Hyperintense Basal Ganglia Research Articles

Hemi-Chorea in a Patient with Ketotic hyperglycemia: An Unusual Presentation

Chorea has often been associated with lesions in the basal ganglia and in the sub thalamic nucleus. It is possible for a patient with chorea-ballismus to have hyperglycemia at the initial presentation. We hereby present a case of an 81-year-old female, who was on treatment for type 2 diabetes mellitus and presented to us with sub acute onset of abnormal movements of right side of the body. She had semi purposeful, rapid and jerky movements of right upper limb and lower limb along with abnormal tongue movements. Laboratory data showed very high blood glucose levels, urine ketones were positive and pH of arterial blood was normal. MRI brain showed hyperintensities in right basal ganglia. So, hyperglycemia induced hemichorea was considered as a possibility and she was treated with insulin. These abnormal movements decreased subsequently with treatment and patient is doing better in the follow-up visits. This presentation is extremely rare, as review of literature showed similar presentations in patients with non ketotic hyperglycemia but not reported so far in diabetic ketosis.

Hyperfamiliarity in Amnestic and Vascular Mild Cognitive Impairment.

Hyperfamiliarity is a phenomenon where new stimuli are perceived as familiar. Previous studies have demonstrated familiarity disorder in mild cognitive impairment (MCI), but mostly from the perspective of a neuropsychological approach, and the exact correlation of MCI aetiologies with the phenomenon remains uncertain. Based on current evidence suggesting a frontal-subcortical pathway contributing to familiarity processing, we hypothesize that individuals with a vascular aetiology of MCI will likely suffer more familiarity deficits. This study aims to examine the real-life hyperfamiliarity symptoms in amnestic versus vascular MCI. Informants of 11 amnestic and 9 vascular cognitive impairment patients were interviewed about the frequency of hyperfamiliarity symptoms in the previous month. MRI brain images of vascular cognitive impairment patients were analysed as well. Patients with vascular cognitive impairment with no dementia (VCIND) showed a significantly higher frequency of hyperfamiliarity for people but not places or objects. Within VCIND patients, overall basal ganglia hyperintensities, particularly in the putamen, were found to significantly correlate to hyperfamiliarity. Patients with VCIND suffer more real-life hyperfamiliarity during people recognition compared to patients with amnestic mild cognitive impairment (aMCI), despite a comparative global decline in cognitive. This is likely due to impaired memory retrieval and matching processes resulting from subcortical ischaemic lesions.

Novel mutation in mitochondrial DNA in 2 siblings with Leigh syndrome.

Leigh syndrome is clinically and genetically heterogeneous, associated with mutations in mitochondrial and nuclear genes.(1) Diagnostic criteria include progressive disorder with motor and intellectual delay/regression; signs and symptoms of brainstem and/or basal ganglia disease; raised lactate concentration in blood and/or CSF; and one or more of the following: (1) characteristic features on neuroimaging (bilateral symmetrical hyperintensities in brainstem, basal ganglia, dentate nuclei, and optic nerves on T2-weighted MRI); (2) typical neuropathologic changes; and (3) typical neuropathology in a similarly affected sibling.(2) We describe 2 African American siblings who have a mutation in the mitochondrial MT-TL2 gene and a clinical diagnosis of Leigh syndrome. The same mutation is also identified in their neurologically asymptomatic mother.

LGI1 antibody encephalopathy overlapping with sporadic Creutzfeldt-Jakob disease

Objective:To report a rare case of leucine-rich, glioma inactivated 1 (LGI1) antibody–mediated autoimmune encephalopathy clinically overlapping with pathologically confirmed sporadic Creutzfeldt-Jakob disease (CJD).Methods:The patient was investigated with repeated brain MRI, EEG, CSF examination, whole-body fluorodeoxy-glucose positron emission tomography, genetic analysis of the prion protein gene (PRNP), and extensive serologic screening for paraneoplastic and autoimmune encephalopathy markers. Written informed consent was obtained from the patient's next of kin for access to clinical files for research purposes and for publication.Results:The patient was a 77-year-old man who presented with faciobrachial dystonic seizures (FBDS) secondary to LGI1 antibody–mediated autoimmune encephalopathy, with suggestive MRI findings and a complete response to treatment with combinatorial immunosuppression. Stereotactic biopsy of a nonenhancing T1 hyperintense basal ganglia lesion during the initial FBDS phase, albeit following immunosuppression, did not disclose evidence of lymphocytic inflammation. Following full remission of the FBDS, the patient manifested a rapidly progressive dementia associated with gross motor decline confirmed to be CJD at autopsy (molecular subtype VV3), with no evidence of a pathogenic PRNP mutation.Conclusions:Our patient highlights that these rare diseases are not invariably mutually exclusive and underscores the benefits of comprehensive neuropathologic examination of the brain to achieve an accurate diagnosis, especially in complex cases when the clinical trajectory dramatically deviates and a concomitant disease may need to be conscientiously considered to best explain the new clinical course.

