A Peculiar Diabetic Striatopathy: A Case Report with Normal Basal Ganglia Imaging

To examine the association between brain magnetic resonance imaging (MRI) characteristics and executive function and bimanual performance in children with unilateral cerebral palsy (CP). Clinical MRI brain scans were classified as: (1) predominant pathological pattern (normal, white matter injury [WMI]; grey matter injury; focal vascular insults [FVI]; malformations; or miscellaneous); and (2) focal lesions (frontal, basal ganglia, and/or thalamus). Assessments included: (1) bimanual performance; (2) unimanual dexterity; and (3) executive function tasks (information processing, attention control, cognitive flexibility, and goal setting) and behavioural ratings (parent). From 131 recruited children, 60 were ineligible for analysis, leaving 71 children (47 males, 24 females) in the final sample (mean age 9y [SD 2y], 6y-12y 8mo). Brain MRIs were WMI (69%) and FVI (31%); and frontal (59%), thalamic (45%), basal ganglia (37%), and basal ganglia plus thalamic (21%). Bimanual performance was lower in FVI versus WMI (p<0.003), and with frontal (p=0.36), basal ganglia (p=0.032), and thalamic/basal ganglia lesions (p=0.013). Other than information processing, executive function tasks were not associated with predominant pattern. Frontal lesions predicted attention control (p=0.049) and cognitive flexibility (p=0.009) but not goal setting, information processing, or behavioural ratings. Clinical brain MRI predicts cognitive and motor outcomes when focal lesions and predominate lesion patterns are considered. What this paper adds Early brain magnetic resonance imaging (MRI) predicts bimanual performance and cognitive outcomes. Brain MRI may identify children requiring targeted interventions. Basal ganglia with/without thalamic lesions predicted bimanual performance. Frontal lesions were associated with attention control and cognitive flexibility. Brain MRI predominant patterns predicted motor, not cognitive outcomes, other than information processing.

Clinical Relevance of Cerebral Small Vessel Diseases.

