Diabetic Striatopathy in a Middle‐Aged African Woman With Poorly Controlled Type 2 Diabetes Mellitus and HIV Coinfection—A Case Report

ABSTRACT

Diabetic striatopathy is a reversible cause of acute chorea occurring in patients with poorly controlled diabetes mellitus, including those even with complex comorbidities like HIV. Timely neuroimaging and prompt metabolic correction are crucial, emphasizing the need for high clinical suspicion.

1. Introduction

Diabetic striatopathy (DS), also termed chorea‐hyperglycemia‐basal ganglia (C‐H‐BG) syndrome or nonketotic hyperglycemic hemichorea, is a rare neurological complication of poorly controlled diabetes mellitus, predominantly type 2 (T2DM) [1]. It is clinically characterized by the acute or subacute onset of unilateral or bilateral chorea‐ballismus, often in the context of marked hyperglycemia, classically nonketotic in nature, although mild ketonuria may occasionally be observed [2]. Radiologically, DS is distinguished by hyperdensity on noncontrast computed tomography (CT) or hyperintensity on T1‐weighted magnetic resonance imaging (MRI) within the striatum (caudate and putamen) [3].

The exact prevalence remains uncertain due to underreporting and diagnostic oversight but is estimated at approximately 1 in 100,000 diabetic individuals [4].

Pathophysiological mechanisms are multifactorial and not fully elucidated. Proposed models include hyperglycemia‐induced hyperosmolarity leading to cerebral hypoperfusion and striatal petechial hemorrhage, reactive astrocytosis, and selective neuronal injury [1, 5]. Additionally, metabolic diversion through anaerobic pathways in nonketotic states may deplete gamma‐aminobutyric acid (GABA), an inhibitory neurotransmitter, resulting in the disinhibition of thalamocortical circuits and hyperkinetic movements [6, 7].

DS typically affects older individuals with long‐standing, uncontrolled T2DM, with a female predominance noted in several series [8, 9]. Diagnosis requires a high clinical suspicion, especially when chorea emerges in the setting of significant hyperglycemia. Coexisting medical conditions, such as HIV infection, can complicate the clinical picture, potentially delaying recognition of DS [10]. Despite growing recognition, DS remains underdiagnosed, particularly in low‐ and middle‐income countries where access to advanced neuroimaging may be limited, and acute neurological presentations are frequently attributed to cerebrovascular disease or central nervous system infections. We present a detailed case of DS in a middle‐aged African woman with long‐standing, poorly controlled diabetes mellitus and newly diagnosed HIV infection, emphasizing diagnostic challenges, radiological findings, management, and outcomes in resource‐limited settings.

2. Case Presentation/Examination

A 59‐year‐old African female presented to the accident and emergency unit of the Komfo Anokye Teaching Hospital (KATH) following three episodes of generalized tonic–clonic seizures, each lasting approximately 5 min, within 24 h. She reported no fever, headache, neck stiffness, vomiting, or abdominal pain. Her medical history included a 15‐year history of T2DM, poorly controlled on oral agents and insulin, and a 4‐year history of hypertension. A month prior to her presentation, she had been diagnosed with HIV at a primary care clinic and started on cotrimoxazole prophylaxis (960 mg daily). Her home medications were: subcutaneous NPH insulin 50 IU daily, pioglitazone 30 mg daily, gliclazide 80 mg daily, nifedipine 30 mg twice daily, losartan 100 mg daily, and atorvastatin 80 mg daily. She lived with her husband and four children.

On examination, she appeared dehydrated with reduced skin turgor. She was afebrile (36.7°C), anicteric, and pale. Vital signs included blood pressure of 180/100 mmHg, a pulse of 98 beats/min, a respiratory rate of 20 breaths/min, and an oxygen saturation of 98% on room air. Cardiovascular examination revealed the apex beat at the 6th left intercostal space, midclavicular line. Chest auscultation indicated reduced air entry in the bilateral lower zones with bronchial breath sounds.

Initial neurological assessment during the post‐ictal phase revealed a Glasgow Coma Scale (GCS) score of 7/15 (E2V3M2). Pupils were bilaterally 3 mm and sluggishly reactive to light. Muscle tone was diffusely reduced in all limbs, and power could not be objectively assessed.

3. Differential Diagnosis, Investigations and Treatment

Arterial blood gas analysis showed a pH 7.351, a bicarbonate 23.9 mmol/L, a sodium 138 mmol/L, potassium 3.5 mmol/L, and a markedly elevated glucose of 35 mmol/L. Effective serum osmolality was calculated at 314.5 mOsmol/kg. Urinalysis revealed glycosuria (3+) and ketonuria (1+). Despite mild ketonuria, the biochemical picture was consistent with a hyperosmolar hyperglycemic state. Initial laboratory results are summarized in Table 1.