Clinicopathological Correlates in a PRNP P102L Mutation Carrier with Rapidly Progressing Parkinsonism-dystonia.

Parkinsonism-dystonia is rare in carriers of PRNP P102L mutation. Severity and distribution of prion protein (PrP) deposition may influence the clinical presentation. We present such clinic-pathological correlation in a 56-year-old male with a PRNP P102L mutation associated with a phenotype characterized by rapidly progressing parkinsonism-dystonia. The patient was studied clinically (videotaped exams, brain MRIs); molecular genetically (gene sequence analysis); and neuropathologically (histology, immunohistochemistry) during his 7-month disease course. The patient had parkinsonism, apraxia, aphasia, and dystonia, which progressed rapidly. Molecular genetic analysis revealed PRNP P102L mutation carrier status. Brain MRIs revealed progressive global volume loss and T2/FLAIR hyperintensity in neocortex and basal ganglia. Postmortem examination showed neuronal loss, gliosis, spongiform changes, and PrP deposition in the striatum. PrP immunohistochemistry revealed widespread severe PrP deposition in the thalamus and cerebellar cortex. Based on the neuropathological and molecular-genetic analysis, the rapidly progressing parkinsonism-dystonia correlated with nigrostriatal, thalamic, and cerebellar pathology.

Chorea as the Neurological Symptom of Delayed Encephalopathy After Carbon Monoxide Intoxication in a Child.

A 10-year-old boy presented to the emergency department with abnormal movements 8 days after he was rescued from a house fire. At the scene, he was comatose and was found to have soot in his airway. He had an elevated carboxyhemoglobin level (24.5%). He received mechanical ventilation with 100% oxygen for 40 hours. He showed rapid recovery in mental status and was discharged home. Eight days after the injury, he developed tremors, dystonia, and chorea involving his face and all extremities (Video). Brain magnetic resonance imaging showed T2 hyperintensity in the caudate, putamen, and globus pallidus bilaterally, and magnetic resonance spectroscopy showed lactate peaks in the basal ganglia (Figures 1A and ​andB).B). Trihexyphenidyl improved his dystonia and tremor within a month, but the chorea worsened. After he discontinued trihexyphenydyl, chorea gradually resolved. Eight months after the injury, he is still experiencing emotional, behavioral, and academic problems as well as intermittent urinary incontinence. Figure 1. A, T2-weighted axial sequence of brain magnetic resonance imaging (MRI) shows hyperintensities in the bilateral basal ganglia. The signal changes are greater involving the left caudate and putamen and greater involving the right globus pallidus. B, Brain ... Delayed encephalopathy after carbon monoxide (CO) intoxication is characterized by mental deterioration, gait disturbance, speech disorder, seizure, and incontinence preceded by an asymptomatic period of variable duration (from 2 to 40 days) following recovery from the acute stage.1 Additionally, destructive lesions of basal ganglia may result in movement disorders. Parkinsonism has been reported to occur in 9.5% of CO-poisoned patients.2 The incidence of chorea after CO poisoning is known to be rare. Its duration ranges from days to months, and it is usually alleviated through the use of neuroleptics and aggravated by anticholinergics.3

