A Note on the Digiti Quinti Sign in Hemiplegic Migraine

Abstract

The digiti quinti sign (DQS) was described as an indication of mild hemiparesis. It consists of a wider space between the fourth and fifth fingers at the affected side when the patient extends both arms horizontally to the front with the palms down. The three successively examined sporadic hemiplegic migraine patients presented herein disclosed no other neurological abnormality except the interictally present DQS on the same side of the motor deficits. This sign is perhaps clinically useful for the diagnosis of hemiplegic migraine.

Migraine is a multisymptom complex neurological disease that may impair several cerebral functions either temporarily or permanently. Unilateral paroxysmal motor dysfunction may occur as part of aura symptoms in sporadic or familial hemiplegic migraine (1, 2). Some non-familial, sporadic hemiplegic migraine patients present the same genetic abnormalities present in the familial cases, suggesting that these conditions are closely related (3). Clinical signs indicative of hemiplegic migraine have not been described. The present report calls attention for the importance of the digiti quinti sign (DQS) (4) interictally.

Case reports

Patient A

A 50-year-old female patient complained of recurring intense, throbbing uni-(alternating) or bilateral headaches with nausea, vomiting, photo- and phonophobia since the age of 16 years, with one to four short-lasting mild attacks per month. Twenty years previously, she had presented a 60-min right hemiparesis aura. Mild motor symptoms had recurred ever since. Analgesic medication overuse had been present for the last 3 years. Visual and/or aphasic aura was present in approximately 10% of the episodes. Migraine without associated hemiplegia was described in two sons, her father, a brother and a sister. Magnetic resonance imaging (MRI) was normal. The only abnormality on neurological examination was a marked right-sided DQS (Fig. 1A).

Figure 1

With arms extended, patients A, B and C show the digiti quinti sign (QDS) ipsilaterally to motor aura side (arrows). Notice the greater fifth finger abduction compared with the non-symptomatic side (stars). The angles between the fourth and fifth fingers at the symptomatic side in patients A, B and C are respectively 38°, 20° and 25° compared with 15°, 15° and 10° (non-symptomatic side).

Patient B

A 17-year-old man had migraine (pulsating and intense pain with photo- and phonophobia, no nausea/vomiting) with aura attacks (visual, sensory and motor) since the age of 10 years, now twice a week, lasting 1 day each. The pain started at the left nuchal area, radiating to either the right or left frontal regions. After some minutes numbness and hypoaesthesia in the left side of the face, tongue and cheek were noticed, followed by the left arm and leg, together with 15 min of marked left-sided weakness (40% of the episodes, independent of the headache side). Visual aura might occur simultaneously. His mother, grandmother and aunt (mother's side) had migraine without aura. An MRI scan disclosed a low-grade glioma (Fig. 2). Apart from a DQS on the left side, neurological examination was normal, including coordination (Fig. 1B). The last attack had been 3 days before the examination.

Figure 2

Nodular area (1.2 cm) located at the right middle cerebellar peduncle with hypersignal in the T2 and fluid-attenuated inversion recovery (shown here) sequences, hyposignal in T1 with no contrast enhancement. Diffusion weighted imaging showed no restricted diffusion. Magnetic resonance spectroscopy revealed relative loss of N-acetylaspartate and an increase in choline-containing compounds, suggesting low-grade glioma.

Patient C

A 25-year-old female patient presented with migraine with aura (unilateral right-sided pulsating intense head pain, nausea, photo- and phonophobia) since the age of 13 years, accompanied by discrete ipsilateral nasal congestion, conjunctival injection and lacrimation. Fortification spectra visual aura (15 min) preceded the pain in every attack. Eight years previously the pain had become locked to the left, when she presented the first left hemiparesis and hemihypoaesthesia (15 min, present in most of the attacks). Aura without pain might occur. Kinetosis, cyclic vomiting, recurrent abdominal pain and short-lasting fits with unconsciousness not related to any identifiable disorder were reported in childhood. Rheumatic fever had been diagnosed at the age of 15 years. Her mother, grandmother and brother had migraine without aura. His father suffered from epilepsy and another brother had had occasional migraine without aura and epilepsy since early infancy. The MRI was normal, as was the neurological examination, except for the DQS to the left (Fig. 1C). The last attack was 1 week before examination.

Discussion

The DQS was described by Alter in 1973 (4). It is elicited by asking the subject to keep arms and fingers horizontally extended with the palms down. According to Alter, ‘with mild hemiparesis, the fifth finger on the paretic side may be observed to assume slight abduction, and a space between it and the ring finger is clearly discernible’. The author stated that the DQS may be the only objective sign of hemiparesis, ‘but usually there is corroborating evidence of unilateral weakness such as flattening of the ipsilateral nasolabial fold’. This is the first report of this sign in migraine.

These last three successively examined sporadic hemiplegic migraine patients presented the DQS ipsilaterally to the reported hemiplegic aura and shared the following aspects: (i) they were simply asked to extend the arms with no instruction as how the fingers should be placed; (ii) no other signs indicative of hemiparesis were observed; (iii) no demonstrable MRI lesions potentially involved with hemiparesis were seen; and (iv) examinations were performed interictally. In patient B, the sign could have been caused by the lesion serendipitously found in the middle peduncle. Alter himself discussed the possibility of this sign being related to cerebellar abnormalities (4). This is improbable, as no other cerebellar abnormality was found. Besides, the other two subjects did not present cerebellar lesions, and this patient showed less marked DQS compared with the other subjects.

The present data suggest that the DQS is not only indicative of mild hemiparesis as originally described, but may also be present constantly in migraine with motor symptoms interictally. Permanent changes may be present in migraine. Visual (5, 6), vestibular (7), cognitive (8), motor (2) and various cortical (9) abnormalities have been detected in migraine patients outside attacks. Furthermore, structural changes in the white (10) and grey matters (11) have been described.

A sequence of three individuals with DQS makes the possibility of coincidence quite unlikely. The precise significance of this finding in hemiplegic migraine remains to be determined. In conclusion, the DQS may be a useful and easily assessable clinical sign in migraine with motor symptoms in the absence of overt motor deficits.