIR demonstrate hyperintesity within the Basal ganglia (BG) and faintly within the thalamus (yellow arrows) CJD: Axial DWI demonstrating BG restricted diffusion (ADC map not shown). CJD: Axial FLAIR at the level of the superior frontal cortex demonstrating hyperintensity CJD: DWI at the same level demonstrate restricted diffusion (ADC map not shown). Demographic: 41 year old male. Clinical Course: Rapid dementia and myoclonus with elevated 14-3-3 protein in CSF. Patient expired shortly after being diagnosed. MR: Hyperintensity of the Basal Ganglia (BG) and cortex on FLAIR images with correlating restricted diffusion. Movement Disorders: Review of Radiological Findings and a Suggested Guideline for Diagnosis Rami Eldaya, MD,MBA ● Omar Eissa, MD ● Jax Pham, DO ● Jorge Lee-Diaz, MD ● Thomas Uribe, MD Disclosure: none University of Texas Medical Branch Disclosure: none University of Texas Medical Branch References Osborn AN. Osborn's Brain Imaging, pathology, and Anatomy; Amirsys; 2013 Pagani E, Ginestroni A, Della Nave R et-al. Assessment of brain white matter fiber bundle atrophy in patients with Friedreich ataxia. Radiology. 2010;255 (3): 882-9 Massey LA, Miranda MA, Zrinzo L et-al. High resolution MR anatomy of the subthalamic nucleus: imaging at 9.4 T with histological validation. Neuroimage. 2012 Feb 1;59(3):2035-44 Brunberg JA, Jacquemont S, Hagerman RJ et-al. Fragile X premutation carriers: characteristic MR imaging findings of adult male patients with progressive cerebellar and cognitive dysfunction. AJNR Am J Neuroradiol. 2002 Nov-Dec;23(10):1757-66 Mascalchi M, Vella A, Ceravolo R. 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