NMDA-receptor encephalitis: An unsual case of refractory status epilepticus

Objective: Case report of NMDA receptor encephalitis in a young man with early refractory status epilepticus and atypical radiological findings. Background: Anti-NMDA receptor encephalitis is an autoimmune disorder due to antibodies to the NR1-NR2B heterodimer of NMDA receptor. On imaging, it typically presents with T2 hyperintensities in mesio-temporal lobes, cerebral cortex and basal ganglia. We present a case with a dramatic clinical evolution and novel imaging findings. Design/Methods: Case report and review of imaging. Results: 29-year-old male presented with mood disturbance followed by partial-complex seizures, facial dyskinesia and choreo-athetotic movements. Initial MRI showed subtle T2-hyperintensities in mesio-temporal lobes. Diagnosis of NMDA-receptor encephalitis was confirmed after CSF antibody detection. Prior to diagnostic confirmation, he developed refractory status epilepticus, and concomitant signs of herniation. A repeated MRI showed increased T2-hyperintensities of thalami and mesencephalon, with cerebellar involvement and transtentorial/foraminal herniation. Restricted diffusion was documented in the cerebellar cortex/thalami/putamina and caudate. IV corticosteroids and hypertonic fluid reversed herniation, and halted the seizures. Conclusions: To our knoweldge, we report the first case report of uncal and tonsillar herniation in NMDA-r encephalitis secondary to atypical, predominant cerebellar involvement. This case highlights life-threatening manifestation that physicians might encounter, and a possible role for high dose IV corticosteroids as an adjunct treatment for brain edema and seizures.

Hemichorea–hemiballism: a case report

A 55-year-old woman with type 2 diabetes mellitus presented with abnormal left-sided movements of her arm and leg. Her initial CT scan of head showed a hyperintensity in the right...

Germinoma in the bilateral basal ganglia presented with cognitive deterioration

Here, we report a case of pediatric germinoma located in the bilateral basal ganglia, which presented with severe cognitive deteriorations. A 15-year-old boy presented with decreased school performance and mild cognitive disturbances. Magnetic resonance images (MRI) of the brain revealed T2 hyperintensity in the bilateral basal ganglia. The patient was initially observed by a local hospital and had screening for metabolic diseases or inflammatory diseases. Lesions with similar characteristics were also found in the pituitary stalk and infundibulum, and these lesions were enhanced with gadolinium (Gad). MR spectroscopy suggested that these should be neoplastic lesions other than metabolic or inflammatory diseases. Biopsy was performed with ventriculoscope, which proved all lesions of infundibulum, pineal, and basal ganglia were pathologically germinoma. The lesions responded well to the chemotherapy and radiation, and his cognitive function improved significantly. A case of germinoma in the bilateral basal ganglia which significantly affect cognitive functions is reported. Differential diagnoses of cognitive symptoms are various, but germinoma could be considered as a possible pathology for it. Early MRI and tumor marker exams are recommended, unless organic brain diseases are completely denied. MR spectroscopy and biopsy with ventriculoscope are useful for diagnosis.

Imaging Evaluation of Pediatric Sensorineural Hearing Loss in Potential Candidates for Cochlear Implantation.

Computerized tomography (CT) and magnetic resonance (MR) are complementary in the imaging of the labyrinth, the internal auditory canal and the brain in children with sensorineural hearing loss who are being evaluated for cochlear implantation. An accurate anatomical description of the inner ear is essential in the preoperative work up. Computerized tomography visualizes the bony structures, whereas MR can discern soft-tissue components including intra labyrinthine fluid, cerebrospinal fluid (CSF), nerves, and vessels within the IAC. This prospective study was conducted in the Department of Otorhinolaryngology, Head & Neck Surgery, Government Medical College, Srinagar. 40 children in the age group of 1-16years with unidentified causes of bilateral SNHL were analysed radiologically over the period of 2years from Dec 2011 to Jan 2014. Each patient underwent MRI and high resolution CT scanning of temporal bone in axial and coronal planes. Out of the 40 patients 22 were males (55%) and 18 were females (45%). 30 patients (72.5%)in our study had normal radiological scans. Five patients (12.5%) had B/L large vestibular aqueduct and two patients (5%) had internal auditory canal stenosis with cochlear nerve hypoplasia on CT and MR imaging. Cochlear dysplasia was present in two patients (5%) and semicircular canal dysplasia was present in one patient (2.5%) as an isolated finding on HRCT. In addition isolated cochlear nerve hypoplasia was present in one patient (2.5%). Hyperintense basal ganglia lesion suggestive of kernicterus was present in one patient (2.5%) and hyperintense posterior parietal and occipital white matter lesions suggestive of congenital CMV infection was present in one patient (2.5%) on MR imaging. Arachnoid cysts of middle cranial fossa was an incidental finding present in one patient. Radiological abnormalities of the inner ear are not uncommon. Computerized tomography and MRI are important modalities to analyze the inner ear in children with unexplained SNHL. MRI with an extremely small field of view should be used to study possible abnormalities of the vestibulocochlear nerves.

