Year : 2014 | Volume
: 1 | Issue : 2 | Page : 90--92
Hemifacial spasm due to non-ketotic hyperglycemia
Department of General Medicine, Institute of Post-Graduate Medical Education and Research (IPGMER), Kolkata, West Bengal, India
Doctor«SQ»s Hostel, Institute of Post-Graduate Medical Education and Research (IPGMER), AJC Bose Road, Kolkata - 700 020, West Bengal
Different movement disorders including chorea and hemichorea-hemiballismus are known to be some interesting presentations of uncontrolled hyperglycemic states (both in type 1 and 2 diabetes mellitus). Hemifacial spasm is rarely reported as a manifestation of hyperglycemic state. Here, the author reports an extremely rare case of hemifacial spasm which developed as the presenting manifestation of non-ketotic hyperglycemia in type 1 diabetes mellitus.
|How to cite this article:|
Chakrabarti S. Hemifacial spasm due to non-ketotic hyperglycemia.Int J Adv Med Health Res 2014;1:90-92
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Chakrabarti S. Hemifacial spasm due to non-ketotic hyperglycemia. Int J Adv Med Health Res [serial online] 2014 [cited 2019 Jun 24 ];1:90-92
Available from: http://www.ijamhrjournal.org/text.asp?2014/1/2/90/148016
A 34-year-old non-smoker and non-alcoholic male presented with intermittent twitching involving all the facial muscles of the right half of face and upper eyelid, exacerbated by eye blinking and chewing for last 12 days. He also complained of increasing thirst and polyuria associated with unintentional weight loss for last 3 months. There was no history of fever or recent flu-like symptoms, head injury, loss of consciousness, altered mentation, seizures, weakness of limbs, difficulty in walking, headache, blurred vision, or slurring of speech. He denied any chronic intake of any drugs (including neuroleptic/anticonvulsant/anticholinergic/anti-Parkinsonian drugs). His past medical history was insignificant. However, both his parents were known to be suffering from insulin-dependent diabetes mellitus (DM), but there was no family history of movement disorders. General examination was unremarkable. Neurological examination also did not reveal any neurodeficit except right-sided hemifacial spasm (HFS) which was severe at level 4 according to Jankovic rating (0 = no spasm, 4 = severe, incapacitating spasm). A random capillary blood glucose level at admission was found to be 365 mg/dl. Urinary ketone was absent. Acid blood gas analysis revealed no acidosis (pH 7.39; N: 7.35-7.45); blood osmolality was normal at 291 mosm/l (N: 285-295). Fasting and post-prandial sugar tests done on the next day revealed elevated values (292 and 401 mg/dl, respectively). C-peptide level was highly suppressed. HbA1c was markedly raised (7.9; normal <6.0). Rest of the routine laboratory parameters including blood counts, renal and liver function tests, serum electrolytes, and antibody profile including (antinuclear antibody (ANA), antineutrophilic cytoplasmic antibody (ANCA), and thyroid autoantibodies) were within normal limits. Urine drug screening was negative. Nerve conduction studies ruled out organic facial nerve lesion. Multiplanar MR imaging of brain and brainstem with contrast showed no abnormality in posterior fossa structures, supratentorial and infratentorial ventricular systems, and extra-axial CSF spaces, and also did not demonstrate any area of altered signal intensity. Normal flow void signals were present in major cerebral blood vessels on MR angiography. Awake EEG revealed no abnormal discharge. A diagnosis of non-ketotic hyperglycemia (NKH) in type 1 DM with HFS was made. Patient was started on subcutaneous regular insulin at 0.1 U/kg/day. Facial spasm gradually subsided in intensity with progressive reduction of blood glucose and correction of acidosis, and ultimately disappeared after 2 days of complete normalization of glucose levels. He did not require administration of drugs like carbamazepine or clonazepam for control of HFS. He was discharged in a stable condition after 10 days following stabilization of glucose levels. Follow-up over 1 year documented stable glycemic control and absence of any recurrence of HFS.