To the Editor: An 86-year-old man with a medical history of diabetes mellitus, hypertension, dyslipidemia, severe tobacco use (10 pack-years), and brain trauma was admitted to the hospital for a left-sided hemichoreoathetosis that had started suddenly 48 hours before. Family history was unremarkable for movement disorders. On neurological examination, he was disoriented to time and space and presented with left-sided abnormal involuntary movements suggestive of hemichoreoathetosis associated with left facial spasms. Brain computed tomography (CT) revealed spontaneous hyperdensity within the right striatum. Blood sample examination showed high fasting glucose (306 mg/dL, normal <126 mg/dL), high glycosylated hemoglobin (HbA1c; 11.9%, normal <6.5%), and high osmolarity (303.3 mOsmol/kg, normal 285–295 mOsmol/kg); urinary ketones were absent; and cerebrospinal fluid analysis revealed high glucose and lactate levels. On brain magnetic resonance imaging (MRI), the right striatum was hyperintense on T1-weighted images without contrast administration, suggestive of diabetic striatopathy. Focal hyperintensity on diffusion-weighted imaging within the right centrum semiovale suggested the presence of an associated recent ischemic stroke (Figure 1). Diabetes mellitus decompensation, with fasting glucose levels reaching 540 mg/dL during the hospital stay, was treated with insulin, and platelet antiaggregation therapy for stroke treatment and recurrence prevention was started. As soon as blood glucose decreased toward normal values, hemichorea gradually subsided, making symptomatic treatment unnecessary; dramatic cognitive and motor improvement were obtained at discharge. Clinical and MRI follow-up were performed. Hypersignal of right lenticular and caudate nuclei and recurrence of left hemichorea were evident until 9 months later in a context of not-yet-satisfactory glycemic control (HbA1c 9.1%). Imaging and clinical findings had disappeared at 24-month follow-up, when better control of diabetes mellitus was evident (HbA1c 8.3%), reinforcing the diagnosis of hyperglycemic hemichorea. He had three recurrent strokes during 24 months of follow-up in the deep territory of the left middle cerebral artery, the right cerebellar hemisphere, and the left medulla oblongata and right corona radiata. This individual was characterized by the overlap of diabetic decompensation with hemichoreic symptomatology and ischemic stroke manifesting with confusion and postural instability. Hemichorea was recurrent during the 9-month period of persistence of basal ganglia abnormalities in the context of poor glycemic control. Diabetic striatopathy is a well-recognized but rare cause of hemiballism-hemichorea, associated with contralateral basal ganglia hyperdensity on CT and hyperintensity on T1-weighted MRI.1-4 The cause of clinical and imaging abnormalities is unclear. Several mechanisms attributed to hyperglycemia have been proposed: transient ischemic injury with microhemorrhage resulting in hyperviscosity caused by hyperosmotic state, calcium deposition, gemistocytosis, altered GABAergic and dopaminergic neurotransmission,5-8 paramagnetic mineral deposition including zinc-containing metallothionein expressed in swollen astrocytes,9 and autoimmunity-mediated inflammation.10 This was the first case, to the authors’ knowledge, of contemporary occurrence of hyperglycemia-related hemichorea and stroke. Two possible diagnostic hypothesis were taken into account: the first considered stroke to have played a central role in diabetes mellitus decompensation, which, in a context of cerebral frailty due to stroke, led to the development of hyperglycemia-related hemiballism-hemichorea; the second considered hyperglycemia as the trigger of stroke through an ischemic injury due to hyperviscosity.5 The available anamnestic data did not help identify which of the two disorders first appeared. The proximity of the stroke lesion to the altered basal ganglia, both in the right middle cerebral artery area, is interesting; although this strengthens the hypothesis of the ischemic mechanism for imaging abnormalities associated to hyperglycemia, it seems in accord with the second hypothesis. It could also be related to the first hypothesis, if the abnormal basal ganglia is considered to enter the ischemic penumbra of the documented stroke. Moreover, a participating direct role of the documented ischemic stroke to the abnormal movements cannot be excluded given the existence also of vascular hemichorea-hemiballism typically associated with lesions of the basal ganglia and, as in this case, of the adjacent white matter.1 Difficulties in optimal glycemic control probably contributed to the persistence for several months of basal ganglia abnormalities accompanied by recurrence of hemichorea, both finally disappearing 24 months later when diabetes mellitus compensation was also evident. Hyperglycemia-related hemiballism-hemichorea and the occurrence of four ischemic strokes during a 24-month period are rare in clinical practice. This man's case raises doubts also about the possible predictive role of hyperglycemia-related hemiballism-hemichorea for recurrence of stroke.

Neuropsychiatric Significance of Subcortical Hyperintensity

Purpose: To describe the brain magnetic resonance imaging (MRI) findings in children with syndromic mitochondrial diseases (MDs).Methods: From 1988 to July 2008, brain MRI of 30 patients diagnosed as syndromic MDs were reviewed. Definite diagnosis of syndromic MDs was made based on the modified diagnostic criteria of mitochondrial diseases.Results: Among the 30 recruited patients, 14 had Leigh syndrome, five exhibited mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome, three manifested mitochondrial cardiomyopathy, two had Kearns-Sayre syndrome, one exhibited fatal infantile mitochondrial myopathy, one manifested myoclonic epilepsy with ragged red fibers, one had Leber hereditary optic neuropathy, one exhibited deafness dystonia syndrome, one manifested chronic progressive external ophthalmoplegia, and one had Pearson syndrome.One patient with mitochondrial cardiomyopathy did not perform brain MRI and five cases (two mitochondrial cardiomyopathies, one fatal infantile mitochondrial myopathy, one chronic progressive external ophthalmoplegia, and one Pearson syndrome) had initially normal brain MRI findings. Initial brain MRI abnormalities were found in 24 out of 29 patients (83%). The most common signal changes on the initial brain MRI were abnormal signal intensity over the basal ganglia, gray matter, and brainstem in 17 (71%), 10 (42%), and 9 (38%) patients, respectively. 14 out of 15 cases (14/15; 93%) were found to have evolutionary changes on the follow-up brain MRI.Conclusions: Brain MRI is a useful tool helping to make the diagnosis of syndromic MDs in children, especially abnormal signal intensity over the basal ganglia. Follow-up brain MRI is important for assessing clinical evolution of the disease.

