Facial twitching: calcium or concussion conundrum? Hypocalcaemia in a young American football player masking an internal carotid artery dissection

Abstract

A 30-year-old male American football player presented to the acute medical unit with left-hand and hemifacial spasms. History and examination revealed hemifacial spasms in keeping with seizure-like activity possibly due to symptomatic hypocalcaemia. Subsequent investigations revealed an adjusted calcium of 1.87 mmol/L and, hence, he was managed with intravenous calcium replacement. He presented two further times in a 1-month period, with subjective limb weakness, despite normal adjusted calcium. During his third admission, he developed slurred speech and a marked facial droop, with absence of power in the right upper limb. Imaging revealed acute and old infarctions in the left middle cerebral artery territory and appearances consistent with left internal carotid artery dissection. This presentation of arterial stroke is atypical but with potentially grave consequences if missed. There is limited literature on the presentation of hemifacial spasm, and its association with ischaemic or haemorrhagic stroke represents a key learning point.

Background

An atypical presentation of internal carotid artery dissection (ICAD) in a post-thyroidectomy patient led to diagnostic uncertainty and misdiagnosis as symptomatic hypocalcaemia. Our case highlights the similar underlying clinical presentation yet grossly different pathophysiology of these conditions and hence highlights the need to constantly revisit an underlying diagnosis, taking into account the wider patient history, if it does not improve as expected with treatment.

Case presentation

A 30-year-old male American football player presented with witnessed seizure-like activity in the form of hand and lip tingling. He has a background of Graves’ disease, for which he had a total thyroidectomy 7 years previously. Following this, he developed hypothyroidism and hypocalcaemia and has since been taking calcitriol, ergocalciferol and high-dose levothyroxine.

Examination revealed signs of hand-tingling and left-sided hemifacial spasm in keeping with clinically symptomatic hypocalcaemia. Subsequent investigations revealed adjusted calcium of 1.87 mmol/L (normal range at our trust is 2.10–2.58 mmol/L) and a CT of his head which revealed no acute intracranial pathology. He was given both oral calcium replacement and intravenous replacement with 10% calcium gluconate intravenously in a litre of 0.9% normal saline at 100 mL/hour, as per guidelines for treatment of severe symptomatic hypocalcaemia. ¹ This increased his adjusted calcium to 2.24 and he was discharged home with a perceived subjective improvement in symptoms.

Seventy-two hours later, he presented with continued paraesthesia, lethargy and reportedly ‘feeling drunk’. The adjusted calcium was again suboptimal at 1.82 mmol/L and deemed to be the cause of his presentation. He was again treated with calcium replacement and discharged home. Three weeks later, he reattended with left facial numbness, right upper limb weakness and tingling. His adjusted calcium was found to be 2.00 mmol/L, and he was given IV replacement despite calcium levels being near the normal range. Clinical examination revealed 5/5 power (Medical Research Council Muscle Score (MRC score))² in the upper limbs, with weakness that was thought to be subjective at the time. Later that night, slurred speech developed and a marked right upper motor neuron facial droop was seen, with complete absence of power in the right upper limb (0/5 throughout). CT of the head and then subsequent MRI of the head revealed acute and old infarctions in the left middle cerebral artery, secondary to left ICAD (figure 1). This condition has been reported in patients who have sustained blunt trauma following contact sport such as American football.

Figure 1.

MRI series demonstrating internal carotid artery dissection.

Investigations

The adjusted calcium values across his multiple admissions are shown in table 1.

Table 1.

Adjusted calcium values

Date	Adjusted calcium (mmol/L)
24 December 2017 (first admission)	1.81
26 December 2107 (discharged)	2.20
29 December 2017 (second admission)	1.82
30 December 2017	2.11
31 December 2017	1.81
1 January 2018	1.79
9 January 2018 (discharged)	1.86
20 January 2018 (third admission)	2.00
21 January 2018	2.34
23 January 2018	2.04

Conclusion of CT report at the time of diagnosis of ICAD

‘Features maybe consistent with OA focal lesion, possibly ischaemic or due to longstanding history and sees a presentation underlying mass lesion needs to be excluded by MRI scan with and without contrast’.