TABLE 1.

Shows key laboratory trends.

LAB	24th July 2024	7th August 2024	Reference range
Hemoglobin	12.1	8.3	11.5–16.5 g/dL
Mean corpuscular volume	78	76	76–99 fL
Mean corpuscular hemoglobin	29.2	28.8	26–34 pg
White blood cells	18.77	11.57	4–11 × 109/L
Neutrophil	17.47	8.14
Platelet count	266	499	150–400 × 109/L
Urea	6	2.78	2.5–7.1 mmol/L
Creatinine	113	87	60–110 μmol/L
Na+	138	135	135–145 mmol/L
K+	4.5	3.7	3.5–5.1 mmol/L
Cl−	99	101	98–107 mmol/L
Random plasma glucose	35		3.9–7.8 mmol/L
Effective serum osmolality	314.5		275–295 mOsmol/kg
HbA1c	14.0		4.0%–5.6%
Total cholesterol	4.6		< 5.2 mmol/L
LDL‐cholesterol	2.6		< 3.0 mmol/L
HDL‐cholesterol	1.2		> 1.68 mmol/L
Triglycerides	1.67		< 1.7 mmol/L
Albumin	39.25		35–52 g/L

Initial diagnoses were: (1) hyperosmolar hyperglycemic state secondary to poorly controlled T2DM, (2) newly diagnosed HIV with a possible intracranial space‐occupying lesion (differential of an acute untyped stroke) complicated by aspiration pneumonitis, and (3) systemic arterial hypertension.

She was managed with intravenous 0.9% normal saline (6 L/24 h), a continuous insulin infusion (6 IU/h), potassium chloride replacement, an intravenous phenytoin loading (1 g stat) followed by maintenance (100 mg 8‐h), and broad‐spectrum antibiotics (ceftriaxone 2 g daily and clindamycin 300 mg 6‐h). Cotrimoxazole was increased to 1920 mg twice daily, and nasogastric feeding was initiated.

By hospital day 3, her GCS improved to 15/15. At this juncture, she was noticed to experience persistent, involuntary choreiform movements involving her left upper and lower limbs. Glycated hemoglobin (HbA1c) was 14.0%, confirming chronic poor glycemic control. HIV‐1 and HIV‐2 antibodies was positive.

A contrast‐enhanced head CT scan performed on day 4 demonstrated hyperattenuating density in the right caudate and putamen as well as the left striatum with sparing of the internal capsules without mass effect or oedema, consistent with diabetic striatopathy. Post‐contrast images showed no enhancement (Figure 1).

FIGURE 1.

An axial, contrast computed tomography (CT) scan of the brain demonstrating characteristic hyperdensities (blue arrows) in the right caudate nucleus and lentiform nucleus, as well as the left striatum, with sparing of the internal capsules, consistent with diabetic striatopathy.

Based on these clinical and radiological features, the diagnosis was revised to:

1. Poorly controlled T2DM with nonketotic hyperglycemic hemichorea (Diabetic striatopathy).

2. Newly diagnosed HIV infection.

3. Systemic arterial hypertension.

4. Hyperosmolar hyperglycemic state (resolved).

The management was multifaceted:

Glycemic control: Transitioned to a subcutaneous premixed insulin regimen (Mixtard 25 IU morning, 10 IU evening).

Symptomatic chorea management: Oral clonazepam 1 mg twice daily and oral haloperidol 2.5 mg daily were initiated, leading to gradual reduction in the choreiform movements.

Antiretroviral therapy (ART): Started on a fixed‐dose combination of dolutegravir/tenofovir/lamivudine (50/300/300) once daily, with continued cotrimoxazole prophylaxis.

Antihypertensives included oral amlodipine 10 mg once daily; oral losartan 100 mg once daily, and oral hydralazine 50 mg three times daily.

Ancillary care: Reviewed by dietetics for diabetic meal planning and by physiotherapy for motor rehabilitation. Enoxaparin 40 mg daily was administered for thromboprophylaxis.

4. Conclusion and Results

The patient showed steady improvement. Her chorea markedly diminished, and her glycemic control stabilized. She was discharged after 20 days. At a follow‐up visit 2 weeks post‐discharge, the choreiform movements had completely resolved. Random blood glucose was 10.9 mmol/L.