Morbidity and mortality after pediatric cardiac arrest are chiefly due to hypoxic-ischemic brain injury. Brain features seen on magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) after arrest may identify injury and aid in outcome assessments. To analyze the association of brain lesions seen on T2-weighted MRI and diffusion-weighted imaging and N-acetylaspartate (NAA) and lactate concentrations seen on MRS with 1-year outcomes after pediatric cardiac arrest. This multicenter cohort study took place in pediatric intensive care units at 14 US hospitals between May 16, 2017, and August 19, 2020. Children aged 48 hours to 17 years who were resuscitated from in-hospital or out-of-hospital cardiac arrest and who had a clinical brain MRI or MRS performed within 14 days postarrest were included in the study. Data were analyzed from January 2022 to February 2023. Brain MRI or MRS. The primary outcome was an unfavorable outcome (either death or survival with a Vineland Adaptive Behavior Scales, Third Edition, score of <70) at 1 year after cardiac arrest. MRI brain lesions were scored according to region and severity (0 = none, 1 = mild, 2 = moderate, 3 = severe) by 2 blinded pediatric neuroradiologists. MRI Injury Score was a sum of T2-weighted and diffusion-weighted imaging lesions in gray and white matter (maximum score, 34). MRS lactate and NAA concentrations in the basal ganglia, thalamus, and occipital-parietal white and gray matter were quantified. Logistic regression was performed to determine the association of MRI and MRS features with patient outcomes. A total of 98 children, including 66 children who underwent brain MRI (median [IQR] age, 1.0 [0.0-3.0] years; 28 girls [42.4%]; 46 White children [69.7%]) and 32 children who underwent brain MRS (median [IQR] age, 1.0 [0.0-9.5] years; 13 girls [40.6%]; 21 White children [65.6%]) were included in the study. In the MRI group, 23 children (34.8%) had an unfavorable outcome, and in the MRS group, 12 children (37.5%) had an unfavorable outcome. MRI Injury Scores were higher among children with an unfavorable outcome (median [IQR] score, 22 [7-32]) than children with a favorable outcome (median [IQR] score, 1 [0-8]). Increased lactate and decreased NAA in all 4 regions of interest were associated with an unfavorable outcome. In a multivariable logistic regression adjusted for clinical characteristics, increased MRI Injury Score (odds ratio, 1.12; 95% CI, 1.04-1.20) was associated with an unfavorable outcome. In this cohort study of children with cardiac arrest, brain features seen on MRI and MRS performed within 2 weeks after arrest were associated with 1-year outcomes, suggesting the utility of these imaging modalities to identify injury and assess outcomes.

Limbic encephalitis is characterized by psychosis, memory loss, and seizures. It is sometimes caused by autoimmune mechanisms, including anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Anti-NMDAR encephalitis is typically a multistage disorder. After a prodromal event, psychiatric symptoms develop, followed by seizures, suggesting cortical involvement. Subsequent subcortical involvement is observed in most patients leading to movement disorder, and reduction in consciousness. The signal changes in brain magnetic resonance imaging (MRI) have been reported in the cerebral parenchyma such as the hippocampi, cerebellar or cerebral cortex, frontobasal and insular regions, basal ganglia, and brainstem [1]. Herein, we present a case with anti-NMDAR encephalitis preceded by dura mater lesions in brain MRI. A 50-year-old man with no medical history and head contusion suddenly developed tonic–clonic seizure. Brain MRI showed signal changes in the dura mater of the occipital areas (Fig. 1a, b). A day later, he developed status epilepticus (SE) and was sedated with propofol. Cerebrospinal fluid (CSF) examination showed normal cell counts and elevated concentration of total protein (48.0 mg/dl) and IL-6 (51.0 pg/ml). The laboratory investigations for herpes simplex viruses and human herpesvirus-6 were negative. Antinuclear antibody, thyroid antibodies, onconeuroal antibodies (Hu, CV2, Ma2, Ma1, Yo, Ri, amphiphysin), and glutamic acid decarboxylase antibodies (GAD-ab) were negative. Although high-dose intravenous methylprednisolone (1,000 mg/day for 3 days) led to recovery from SE, he exhibited cognitive impairment (15/30 on the Mini-Mental State Examination), psychomotor agitation, nystagmus, rigidity, postural tremor, and ataxia. Brain MRI on the 14th hospital day showed signal changes in cerebellum and cingulate gyrus with improvement of lesions in the dura mater (Fig. 1c). No additional treatment led to complete recovery from the neurological deficit within 2 months after disease onset. Antibodies to NR1/NR2B heteromers of the NMDA receptor (antiNMDAR antibody), examined by a cell-based assay as described previously [2], were positive in serum and CSF obtained upon admission. Despite an extensive oncologic workup such as whole-body CT and fluorodeoxyglucosepositron emission tomography (FDG-PET), there was no evidence of tumor. He has not had relapsing episodes of encephalitis for 26 months. Repeat FDG-PET every 6 months showed no tumor. Anti-NMDAR encephalitis was originally considered as having paraneoplastic syndrome in young women with ovarian teratoma [1]. However, recently it has become clear that patients with anti-NMDAR encephalitis are not always women and paraneoplastic [3–5]. Brain MRI abnormalities have been reported in the cerebral parenchyma such as the hippocampi, cerebellar or cerebral cortex, frontobasal and insular regions, basal ganglia, and brainstem [1]. Abnormalities in medial temporal lobes are H. Suzuki (&) M. Kitada S. Ueno S. Kusunoki Department of Neurology, Kinki University School of Medicine, Osaka, Japan e-mail: hide_green74@yahoo.co.jp

