A Rare Case of Hyperglycemia, Ketoacidosis, and Central Facial Paralysis in a Newly-Diagnosed Diabetic Woman

Introduction

Diabetes mellitus is a chronic disease that occurs when the pancreas does not produce enough insulin or when the body is unable to effectively use the insulin it produces [1]. In 2021, 9.3% of the world’s adult population had diabetes (9% for women, 9.6% for men), with an increase by age (18.8% after 65) and strong regional disparities [1].

Hyperglycemia is defined as a blood glucose level above the target values for most patients with diabetes of either above 7 mmol/L on an empty stomach or before a meal or above 10 mmol/L at 2 h after the start of a meal [2]. Diabetic ketoacidosis, a life-threatening complication of diabetes, generally observed in patients with type 1 diabetes mellitus, is characterized by blood sugar levels above 250 mg/dL, arterial pH below 7.3, serum bicarbonate level less than 15 mEq/L, and presence of ketonemia or ketonuria, with an anionic gap greater than 14–15 mEq/L [3]. Rehydration, insulin therapy and correction of electrolyte disorders are the pillars of the management of diabetic ketoacidosis [3].

Impaired glucose metabolism, ranging from hypoglycemia to hyperglycemia, can be accompanied by some neurological manifestations [4]. These manifestations can occur in known diabetic patients or can reveal ketosis-free hyperglycemia [4]. Much of the literature mentions its manifestations, including seizures partial epileptics, abnormal movements (hemichorea and athetosic), aphasia, homonymous hemianopia, generalized tonic-clonic crises, hemifacial spasms, and peripheral neuropathy, including facial paralysis [2,4–9].

Diabetic peripheral neuropathy is the most common subtype of many possible causes of peripheral neuropathy; approximately 10% to 20% of patients have a concomitant diagnosis of peripheral neuropathy [10]. The literature reports that 50% to 66% of patients with diabetes mellitus will eventually develop peripheral neuropathy in their lifetime [11]. Studies have shown that people with diabetes mellitus have an increased risk of developing peripheral facial paralysis, including Bell paralysis and Hunt Ramsey syndrome [12]. The incidence rates of Bell paralysis and Hunt Ramsey syndrome, as assessed by Seo et al, were 31.42 and 4.58 per 10 000 person-years, respectively [9].

Cases of abnormal hemifacial spasm-like movements in diabetes mellitus, a rare acute complication of diabetes mellitus, have been reported in the literature [13,14].

Except for that of diabetic peripheral neuropathy, the pathophysiology of these neurological manifestations is poorly known. Some authors suggest an increased metabolism of gamma aminobutyric acid (GABA) causing GABAergic depression in the brain [6], and for others, hyperglycemia would act through hyperosmolarity, causing a hyperosmolar gradient between the intra- and extracellular sector of the neuronal environment. This results in intracellular dehydration, which is at the origin of neuronal lesions [4,7].

We describe a 59-year-old patient with central facial paralysis, with no radiological abnormalities and hyperglycemia with ketoacidosis, due to newly diagnosed diabetes mellitus. The neurological manifestation resolved within a few days after medical treatment and metabolic correction.

Case Report

A 59-year-old female patient was hospitalized for the discovery of diabetes mellitus, revealed by a polyuro-polydipsic syndrome, causing a weight loss of 16 kg in 1 month. Three of her sisters and 1 brother had known diabetes. She had hypertension for more than 3 years, which was treated with prolonged-release nicardipine 50 mg and irbesartan 75 mg. Clinically, the patient reported intense thirst, physical asthenia, abdominal pain, and postprandial vomiting. Blood pressure was 144/87 mm Hg, heart rate was 93 beats per min, respiratory rate was 26 breaths per min, and temperature was 36°C. She had a good oxygen saturation, at 99% in ambient air, weighed 81 kg, and had a height of 163 cm. The general clinical examination was marked by a dryness of the oral mucosa. The neurological examination essentially showed drowsiness with temporal disorientation, without focal signs. Biologically, the following were found: elevated blood sugar level of 34.7 mmol/L (3.8–6.38 mmol), elevated ketone level of 5.3 mmol/L (0.5 mmol/L), HbA1c of 12.9%, (6%), acid pH of 7.06 (7.31–7.41), elevated plasma osmolarity of 326 mosml/L (281–310 mosml/L), increased ionic gap of 30 mmol (8–12 mmol), sodium level of 139 mmol/L (136–145 mmol/L), potassium level of 4.7 mmol/L (3.5–5.1), chloride level of 102 mmol/L (98–102 mmol/L), and alkaline reserve of 5 mmol/L (22–29 mmol/L).