Role of Plasma Amino Acids and Urinary Organic Acids in Diagnosis of Mitochondrial Diseases in Children

BackgroundDiagnostic difficulty in mitochondrial diseases (MD) results not only from the wide spectrum of symptoms and signs but also from the absence of a reliable screening or diagnostic biomarker. AimTo investigate the likelihood of MD in patients with symptoms and signs impressive of MD through quantitative measurement of plasma amino acids, and urinary organic acids. MethodsTwenty patients with symptoms and signs suggestive of MD were further evaluated by quantitative plasma amino acids and urinary organic acids assay and neuroimaging. ResultsPlasma amino acid results revealed elevation of alanine in 11, glycine in five, and proline in two patients. Abnormal urinary organic acid analysis was present in six patients; increased urinary lactate (20%), dicarboxylicaciduria (15%), and urinary ketone bodies (10%). Upon enrollment our patients scored as possible MD according to the MD scoring system. At the end of the study, five patients still scored as possible MD, eight patients as probable MD, and seven patients as definite MD. All patients with definite MD had elevated serum lactate. In three patients, elevated urinary lactate was the only abnormality. Alanine was elevated in all patients with definite MD, whereas proline was elevated in only one. Magnetic resonance imaging of the brain showed atrophic changes in one patient and bilateral basal ganglia hyperintensity in another. ConclusionUrinary organic acids and quantitative plasma amino acids can help in the diagnosis of MD, especially when the economic burden and absence of specialized centers limits the diagnosis.

Posterior reversible encephalopathy syndrome after depletive lumbar puncture: a case report

IntroductionPosterior reversible encephalopathy syndrome is a rare entity. Its pathophysiology is still poorly understood.Case presentationWe report the case of a 69-year-old White European woman who presented complete and proportional right hemiplegia, confusion, deviation of her head and eyes to the right, cortical blindness, and generalized tonic-clonic seizure 12 hours following a depletive lumbar puncture. Emergency cerebral magnetic resonance imaging showed bioccipital and left-side basal ganglia hyperintensities in the fluid attenuated inversion recovery and the diffusion-weighted images suggesting a radiological diagnosis of posterior reversible encephalopathy syndrome.ConclusionsThe diagnosis is established on clinical and radiological signs. This is the first report of this kind in the literature. We present a case of posterior reversible encephalopathy syndrome after depletive lumbar puncture and we discuss the pathophysiology.

More than memory impairment in voltage-gated potassium channel complex encephalopathy.

Autoimmune encephalopathies (AEs) are a heterogeneous group of neurological disorders that affect cognition. Although memory difficulties are commonly endorsed, few reports of AEs inclusively assess all cognitive domains in detail. Our aim was to perform an unbiased cognitive evaluation of AE patients with voltage-gated potassium channel complex antibodies (VGKCC -Abs) in order to delineate cognitive strengths and weaknesses. Serial VGKCC -Ab AE subjects (n = 12) were assessed with a comprehensive evaluation of memory, executive functions, visuospatial skills and language. Clinical magnetic resonance imaging (MRI) (n = 10/12) was evaluated. Five subjects had serial cognitive testing available, permitting descriptive analysis of change. Subjects demonstrated mild to moderate impairment in memory (mean Z = -1.9) and executive functions (mean Z = -1.5), with variable impairments in language and sparing of visuospatial skills. MRI findings showed T2 hyperintensities in medial temporal lobe (10/10) and basal ganglia (2/10). Serial cognitive examination revealed heterogeneity in cognitive function; whereas most patients improved in one or more domains, residual impairments were observed in some patients. This study augments previous neuropsychological analyses in VGKCC -Ab AE by identifying not only memory and executive function deficits but also language impairments, with preservation of visuospatial functioning. The study further highlights the importance of domain-specific testing to parse out the complex cognitive phenotypes of VGKCC -Ab AE.

Toxic encephalopathy due to colchicine—Gloriosa superba poisoning

Gloriosa superba, a flowering plant widespread in South and Southeast Asia, is implicated in many cases of self-poisoning. Colchicine is concentrated in the seeds and tubers and this mediates its...