to investigate the frequency and distribution of new ischemic brain lesions detected by diffusion-weighted imaging on brain magnetic resonance imaging after aortic arch surgery. This preplanned secondary analysis of the randomized, controlled ACE (Aortic Surgery Cerebral Protection Evaluation) CardioLink-3 trial compared the safety and efficacy of innominate versus axillary artery cannulation during elective proximal aortic arch surgery. Participants underwent pre and postoperative magnetic resonance imaging. New ischemic lesions were defined as lesions visible on postoperative, but not preoperative diffusion weighted imaging. Of the 111 trial participants, 102 had complete magnetic resonance imaging data. A total of 391 new ischemic lesions were observed on diffusion-weighted imaging in 71 (70%) patients. The average number of lesions in patients with ischemic lesion were 5.5±4.9 with comparable numbers in the right (2.9±2.0) and left (3.0±2.3) hemispheres (P=0.49). Half the new lesions were in the middle cerebral artery territory; 63% of the cohort had ischemic lesions in the anterior circulation, 49% in the posterior circulation, 42% in both, and 20% in watershed areas. A probability mask of all diffusion-weighted imaging lesions revealed that the cerebellum was commonly involved. More severe white matter hyperintensity on preoperative magnetic resonance imaging (odds ratio, 1.80 [95% CI, 1.10-2.95]; P=0.02) and lower nadir nasopharyngeal temperature during surgery (odds ratio per 1°C decrease, 1.15 [95% CI, 1.00-1.32]; P=0.05) were associated with the presentation of new ischemic lesion; older age (risk ratio per 1-year increase, 1.02 [95% CI, 1.00-1.04]; P=0.03) and lower nadir temperature (risk ratio per 1°C decrease, 1.06 [95% CI, 1.00-1.14]; P=0.06) were associated with greater number of lesions. In patients who underwent elective proximal aortic arch surgery, new ischemic brain lesions were common, and predominantly involved the middle cerebral artery territory or cerebellum. Underlying small vessel disease, lower temperature nadir during surgery, and advanced age were risk factors for perioperative ischemic lesions. URL: https://www. gov; Unique identifier: NCT02554032.