5. Discussion

This case illustrates a classic presentation of DS that was initially obscured by a dramatic presentation with seizures and altered consciousness due to hyperglycemic hyperosmolar state (HHS)—a known precipitant of DS [2]. The co‐occurrence of seizures and chorea represents two distinct neurological complications of hyperglycemia, which have been described by Dubey et al. [11] as ‘glycemic brain injury’ to integrate hyperglycemia‐related neurological syndromes according to the principal neuroanatomical compartment involved. Within this framework, seizures—especially focal seizures or epilepsia partialis continua—reflect predominantly cortical dysfunction, whereas DS reflects striatal dysfunction [11]. Our patient therefore had overlapping cortical and striatal manifestations of glycemic brain injury during HHS. Published reports have similarly documented the simultaneous or sequential occurrence of seizures and DS. Safan et al. [12] described focal and generalized seizures with hemichorea‐hemiballismus and characteristic lentiform T1 hyperintensity during ketotic hyperglycemia. Chatterjee et al. [13] reported concurrent epilepsia partialis continua and hemichorea in nonketotic hyperglycemia, illustrating that cortical seizures and a striatal movement disorder may coexist. Other reports have described partial seizures with secondary generalization in ketotic hyperglycemia‐associated DS [7]. These observations support a shared metabolic trigger without requiring a single anatomical lesion to explain both manifestations. Although DS is classically associated with nonketotic hyperglycemia, mild ketonuria, as observed in our patient, has been described in some cases [2]. The hallmark clinical manifestation of DS is the development of chorea or hemiballismus, typically unilateral, although bilateral involvement has been described [4, 14]. In this patient, the appearance of unilateral choreiform movements after partial metabolic stabilization was a critical diagnostic clue. Such delayed recognition is common, as early neurological findings may be obscured by altered mental status related to hyperglycemia or other systemic illnesses.

DS is an uncommon but important metabolic complication of diabetes mellitus that manifests as a hyperkinetic movement disorder in association with characteristic striatal imaging abnormalities. Although most cases have been reported in Asian populations [1], increasing reports from other regions suggest that DS is likely underrecognized rather than truly rare [15].

In resource‐limited settings, DS is frequently misdiagnosed as an acute stroke, particularly when symptoms are unilateral [16]. Also, striatal density on CT scan can be misrepresented as a hemorrhagic stroke. Typical DS, however, usually lacks a vascular‐territory pattern, substantial mass effect, edema, or persistent diffusion restriction, and symptoms often improve after correction of hyperglycemia [3, 16]. Nevertheless, DS and stroke are not mutually exclusive. Alkhaja et al. [17] reported DS occurring with acute ischemic stroke, emphasizing that new weakness, aphasia, pyramidal signs, cortical deficits, or imaging evidence of infarction should prompt evaluation for a concomitant vascular event rather than the attribution of all neurological findings to DS. Diabetes, hypertension, hyperosmolarity, endothelial dysfunction, and increased blood viscosity may create a common vascular‐metabolic substrate for both conditions. In the present case, the absence of pyramidal signs, preserved cortical function, characteristic striatal neuroimaging findings, and complete neurological recovery following the correction of hyperglycemia favored a metabolic rather than a vascular etiology.

The pathophysiology of DS is thought to involve a triad of metabolic, vascular, and histological changes in the striatum. The metabolic theory suggests that in nonketotic hyperglycemia, the brain preferentially metabolizes acetate via the GABA shunt, leading to depletion of GABA, the brain's primary inhibitory neurotransmitter and possibly acetylcholine in the striatum [2, 4]. This resulting neurotransmitter imbalance reduces inhibitory output from the globus pallidus interna, resulting in the disinhibition of the thalamocortical motor pathways and manifesting as chorea [2].

Also, the vascular theory postulates that severe hyperglycemia induces a hyperosmolar state that which increases blood viscosity and causes transient hypoperfusion in the small penetrating arteries supplying the basal ganglia [2]. This may lead to petechial hemorrhages, ischemic injury, and the breakdown of the blood–brain barrier without frank infarction. Histopathological studies of DS have demonstrated a spectrum of pathological changes, including gemistocytic astrocytosis (proliferation of swollen reactive astrocytes), reactive gliosis, perivascular lymphocytic cuffing, and occasional hemosiderin‐laden macrophages, with little or no evidence of significant neuronal loss or frank neuronal necrosis [18, 19]. The prominent astrocytic response is believed to contribute to the CT hyperdensity and T1 MRI hyperintensity due to mineralization or protein deposition [20].

Neuroimaging is crucial for diagnosis. Although, noncontrast CT typically shows unilateral or bilateral hyperdensity in the caudate and putamen, often with sparing of the internal capsule [5], similar abnormalities were appreciable on the contrast‐enhanced CT scan performed in our patient. MRI findings include T1 hyperintensity and variable T2 signal, usually without restricted diffusion or contrast enhancement [16]. In our patient, the more marked involvement of the right basal ganglia correlates well with the left‐sided chorea, suggesting that maybe the predominant radiological abnormality, rather than the mere presence of bilateral lesions, determines the clinical lateralization. This highlights that bilateral imaging findings may not necessarily result in bilateral choreiform movements and should not exclude the diagnosis of diabetic striatopathy. These changes are reversible with glycemic control, differentiating DS from ischemic stroke, hemorrhage, or neoplastic processes [4].