MRI report

The conventional and diffusion weighted scans shows acute and old infarctions in the posterior branches in the territory of the left middle cerebral artery. The axial T2 scan shows an area of loss of flow void and possible thrombus in the left internal carotid artery at the skull base and the axial T2 T1 scan with fat suppression confirms that there is thrombus in the left internal carotid artery. The contrast enhanced MR angiography also shows a filling defect in the left internal carotid artery above the bifurcation. These appearances are consistent with left ICAD but above the carotid artery bifurcation (figure 2).

Figure 2.

CT and MRI of the head 2 months after diagnosis of Internal carotid artery dissection, after recovery of neurological function. CT angiogram of the aortic arch and both carotids was also performed prior to his discharge from the stroke follow-up clinic after 3 months of anticoagulation.

CT angiogram conclusion

Both carotid arteries appear within normal limits with no evidence of any vascular dissection or any stenosis shown. Both vertebral arteries appear unremarkable. There is no significant abnormality shown.

Differential diagnosis

Hemifacial spasm, twitching and tingling are symptoms attributable to a wide range of aetiologies. Common causes include electrolyte disturbance, medication-induced compression by vasculature or mass, trauma and other pathologies, such as hypothyroidism, hypoparathyroidism, demyelinating conditions and trigeminal neuralgia.³ From these causes, the main ones which feature in our patient case include electrolyte disturbance and post-trauma.

Acute hypocalcaemia is an electrolyte disturbance which can present in a variety of manners, varying from asymptomatic, being identified as an incidental finding, to life-threatening and requiring urgent treatment.¹ Symptoms and signs of hypocalcaemia (table 2) often develop when adjusted serum calcium is <1.9 mmol/L. Tetany is characterised by neuromuscular irritability, and symptoms can include perioral numbness and parasthesiae of the hands and feet, such as seen in this patient.³ Other symptoms include muscle cramps, carpopedal spasm, laryngospasm and seizures.³ The increased peripheral neuromuscular irritability seen in acute hypocalcaemia can predispose to seizures.³

Table 2.

Symptoms and signs of hypocalcaemia

Symptoms of hypocalcaemia	Signs of hypocalcaemia
Perioral paraesthesia	Chovstek’s and Trousseau’s sign
Digital paraesthesia	Tetany and carpopedal spasm
Seizure	Laryngospasm
	Prolonged QT interval on ECG

Hence, symptomatic hypocalcaemia causing seizure-like activity is understandably high up in the differential diagnoses for this patient, given his surgical history. Total thyroidectomy carries risk of operative insult to the parathyroid glands; thus, such patients can develop secondary parathyroid hormone deficiency and consequently hypocalcaemia (2,4). The latter develops due to reduced concentration of parathyroid hormone, resulting in excessive renal calcium loss and reduced intestinal absorption of calcium secondary to underproduction of 1,25-dihydroxyvitamin D.⁴ Hence, calcitriol or alfacalcidol is required to maintain normal calcium homeostasis, as in this patient case.

Hypocalcaemia can also be seen following medical treatment for thyroid disease.⁵ Meek et al report asymptomatic hypocalcaemia in a young patient with hyperthyroidism following treatment with carbimazole and atenolol. This was then diagnosed as hypoparathyroidism and calcium levels normalised with oral alfacalcidol.