Brain magnetic resonance imaging abnormalities after the Norwood procedure using regional cerebral perfusion

Short stature is a common concern that necessitates pediatric endocrinology evaluation. Growth hormone (GH) deficiency is often included as an etiology. Brain and pituitary Magnetic Resonance Imaging (MRI) with gadolinium-based contrast agents (GBCAs) is the imaging modality of choice in assessing patients with GH deficiency. Given the significant strides made in MRI technology that allow improved spatial and contrast resolution, the necessity of using contrast material when obtaining brain and pituitary MRI in cases of short stature and isolated GH deficiency should be reassessed. We preformed a retrospective review of otherwise healthy patients with short stature and/or GH deficiency who underwent brain and pituitary MRI without and with contrast, to assess the benefit of contrast administration. Introduction: Short stature is a common concern that necessitates pediatric endocrinology evaluation. The etiologies of short stature are diverse. GH deficiency is often included as an etiology although it accounts for only 1-2% of short stature cases. The prevalence of GH deficiency is reported to be ~ 1:3500. The vast majority of GH deficiency cases are idiopathic in nature with only 20% due to organic causes. The organic causes of GH deficiency include congenital central nervous system (CNS) anomalies, tumors and other pathologic conditions that involve the pituitary-hypothalamic region. As a result, the radiological assessment of the hypothalamic-pituitary region is considered standard of care for evaluating patients with GH deficiency. Although brain and pituitary MRI is the imaging modality of choice in assessing patients with GH deficiency, its yield in cases of isolated GH deficiency is very low. In a study of 40 otherwise normal patients with isolated GH deficiency, 35 (87.5%) had normal brain MRIs. The abnormal findings of brain MRI in the minority of isolated GH deficiency cases included pituitary hypoplasia, pituitary stalk agenesis, lack of the normal T1-weighted pituitary hyperintensity in the posterior part of the sella turcica, and the presence of a high-intensity signal at the infundibular level representing ectopic neurohypophysis. Traditionally, these brain and pituitary MRI images are obtained with the use of contrast material (gadolinium). The main purpose of using contrast material is for the evaluation of pituitary microadenomas. Given the fact that significant strides made in MRI technology and pituitary microadenomas are not appeared to be associated with GH deficiency, the necessity of using contrast material when obtaining brain and pituitary MRI in cases of short stature and isolated GH deficiency should be reassessed. GBCAs have been shown to deposit in different tissues including the kidneys and the brain. The risk increases with repeated doses. The clinical significance of this deposition is unclear at this time but warrants caution especially in pediatric population who have a longer expected lifespan to manifest any delayed effects. Allergic reactions and gastrointestinal symptoms in pediatric patients can occur with GBCA administration, although the incidence is low. Using contrast material also increases the total cost of the MRI study and prolongs the time needed to complete it. Moreover, in order to use contrast material, intravenous venous (IV) access is required which causes discomfort and additional stress to children and their families. Therefore, we performed a retrospective review of otherwise healthy patients with short stature and/or growth hormone deficiency who underwent brain and pituitary MRI without and with contrast, to assess whether contrast administration led to diagnoses that would have otherwise been missed and/or impacted the patient’s clinical course.Objectives: - To compare the diagnostic yield of non-contrast MRI with pre and post-contrast MRI of the brain and pituitary in evaluation of pediatric patients with short stature and/or growth hormone deficiency.- A secondary objective is to measure the size of the pituitary gland and correlates it with peak growth hormone levels (using insulin/argenine). Methodology: We included patients who underwent brain/pituitary MRI with/without contrast performed at our institution between Jan 2013-Dec 2018 who have short stature/GH deficiency. We excluded patients with known diagnosis of other pituitary hormone deficiencies prior to obtaining MRI studies, genetic and neurological disorders, known tumors/malignancies of any type, or renal failure. Two pediatric neuroradiologists independently reviewed the brain and pituitary MRI of these patients (each read 50% of the cohort) blinded to the clinical data and diagnoses. Each radiologist initially reviewed only the non-contrast portions of the studies, and subsequently, the same radiologist reviewed the entire study, including pre- and post-contrast portions in a separate session. The two sessions were 6 weeks apart to avoid recall bias. Several imaging findings including size and morphology of pituitary gland, presence of congenital anomalies or focal lesions and any associated intracranial findings systematically recorded, and subsequently analyzed. Hypotheses: 1.The incidence of finding congenital pituitary cysts is the same when obtaining brain/pituitary MRI imaging using gadolinium contrast versus when not using contrast in patients with short stature and or isolate GH deficiency. 2.The incidence of discovering abnormal infundibulum is the same when obtaining brain/pituitary MRI imaging using gadolinium contrast versus when not using contrast in patients with short stature and or isolate GH deficiency. 3.Small pituitary size correlate with GH deficiency. Results: -We identified 327 patients with short stature/GH deficiency from Jan 2013-Dec 2018-224 (68.5%) are males and 103 (31.5%) are females. -The mean age at the time of imaging is 10 years and the median is 11 years. -161 (49.24%) have height z-score &amp;lt; -2.25 and 166 (50.76%) have height z-score &amp;gt; -2.25.-82 (25.07%) have IGF1 z-score for age &amp;lt; -2, 102 (31.19%) have z-score ≥-2 to ≤ -1, 141 (43.12%) have z-score &amp;gt; -1 and 2 (0.62%) have no level done.-63 (19.27%) have GH peak &amp;lt;5, 87 (26.61%) have GH peak 5-7.99, 53 (16.21%) have GH peak 8-9.99, 30 (9.17%) have GH peak &amp;gt; 10 and 94 (28.75%) did not undergo GH provocative testing. -The kappa coefficient for pars intermedia cyst on pre vs. post contrast imaging is 0.74 and 0.55 for the infundibulum on pre vs. post contrast imaging. -The mean pituitary height for patients with IGF z-score &amp;lt; -2 is 3.9 mm, 4 mm for z-score ≥- 2 to ≤ -1 and 4.3 mm for z-score &amp;gt; -1-The mean pituitary height for patients with peak GH &amp;lt; 5 is 3.8 mm, 4.2 mm for peak 5-7.99, 4.3 mm for peak 8-9.99 and 4.4 mm for peak &amp;gt; 10. Conclusion: This question has not been answered or even raised in the literature. Our findings suggest that the there is no added benefit to use gadolinium when obtaining brain/pituitary MRI for the evaluation of GH deficiency/short stature. Furthermore, it seems that there is an association between the pituitary height and the GH status of the cohort which is in line with previous published studies.