Poor long‐term glycemic control is the most consistent risk factor associated with DS. The markedly elevated HbA1c of 14.0% in this patient reflects prolonged exposure to hyperglycemia, which likely predisposed the striatum to metabolic injury. Although DS may occur in newly diagnosed diabetes, chronic uncontrolled disease appears to increase vulnerability and symptom severity, with an anaverage HbA1c of 13.1% reported [4].

This case is particularly instructive due to the concurrent HIV infection. HIV‐associated neurocognitive disorders (HAND) and opportunistic infections were legitimate initial concerns given the patient's immunocompromised state and neurological presentation as the basal ganglia and dopaminergic systems are important targets in HIV‐related neurological disease [21]. Movement disorders reported in people living with HIV include parkinsonism, chorea, dystonia, tremor, myoclonus, tics, and paroxysmal dyskinesias [10, 22]. In untreated or advanced infection, these disorders frequently arise from opportunistic infections or mass lesions involving the basal ganglia, including cerebral toxoplasmosis, tuberculomas, cryptococcosis, progressive multifocal leukoencephalopathy, and primary central nervous system lymphoma. HIV‐associated neurocognitive disorder may also produce psychomotor slowing, gait impairment, and parkinsonian features through neuroinflammation and the disruption of frontostriatal and nigrostriatal circuits [10]. In treated patients, medication effects, immune reconstitution inflammatory syndrome, vascular disease, and coincidental neurodegenerative disorders become increasingly relevant [10].

In this patient, HIV infection was considered a potential alternative or contributory cause but was unlikely to be the primary explanation for the acute chorea. The CT showed the characteristic nonenhancing striatal pattern of DS rather than a focal ring‐enhancing lesion or mass; there were no systemic features of an opportunistic infection, and the movement disorder resolved rapidly after glycemic correction. Nonetheless, chronic HIV infection is a pro‐inflammatory and pro‐thrombotic state associated with endothelial dysfunction, accelerated atherosclerosis, and microvascular disease [23, 24] and this underlying vascular vulnerability could potentially have lowered the threshold for metabolic injury to the striatum during the hyperglycemic crisis. This remains a biologically plausible interaction rather than a proven causal relationship.

The management of DS is twofold: urgent correction of hyperglycemia and the symptomatic control of chorea [4, 5]. Intensive insulin therapy and hydration remain the cornerstone, often leading to gradual resolution of the movement disorder over days to weeks [2]. For severe or distressing chorea, dopamine receptor antagonists (e.g., haloperidol) or benzodiazepines (e.g., clonazepam) are effective, as demonstrated in the management of our patient [14]. The need for these drugs is typically transient, and they can be tapered off as chorea resolves.

The prognosis is generally excellent, with most patients experiencing the complete resolution of neurological symptoms and imaging abnormalities within weeks to months of achieving euglycemia [4]. This favorable outcome reinforces that DS is a functional and metabolic disturbance rather than a destructive infarct.

DS is a treatable neurological emergency that should be considered in any diabetic patient presenting with acute‐onset chorea, particularly in the context of nonketotic hyperglycemia. This case highlights that DS can occur even in patients with complex comorbidities like HIV, which may also contribute to movement disorders, necessitating a high index of suspicion to avoid misdiagnosis. Early neuroimaging and prompt glycemic correction with symptomatic therapy are key to successful outcomes. The excellent neurological recovery seen in this and most reported cases underscores the importance of recognizing DS as a reversible cause of acute movement disorders. This case reinforces the critical importance of long‐term glycemic control in preventing diabetic complications, including rare neurological syndromes. Clinicians should consider DS in any diabetic patient presenting with acute chorea, especially when imaging shows striatal changes, to avoid misdiagnosis and ensure rapid treatment.

Author Contributions

Kojo Awotwi Hutton‐Mensah: conceptualization, data curation, investigation, methodology, project administration, supervision, writing – original draft, writing – review and editing. Francis Asante Baadu: conceptualization, data curation, investigation, methodology, project administration, writing – review and editing. Allswell Ackon: data curation, methodology, writing – review and editing. Alfred Opata: data curation, investigation, methodology, writing – review and editing. Ranis Anamon Amon: investigation, writing – review and editing. Frank Micah: supervision, writing – review and editing.

Funding

The authors have nothing to report.

Consent

The patient provided written informed consent to publish this case report and accompanying images. Our institution does not require ethical approval to report individual cases or case series.

Conflicts of Interest

The authors declare no conflicts of interest.

Data Availability Statement

Data is available upon reasonable request.