Diagnostic profiles of patients with late-onset Creutzfeldt–Jakob disease differ from those of younger Creutzfeldt–Jakob patients: a historical cohort study using data from the German National Reference Center

In contrast to other neurodegenerative diseases, sporadic Creutzfeldt-Jakob disease (sCJD) is rarely diagnosed in patients older than 75 years. Data describing the characteristics of sCJD in the very old are rare and inconclusive. Therefore, a historical cohort study was designed to evaluate clinical, cerebrospinal fluid (CSF), electroencephalography (EEG), and magnetic resonance imaging (MRI) features of this group. Patients older than 75 years identified via the German surveillance program from 2001 to 2012 (n = 73) were compared to a random subsample of sCJD patients younger than 75 (n = 73) from the same time period using an historical cohort design. Older patients showed a faster disease progression represented by an earlier point of diagnosis and a shorter survival time (p < 0.001). In the early stages of disease, older patients presented slightly more often with dementia (p = 0.127) or dysarthria (p = 0.238), whereas disorders of the extrapyramidal (p = 0.056) and visual system (p = 0.015) were more common in the younger group. Atypical MRI profiles such as MRI lesions restricted to one hemisphere (p < 0.001) or cortical lesions only (p = 0.258) were found more frequently in patients older than 75 years, whereas typical cortical and basal ganglia hyperintensities were more common in the younger group (p = 0.001). We demonstrated for the first time that patients with late-onset sCJD differ from younger sCJD patients with respect to MRI profiles and initial clinical presentation, but not among CSF markers. Misclassification of Creutzfeldt-Jakob disease cases in patients older than 75 years seems likely due to atypical clinical and radiological presentation. This might contribute to lower sCJD incidence rates in this age group.

Delayed hemichorea syndrome associated with nonketotic hyperglycemia

A 69-year-old woman was admitted for investigation of an acute-onset right hemichorea1. T1-weighted brain magnetic resonance imaging (MRI) showed hyperintensity in the left basal ganglia (Figs 1A and B). Although diabetic, she had no hyperglycemia. Interestingly, two weeks earlier, the patient was admitted due to nonketotic hyperglycemia. Brain computed tomography, previously reported as normal (Fig 1C), showed subtle hyperdensity in the left basal ganglia. However, there were no movement disorders. Choreic movements have been reported in patients with nonketotic hyperglycemia. Usually, dyskinesia [...]

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) in Two Siblings with Neuropsychiatric Symptoms

erebral autosomal dominant arteriopathy with subcorti-cal infarcts and leukoencephalopathy (CADASIL) is arare hereditary disease that presents with various neurologicaland/or psychiatric symptoms, such as stroke, migraine with aura,cognitive changes, mood disorders, and dementia. Mutations inthe Notch3 gene have been documented in most of the patients.Clinically, the most useful diagnostic tool is cranial magneticresonance (MR) imaging, which demonstrates widespread sub-cortical leukoencephalopathy associated with hyperintensities inbasal ganglia, thalamus, and brainstem. Psychiatric symptomsare seen in 20%–41% of the patients, and are rarely the initialpresenting symptom. Here we present two siblings in their 40swith prominent psychiatric complaints. Clinical diagnosis ofCADASIL was made years later, after a careful personal andfamily history combined with typical MR imaging findings.Both patients had a missense mutation in exon 4 of Notch3. Ifpatients with various psychiatric symptoms have a history ofheadache, stroke-like episodes, or cognitive changes, and a pos-itive family history of similar complaints, they should undergocranial MR imaging. In case the imaging demonstrates unex-plained leukoencephalopathy, patients should be investigated forCADASIL.Cerebral autosomal dominant arteriopathy with subcor-tical infarcts and leukoencephalopathy (CADASIL) is a rarehereditary brain disease that in most patients occurs due tomutations of the notch 3 gene on chromosome 19q12.

The first inborn error of manganese metabolism caused by mutations in SLC30A10, a newly identified manganese transporter