Purpose Hemichorea-hemiballism (HCHB) is an uncommon involuntary movement that designates a unilateral uncontrollable, poorly patterned flinging movement of limb at variable amplitude. Our aim of this review is for further etiology recognition and its correlated neuroimaging study in patients with HCHB. Methods We retrospectively studied the records of thirteen patients with HCHB obtained during a period of 2 years (five men and eight women) with ages ranged from 40 to 89 years (mean age 69 ± 11.36 years). Seven patients underwent only brain CT, four patients only with brain MR images and two patients underwent both brain CT and MR images. Results Seven patients had hyperglycemia secondary to diabetes mellitus and six patients had stroke. Among the patients with hyperglycemia, the average blood sugar at the onset was 392.21mg/dl, two patients were found with increase attenuation change at basal ganglia via brain CT, three of them revealed hyperintense lesion at putamen, caudate nucleus and globus pallidus on T1-weighted brain MR images, two showed no evidence of brain lesion on brain CT. Two patients of stroke had basal ganglia infarction, one had thalamus infarction, one had multiple infarction at frontal and corona radiata on brain MR image; one had caudate nucleus infarction, and one with no lesion on brain CT. One of thirteen patients had spontaneous remission in days, four of them had resolution after neuroleptic drugs treatment in days to a month, one patient had symptoms recurrence 10 months after the first attack, the remaining patients had intermittent hemiballistic movement with treatment at out-patient clinic. Conclusion Hyperglycemia secondary to diabetes mellitus and stroke remain as the major causes of HCHB. Seven patients had lesions of basal ganglia and two patients had lesion outside the basal ganglia. The prognosis of HCHB is benign but variable in resolution time. Most of the patients required days to months for symptoms resolution under the neuroleptic agent.