NKH in DM may manifest with diverse movement disorders. Focal seizures, epilepsia partialis continua, myoclonus-opsoclonus, asterixis, choreoathetosis, etc. are some often reported forms of abnormal movements in NKH. These movement disorders may mimic cerebrovascular accidents, meningitis, or psychiatric disorders. Disorders involving facial motor function including facial muscle twitching and jerks in the form of HFS have also been noted rarely. ,,
HFS manifests as tonic-clonic contractions of the muscles innervated by the ipsilateral facial nerve. It is almost always paroxysmal in nature. HFS is both induced and aggravated by voluntary as well as reflexive movements of the face. Most cases of primary HFS are attributed to an aberrant artery which is found to compress the facial nerve at its root exit zone (with anterior inferior cerebellar, posterior cerebellar, and vertebral arteries being the most commonly incriminated).  Secondary HFS which often requires surgical interventions is usually caused by cerebellopontine angle tumors, intracranial tumors, arteriovenous malformations, and aneurysms. Vascular compression at the root exit zone of the facial nerve is frequently demonstrated by magnetic resonance imaging and angiography studies in these cases. Metabolic derangements like NKH are extremely rare etiologies of secondary HFS. 
Focal motor abnormalities may manifest as the initial presentation of DM in 6% of cases.  The involuntary movements in diabetes are often the result of metabolic derangements (hyperglycemia), associated thyroid/parathyroid disorders, infections, and even immunological abnormalities. Asians are found to have a specific predisposition to movement disorders caused by NKH, possibly due to genetic predisposition.  It can be further noted that although movement disorders, especially chorea, hemichorea-hemiballismus, partial seizures etc., have often been observed as the presenting feature of NKH in elderly adults with type 2 DM, similar cases in type 1 DM are comparatively rare. The index case was only 34 years old and had type 1 DM. 
Although not clearly defined, possible pathogenic mechanisms for these movement disorders in hyperglycemic states include relative dopaminergic hyperactivity, impaired synthesis of amino acids, namely acetylcholine and gamma-aminobutyric acid, and focal cerebral ischemia from cerebral hypoperfusion. It is postulated that cerebrovascular resistance increases resulting from hyperviscosity and higher brain water content during hyperglycemia or from loss of flow regulation usually attributed to impaired regional brain metabolism.  Also, hyperglycemia can result in a global decrease in regional cerebral blood flow with maximal reduction being noted in the basal ganglia causing impaired neurotransmitter processing in that region. Electrolytic imbalance, especially hyperkalemia, hyponatremia, and hypocalcemia, can cause neuromuscular irritability resulting in localized involuntary movements in diabetic ketoacidosis.  Again, in advanced diabetic nephropathy, apart from hyperglycemia, uremia itself can independently cause movement disorders. 
It is debatable whether hyperglycemia and ketoacidosis, and/or hyperosmolarity together are required to express the movement abnormality or each in isolation could manifest the abnormality. Bandyopadhyay et al. reported a case of HFS in diabetic ketoacidosis.  However, the authors could not pinpoint whether hyperglycemia alone contributed to the HFS or ketoacidotic state was equally or partly responsible for this neurological manifestation. In the index patient, no specific intracranial pathologic lesion could be detected by imaging, and also, diabetic ketosis or hyperosmolar state was not documented. Uncontrolled hyperglycemia was the sole metabolic derangement, and HFS disappeared after correction of hyperglycemia without requiring specific drugs and never returned during 1 year follow-up, with stable glucose control noted. So, it is probable that HFS in this case occurred as a complication of hyperglycemic state alone. This scenario is being reported for the first time. However, absolute causal association cannot be inferred based on solitary case report. Further case reports depicting similar clinical scenario can consolidate this probable association. Clinical studies including animal studies are warranted in this regard to further shed light on this issue.
Drugs like carbamazepine (CMZ) and clonazepam are usually used for symptomatic relief, but the most effective treatment consists of botulinum toxin A injections at regular intervals of 3-4 months. , However, in cases of secondary HFS, appropriate management of the underlying pathology (including surgical procedures in relevant cases) should be attempted. In the index case, keeping in mind the varied movement disorders possible in hyperglycemic states and that the patient's clinical evaluation was absolutely normal except for the presence of HFS, drugs like clonazepam and CMZ were not initiated at the first place and only normalization of blood sugar was attempted.
Early recognition of hyperglycemia-induced movement disorders is important, as hyperglycemia is a well-treatable disorder, and these cases carry a good prognosis. When hyperglycemia is detected as the cause and it is promptly corrected, the movement disorder usually resolves within 2 days and may not require any symptomatic or specific therapy, as observed in the index patient. Keeping in mind that diverse movement disorders including HFS can be one of the presenting features of DM, screening for DM in all patients who present with sudden and unexplained involuntary movements is important, even in those without a previous history of diabetes, especially in India, where diabetes is highly prevalent and grossly underdiagnosed. This makes possible both diagnosing previously undiagnosed cases of DM and initiating their management, as well as rapid correction of the abnormal movements and preventing unnecessary investigations, resources, and time.
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