Case Presentation: Patient is 46 y/o w/ PMH poorly controlled DMII, HTN, polysubstance use disorder, PVD c/b LLE BKA and chronic wounds who was brought in by family to the emergency room with new hemichorea of left upper extremity, dysarthria/anarthria, generalized weakness. Unable to communicate verbally 2/2 dysarthria, but able to communicate using written language. Patient also noted to have progressively worsening incontinence of stool and urine, unable to leave his bed 2/2 weakness, last heroin use one week ago, and last insulin use seven months ago. Presenting blood glucose 1253 and BMP showed normal Ph, tachypnea, iso CO2 elevated 58, lactate 4.62, mag mildly elevated at 2.5. Urine drug test positive for amphetamines, opiates. Head CT negative for intracranial hemorrhage. Patient was evaluated by Neurology in the ED and diagnosed with presumed hemichorea-hemiballismus syndrome (symptomatic diabetic striatopathy), versus simple partial seizures which have also been well-described in hyperglycemia. They recommended CTA head and neck (normal), and MRI brain w/ c-spine which showed focal change suggestive of partial seizure affecting LUE. However, EEG showed no epileptiform changes. Over his hospital stay, this patient did have return of speech, and cessation of hyperkinetic symptoms with adequate treatment of HHS. It was ultimately determined that likely trigger of severe hyperglycemia episode was self-cessation of home insulin along with severe necrotizing soft tissue infection of RLE.
Discussion: Diabetic striatopathy is a rare cause of hemichorea-hemiballismus syndrome (a movement disorder characterized by involuntary movements of the unilateral arm, leg, or face). It is one of the known neurological sequelae of non-ketotic hyperglycemia, often precipitated by an acute surge in blood glucose in the setting of poorly controlled DMII. It is frequently diagnosed through the combination of characteristic movements and signature changes on imaging of the striatum. Interestingly, in this case, the typical changes to the striatum were not noted on MRI imaging, prompting evaluation for potential seizure. Because of its focal and unilateral presentation, this condition can be easily mistaken for an acute neurological event. The mainstay of treatment is glycemic control, and complete resolution of symptoms is expected. Extensive neurological workups and imaging are not recommended. We present a case report here that explores presentation, diagnosis, and management of this condition in an effort to contribute to efficient recognition and patient care. What complicated this case was the confounding PSUD, BKA of LLE (would theoretically have been affected), and lack of classic MRI findings. Ultimately the diagnosis was made after excluding several other possible etiologies of his presentation.
Conclusions: In a patient presenting with severe hyperglycemia and a new movement disorder, physicians should consider diabetic striatopathy. It is reasonable to assess neurological accidents, metabolic disorders, along with intoxication/withdrawal as possible etiologies of presentation up front, but these may co-exist, as in our case. Diabetic Striatopathy can go undiagnosed if the presentation and workup anchors to other neurological or metabolic disorders, and providers must maintain a certain level of suspicion in the setting of poorly controlled diabetes or significant hyperglycemia.