Sudden-onset left central facial paralysis was observed at 6 h after admission. Brain magnetic resonance imaging (MRI) performed 1 h after the onset of facial paralysis showed no abnormalities on all sequences. Angioscan of the supra-aortic trunks and Willis polygon was normal. The diagnosis of transient ischemic accident in the setting of newly diagnosed diabetes mellitus was retained. She was treated with insulin and rehydration, with normal saline solution (4 L for the first 8 h) with potassium supplementation, aspirin 75 mg, clopidogrel 75 mg, and atorvastatin 80 mg. Because of the persistence of central facial paralysis beyond 96 h, a second brain MRI was conducted, which showed no parenchyma damage. Facial paralysis completely regressed on the fifth day of hospitalization, 48 h after normalization of ketone bodies and blood sugar levels (Tables 1, 2). The diagnosis of central facial paralysis on diabetic acido-ketosis was retained, after exclusion of focal brain lesions and based on its evolution in relation to the normalization of blood sugar levels and ketone bodies.

Discussion

Acute neurological manifestations regressing after correction of non-cetosic or ketosic hyperglycemia are increasingly reported in the literature as rare complications of poorly treated or newly diagnosed diabetes mellitus [4,6,7,14]. Brain MRI often shows specific abnormalities, and sometimes is normal [6,13,15].

Our patient acutely presented central facial paralysis in a context of hyperglycemia, with ketoacidosis revealing diabetes mellitus, with regression of symptomatology in a few days after metabolic correction by insulin therapy and rehydration.

Central facial paralysis is usually a symptom associated with an organic brain injury of vascular, tumor, or infectious origin [16,17]. The occurrence of central facial paralysis in a context of hyperglycemia with ketoacidosis and its regressive character after metabolic correction seems to be unusual. Cases of acute peripheral facial paralysis and acute hemifacial spasm as a complication of diabetes mellitus have been reported in the literature [9,13].

Brain MRI of patients with transient neurological manifestations during transient metabolic disorder and cases of hyperglycemia are particularly evocative, with cases of hyposignal T2/fluid attenuated inversion recovery and a decrease in the apparent coefficient of diffusion of the white substance underlying the epileptic cortex, striatum hypersignal on T1, in diabetic striatopathy [15,18]. In the present report, the patient’s brain MRIs performed 1 h and 96 h after the onset of facial paralysis showed no damage to the cerebral parenchyma. Our observation corroborates those of the study conducted by Baltyde et al [5], who observed an absence of cerebral parenchyma lesions on MRI in 8 out of 18 patients. As in our case, the spectacular clinical evolution characterized by the regression of symptomatology after metabolic correction by insulin therapy has been observed in most reported cases [6,13].

Although there are pathophysiological hypotheses in the literature that can explain the occurrence of epileptic seizures [4,5,19] and abnormal movements, such as hemichorea in a context of non-ketotic hyperglycemia [15], those that can explain the occurrence of central facial paralysis are not yet described and remain unknown.

Conclusions

Central facial paralysis in the context of diabetic ketoacidosis is a rare neuroendocrine entity. This report highlights the importance of diagnosis, early management of hyperglycemia and diabetic ketoacidosis, and reversibility of central facial paralysis after treatment.

The pathophysiological mechanisms that can explain the occurrence of central facial paralysis are not yet described and require further investigation.

When a patient presents with central facial paralysis occurring in a context of hyperglycemia with acido-ketosis and a normal brain MRI, and with symptomatology regressing after metabolic and hydroelectrolytic correction, the metabolic cause must be evoked, after eliminating any organic cause.