Following the initial diagnosis of symptomatic hypocalcaemia in this patient, the symptoms of twitching and tingling continued to persist. All clinicians (junior doctors through to consultants in general internal medicine) involved felt this was possible despite treatment; however, these symptoms were in fact alluding to another underlying neurological cause. The onset of arm weakness and facial droop in the patient’s third admission warranted necessary reassessment of the case, ultimately confirming a diagnosis of cerebrovascular infarct of the middle cerebral artery. As our patient developed new symptoms of limb weakness and facial droop, we moved towards consideration of neurological differentials such as acute stroke. Symptoms can be transient, lasting from seconds to minutes, or may persist for longer periods.⁶ Presentations range from ‘face, arms, speech, time to act’ (‘FAST’) positive symptoms⁶ to more unconventional symptoms, such as hemifacial spasm, which can mimic clinical symptomatic hypocalcaemia. Hemifacial spasm can be caused by compression of the facial nerve root exit zone by a vascular loop. Presentations of hemifacial spasm secondary to cerebrovascular infarcts are rare but have been reported.⁷

ICAD was not a differential we initially considered, though it was the confirmed diagnosis in this patient. ICAD is known to cause carotid territory ischaemia and symptoms localised to the ipsilateral side of dissection. These symptoms are varied and include head, facial or neck pain, Horner’s syndrome, pulsatile tinnitus, cranial nerve palsies^{8 9} and symptoms causing mass effect on the brainstem. The repeated presentations of facial weakness and limb tingling were alluding to ICAD in this patient. Almost three-quarters of spontaneous ICADs cause ischaemic events, including stroke in 80%–84%⁸; thus, early diagnosis to prevent cerebral ischaemia is key. It is important to note the potential (although rare) presentation of multiple cervical artery arterial dissections, which have been reported in the literature and associated with poor outcomes.¹⁰ Particularly relevant to this case is that a number of sources have reported ICAD in patient populations who have sustain repeated blunt trauma following contact sport, such as American football.^11–15

Treatment

Internal carotid artery thrombectomy was discussed in a multidisciplinary setting including input from stroke, neurology and vascular surgery. Overall, this procedure was deemed too risky with a risk of embolism and further stroke as thrombus was located in the distal internal carotid artery. Thus, no surgical or endovascular intervention was undertaken and the patient was commenced on dual antiplatelet therapy with lamotrigine for his twitching symptomology. The patient was tested for causes of young stroke (Box 1) with a detailed history and panel of young stroke bloods, all of which were normal and thus did not allude to an underlying cause.

Box 1. Secondary screen for stroke.

• Antinuclear antibodies.

• Antiphospholipid antibodies, anticardiolipin antibodies, lupus anticoagulant.

• Blood culture.

• Cardiac enzymes: troponin, creatine kinase (creatine phosphokinase), lactic dehydrogenase isoenzymes.

• Coagulation factors: antithrombin III, protein C, protein S; factor VIII; activated protein C resistance (factor V Leiden).

• Erythrocyte sedimentation rate.

• Haemoglobin electrophoresis.

• Homocysteine.

• Syphilis serology (Venereal Disease Research Laboratory, fluorescent treponemal antibody and others).

• Toxicology screen (serum or urine).

Outcome and follow-up

This patient received neurorehabilitation input for his unilateral right-sided arm weakness and was discharged from the stroke unit after a few days. He made good physical recovery after 2.5 months, at which point he was discharged from the stroke follow-up clinic following results of his latest CT angiogram of the carotid arteries and aortic arch. The carotid arteries appeared within normal limits, with no evidence of vascular dissection or stenosis. Vertebral arteries appeared unremarkable, with good circulation and no evidence of torn blood vessels. Based on this scan, the stroke team advised him to reduce his antiplatelet therapy to clopidogrel only. He is now slowly resuming physical activity.