Acute leukoencephalopathy with restricted diffusion (ALERD) is a clinicopathological diagnosis that is characterized by severe encephalopathy and seizures with extensive areas of restricted diffusion in cerebral parenchyma. Case 1: Apparently healthy 13-month-old male child presented with acute febrile encephalopathy with a flurry of seizures with raised intracranial pressure. Investigations were consistent with multiorgan dysfunction along with severe metabolic acidosis, ketosis, and hyperammonemia. Initial magnetic resonance imaging (MRI) Brain was normal. However subsequent MRI brain suggested features of diffuse ALERD. With immunotherapy and supportive management, he improved and was discharged without sequelae. Case 2: A 9-month-old male child who was premorbidly normal presented with fever, status epilepticus, and encephalopathy. MRI Brain was suggestive of central sparing ALERD. He was discharged with sequelae with the advice of rehabilitation. Unfortunately, he got succumbed due to aspiration. Case 3: A 10-day-old neonate presented with the refusal of feeds, multiple seizures, and encephalopathy. MRI Brain revealed central sparing ALERD. Improved with immunotherapy and was developmentally normal at five months of age. ALERD is a clinic-radiological syndrome characterized by acute encephalopathy with restricted areas of diffusion in subcortical white matter on MRI. Reported two categories are Diffuse and Central sparing ALERD. Diffuse ALERD has severe manifestations. However, Case 1 had a good outcome, suggesting a variable prognosis. Central sparing ALERD is a milder form, but when basal ganglia are involved, it may have a worsened outcome, as seen in Case 2. Case 3 had central sparing ALERD, and had a good prognosis as described in literature. This study highlights the varied signs and symptoms of ALERD, including neonatal age of onset. Diagnosis is based on the restricted diffusion in white matter on diffusion-weighted imaging and the apparent diffusion coefficient in MRI of the brain. MRI may be normal in the initial course; hence, it’s important to repeat if strongly suspected. Prompt immunotherapy and supportive care are emphasised.

Diabetic striatopathy (DS) is a rare complication secondary to hyperglycemia, featured by the choreiform movements and reversible striatal abnormalities on neuroimaging. Several studies have described the clinical characteristics of DS, however, the simultaneous occurrence of DS and acute ischemic stroke (AIS) in the striatum has not been reported. Herein, we report a 68-year-old man with uncontrolled type 2 diabetes who experienced the progressive involuntary movement of the right upper and lower limbs for 10 days. We initially considered this patient as an AIS with hemorrhage in the left basal ganglia and adjacent area because his brain magnetic resonance imaging (MRI) showed hyperintensity on fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI) images, as well as slight T1-hyperintensity around T1-hypointensity. However, his symptoms worsen persistently, which was inconsistent with neuroimaging findings. Further computed tomography (CT) scan revealed an extensive hyper-density and focal low-density in the left striatum, suggesting the diagnosis of DS and AIS. His symptoms were in complete remission after 2 months of glucose control. However, striatal hyperintensity on T1 images was significantly increased compared to the initial images, which disappeared 18 months later. Additionally, DWI hyperintensity on infarction lesions disappeared, while softening lesions and gliosis were observed on the follow-up MRI images. Therefore, we finally diagnosed the patient as DS complicated with AIS. This report highlights that DS and AIS could occur simultaneously in the striatum after hyperglycemia, which is easily misdiagnosed as AIS with hemorrhage and requires clinicians to pay more attention to avoid misdiagnosis and delayed treatment.

Diabetic Striatopathy (DS) is a rare complication of diabetes mellitus (DM) with poor glycemic control and typical neuroimaging findings. DS predominantly leads to a unilateral reversible injury of the basal ganglia resulting in various movement disorders such as hemichorea and hemibalismus. We present a rare case of DS with left-sided hemichorea as a first presentation for a newly diagnosed Diabetes Mellitus (DM) type II and to make a thorough review of this disorder and to provide a practical approach to its management. A 63-year-old female was admitted to the neurology ward with symptoms of involuntary hyperkinetic movements for the left limbs and the left half of the face. The blood samples revealed random serum glucose of 30.8 mmol/l and an HbA1c of 15.13%. The computed tomography of the brain showed asymmetric, unilateral hyperdensity in the right basal ganglia (BG) – the putamen and nucleus caudatus. The patient was managed with Insulin and Haloperidol as the symptoms entirely resolved on the tenth day. In conclusion (DS) is a condition with a good prognosis and reversible clinical and neuroimaging findings after a prompt diagnosis and management. DS should be considered a possible differential diagnosis in patients with abrupt onset of hyperkinetic movement disorders.

Brain MRI Findings and Neuro-Psychiatric Involvement in Paroxysmal Nocturnal Hemoglobinuria (PNH)