BackgroundWe have identified an autosomal recessively inherited disorder of manganese metabolism that causes manganese accumulation in liver and brain with characteristic MRI brain appearances of hyperintense basal ganglia on T1-weighted sequences. Most affected individuals present in childhood with difficulties walking and fine motor impairment due to dystonia. Movement disorder is accompanied by liver cirrhosis, and some patients have died at a young age following complications of cirrhosis. An adult-onset form of parkinsonism associated with hepatomegaly and hypermanganesaemia has also been described. Further characteristics of both phenotypes include polycythaemia and features of iron depletion. MethodsHomozygosity mapping was performed using an Illumina CytoSNP-12. The candidate gene was sequenced on an ABI sequencer. Functional studies in the manganese-sensitive yeast strain Δpmr1 were performed using Gateway technology (Invitrogen). FindingsHomozygosity mapping identified SLC30A10 as the affected gene, and homozygous sequence changes were found in all affected individuals. SLC30A10 had previously been presumed to belong to a class of zinc transporters. However, expression of human wildtype SLC30A10 in Δpmr1 rescued growth in high manganese conditions confirming its role in manganese transport. The presence of missense and nonsense mutations in SLC30A10 failed to restore manganese resistance. InterpretationEvidently, evolutionary changes in the aminoacid sequence have altered the substrate specificity of this transporter from zinc in yeast to manganese in mammalian cells. SLC30A10 is the first recognised human manganese transporter that, when defective, causes two distinct phenotypes—childhood onset dystonia and adult onset parkinsonism—that are associated with hepatic cirrhosis and polycythaemia. Present treatment strategies, including chelation therapy with disodium calcium edetate and iron supplementation, lead to significant improvement of clinical symptoms and blood manganese levels. FundingAction Medical Research.

Variability in Diagnosing Creutzfeldt–Jakob Disease Using Standard and Proposed Diagnostic Criteria

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive dementia with a median survival of 2-14 months. The diagnosis can only be made accurately by biopsy/autopsy. However, this is not always feasible or desirable. Thus, diagnostic criteria have been proposed by UCSF, European MRI-CJD Consortium, and WHO. We will compare these criteria. Retrospective study of 31 patients (average age of 69.2 years) between 2003 to 2010 by ICD9 codes 046.1, 046.11, and 046.19. All patients presented with rapidly progressive dementia (mean duration of 4.25 months). Pyramidal and extrapyramidal findings, myoclonus, cerebellar changes, akinetic mutism, and visual disturbances were observed in 6.5-48.4%. Five had periodic pattern on EEG. CSF biomarker 14-3-3 was positive in 11. Tau was positive in 6. Neuron specific enolase was positive in 9. By consensus (kappa = 0.62), MRI was "typical" of CJD in 23 with cortical ribboning (n = 16), basal ganglia hyperintensity (n = 15), or combination (n = 8). By WHO criteria, which does not include neuroimaging, CJD was diagnosed in 10, but 14 if any CSF biomarker was used (p = NS). The UCSF criteria, which does not include CSF biomarkers, diagnosed 18 cases, and the European MRI-CJD Consortium, which includes neuroimaging and CSF biomarkers but with less neurological signs, diagnosed 23 cases (p < 0.05 and p < 0.001, respectively). CJD-mimics included urosepsis, neurosarcoidosis, idiopathic left temporal lobe epilepsy, alcohol intoxication, central nervous system vasculitis, viral encephalitis, and non-Hodgkin's lymphoma. This study illustrates the variability in diagnosing CJD and emphasizes the diagnostic utility of neuroimaging. It also highlights false-positives that occur with neuroimaging.

Dyskinesia associated with hyperglycemia and basal ganglia hyperintensity: report of a rare diabetic complication

The syndrome of dyskinesia associated with hyperglycemia and basal ganglia hyperintensity on T1 - weighted MR images is rare and most often affects elderly patients with type 2 diabetes. We report a case of a 79year-old female patient who presented to the ED with a 12h history of a left sided hemichoreoathetosis. Laboratory results revealed pronounced nonketotic hyperglycemia [27mmol/L (486mg/dL); HbA1c 140mmol/mol (15%)] and brain MRI showed bilateral T1 hyperintensity in the basal ganglia, more noticeable on the right side. One week before she had been admitted with a diagnosis of transient ischemic attack consisting in left hemiparesthesia, also with nonketotic hyperglycemia [38.9mmol/L (700mg/dL)] and was discharged home with partial correction of her metabolic disturbance. The movement disorder did not improve with adequate glycemic control so haloperidol was started. Six weeks later she was seen on an outpatient basis. She still had minimal residual involuntary movements of the left arm and leg. Laboratory exams revealed a well controlled diabetes mellitus [glycemia 6.0mmol/L (109mg/dL), HbA1c 57mmol/mol (7.4%)]. In conclusion, the syndrome of dyskinesia associated to hyperglycemia and hyperintensity in the basal ganglia on T1 - weighted MR images is a rare, intriguing and yet incompletely understood complication of diabetes mellitus. The increasing number of reported cases may help to better understand its peculiarities such as the existence of a clear clinical radiological dissociation and to unveil pathophysiological aspects. We suggest the possibility that the metabolic disturbances unmask a previous established asymptomatic striatum vasculopathy.