Discussion

This case describes a situation where symptomatic hypocalcaemia masked a diagnosis of ICAD. His initial presentation highlighted clinicians to a severe and symptomatic hypocalcaemia. As extracellular calcium concentrations are vital to maintain normal functioning of muscles and nerves, hypocalcaemia predisposes to neuromuscular excitability, thus giving rise to twitching, spasms, tingling and numbness.¹¹ In severe cases, carpopedal spasm can progress to tetany, seizures and cardiac dysrhythmias.¹¹ Hence, it was appropriate for his hypocalcaemia to be treated in the first instance. However, the near-miss in this scenario was failing to consider other differentials for the persisting symptomology, even after the adjusted calcium had been biochemically corrected. Perhaps a thorough social, occupational and personal interest history would have alerted clinicians to the patient’s regular American football playing and prompted consideration of blunt trauma provoking carotid artery dissection.

Cuellar et al¹² report cerebrovascular injury in association with blunt trauma provoked by contact sports. Similar to our patient’s presentation, they reported carotid artery dissection in a young man who had presenting symptoms of unilateral paraesthesia following blunt trauma in a rugby match.¹³ Pathophysiology in such cases has been attributed to crush injury in the carotid¹² and also possibly extreme neck extension, causing the common carotid artery to stretch over C3–C5.¹⁴ Cuellar et al’s¹² patient case had near full resolution of symptoms with conservative management, receiving no anticoagulation or antiplatelet therapy.

Additional cases of blunt cerebrovascular injury causing carotid dissection include presentation of acute hemiplegia 2 weeks after the injury; initial symptoms postinjury include ‘feeling dazed’ like our patient did.¹⁴ This highlights the importance of recognising cases whereby blunt cerebrovascular injury can lead to delayed onset of neurological symptoms. A similar case series in a Mayo clinic suggest that neurological symptoms can present anywhere between 30 min to 10 years postinsult.¹⁵ Hence, complexity in diagnosis exists, especially as our patient had what seemed like an obvious reason for his symptoms: hypocalcaemia following thyroidectomy.

Boden et al¹⁶ studied traumatic fatalities in American football players, finding that out of 164 patients, 1 died from vascular injury due to cervical fractures. While rare, these described cases suggest a possible cause for vascular injury in our patient.

Spontaneous extracranial artery dissection is an uncommon cause of stroke due to formation of an intramural haematoma in an extracranial artery.¹⁷ In patients with early presentation of acute ischaemic stroke due to arterial dissection, thrombolytic therapy is advised; beyond the hyperacute period, antithrombotic therapy with anticoagulation or antiplatelet medication is given. However, controversy remains over such treatments in patients with intracranial extension of extracranial dissection, as risk of provoking subarachnoid haemorrhage exists.¹⁷ Hence, best treatment of internal artery dissection remains ambiguous due to the difficulty in establishing a certain diagnosis within the acute/interventional period. The hallmark features on imaging are often difficult to identify, given the small size of extracranial arteries.¹⁷ Furthermore, low incidence and variation in patient presentation of internal artery dissection pose challenges in reaching this diagnosis, also possibly explaining how our patient’s presentation was overlooked as severe symptomatic hypocalcaemia.

This case study adds to the evidence that exists on blunt trauma in contact sport, possibly causing ICADs. Further research into the associations of American football with cerebrovascular injury is warranted, alongside implementation of guidelines to consider this differential earlier in the patient presentation.

Learning points.

• Extracranial arterial dissections are rare and easily missed; clinicians should be alert for symptoms, such as head, facial or neck pain or weakness, Horner syndrome, pulsatile tinnitus and acute cranial nerve palsies, and neurological symptoms attributed to mass effect on the brainstem.

• All strokes in young patients must be investigated for an underlying cause.

• One of the the most common causes for a stroke in a young patient is carotid artery dissection.

• Social history, including a patient’s occupation and hobbies, should not be overlooked. In this case, the possibility of American football was the likely cause of ICAD.

• Physicians should be vigilant for the overlap between the clinical features of hypocalcaemia and neurological conditions. When a patient has received adequate treatment for the primary diagnosis in a differential yet symptoms persist, it is important to consider alternative diagnoses.

• Internal carotid artery dissection is a significant cause of morbidity and mortality. Interventional endovascular management is risky outside the hyperacute stage.