Journal of Neurology Research Reviews & Reports

Introduction

Migraine limb pain is a very under-recognized manifestation of migraine in which episodic pain happens in the peripheral limbs. Though it has been described as early as 1857 [1]. Only case reports and case series with limb pain as a symptom of migraine are reported in the literature [2]. It has mostly been described in association with the headache. Here, the author (a neurologist) describes his own experience of episodic, unilateral limb pain without any associated headache in the childhood and episodic headache in adulthood as a manifestation of migraine. This should help in understanding the clinical details of peripheral limb pain -a relatively under-diagnosed symptom of migraine.

Case Presentation
Demographic Details

The case details are of the author himself. The author is currently 42 years age , was born of non-consanguinenous parentage. There was no significant history to suggest any childhood insult.The milestones were normal.

Medical History
Symptoms and Signs

It all started in the early teenage with episodic unilateral pain the lower extermities on the either side, mainly affecting the calf region. At the onset, the pain used to be mild but will slowly become severe enough to disturb the day to day activity. The pain used to be dull boring in character affecting the posterior and sometimes the anterior aspecet of the calf region but at times will also ascend proximally to involve the anterior aspect of the thigh (mainly quadricep region). But it never involved the hip or pelvic region. However, it has rarely involved the ipsilateral upper limb as well. In the upper limb, the commnest area affected was between the elbow to the wrist. It used to last for 4-5hours but occasionally continued even for the whole day or so. If pain persists for more than one day then it may disappear in the affected limb and start in the other lower limb. However, it was not associated with any weakness and there was no aggravtion by prolonged walking, running, or climbing stairs.

The commonest precipitating factor for this peripheral pain was direct exposure of limbs to cold air especially while sleeping. The other precipitating factors were stress, fasting, and sleep deprivation. The relieving factors were compressing the affected leg by either tying a tight cloth around it or by sitting in a way to put own body weight on the affected leg. However , the symptoms may reappear soon after relieving the compression. The best relief used to happen with low dose non -steroidal anti-inflammatory drugs (NSAIDs) like paracetamol or acetylsalicylic acid. The pain frequency was once in 2-3months.

Subsequently, at around the age of 25years, episodic headache started with typical symptoms like severe headache on either side, with photophobia, phonophobia , and nausea lasting for 4-5hours to 48hours at a frequency of 2-3per month fullfilling the ICH criteria of classical migraine [3]. The headache is still there atthe same frequency. The peripheral limb pain is occurring rarely, almost 1-2 times in a year that too after exposure to cold air

Family History

The author is having five brothers and three sisters. All of them are having episodic unilateral headache without any aura qualifying for the criteria of common migraine. All brothers and one sister used to have leg pain at the age of 10 to 20years but 2 elder brothers are still getting leg pain rarely in their forties and fifties. The next generation is also getting leg pain in childhood and headache at a later age

Differential Diagnosis

Various differentials for episodic limb pain were excluded by appropriate investigation. There was no vascular etiology, no vitamin deficiency or nocturnal leg cramps noted. There was no spondylosis or root compression noted by appropriate imaging. There were no features to suggest fibromyalgia or metabolic etiology

As described above various characteristic clinical features can be noted for this peripheral limb pain (peripheral migraine).

Migrating Unilateral Pain- episodic, unilateral pain with occasional migration to the other side.
The Character of the Pain- Starting with dull boring pain in the leg and then increasing in intensity and may involve ipsilateral thigh followed by upper limb.
Duration- Typical duration of a few hours to one to two days
Precipitating Factors- exposure to cold, sleep deprivation, and stress.
Relieving Factors- Relief with NSAIDs or local compression. Applying a band over the head is known to produce relief in headache [4].
Anatomical Localization- Ipsilateral involvement of upper and lower limb will localize the pathology clinically above the cervical region, indicating thalamic dysfunction
Family History - A strong family history of headache and episodic unilateral leg pain, especially in childhood will again favor some relation of this pain to the migraine

Discussion and Conclusion

This case represent clinical features of unilateral episodic limb pain in detail and highlights similarity with unilateral headache seen in migraine. It’s very important to replicate these findings in a large series. A genetic study may help to find a unique genetic variation.

There is very limited data on limb pain in migraine. Prakash et al (2009) described recurrent limb pain and migraine which is quite similar to these details [5]. However they have described pain mainly in the upper limb and some of the subjects had migraine in the beginning and limb pain later in the life. While in this case, it was unilateral lower limb pain in childhood and headache started quite late. Recently familial limb pain has been described in four generations (Heather Angu-Leppan) [6]. It mentions that children are more likely to have leg pain while adults suffer mainly from headache as a manifestation of migraine.

As migraine can have various other peripheral manifestations like episodic abdominal pain, cyclic vomiting, and episodic vertigo, this peripheral pain can be explained with the same theory. The author would like to use the term ‘Peripheral Limb Migraine’ as this can explain the episodic unilateral limb’s symptoms in a better way. In migraine pathophysiology, trigemino-thalamic connections are involved [7]. Similarly spinothalamic connections might be involved in peripheral limb pain.

Conclusion- Episodic unilateral limb pain is similar to unilateral headache in migraine. It is more common in childhood and may preceed by many years before onset of classical headache.

Clinical Implications

• Episodic unilateral limb is a unique symptoms seen in patients with migraine.

• It may be the only symptom in childhood and can be considered as migraine variant.

A proper diagnosis will help in prognostic the patients at an early stage.

Declarations

- Ethics approval and consent to participate- The author is describing his own history so not applicable
- Consent to publish-Yes , consent is there
- Availability of data and materials-reference added
- Competing interests
- Funding-NIL
- Authors’ Contributions-Writing the manuscript
- Acknowledgements-My family members for the inputs

References

1. Guiloff RJ, Fruns M (1990) Migrainous limb pain. A historical note. Headache 30: 138-141.
2. Guiloff RJ, Fruns M (1988) Limb pain in migraine and cluster headache. JNNP 51:1022-1031.
3. Headache Classification Committee of the International Headache Society (2013) The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia .33: 629-808.
4. Headache Classification Committee of the International Headache Society (2013) The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia .33: 629-808.
5. Prakash S, Shah ND, Dholakia SY (2009) Recurrent limb pain and migraine: case reports and a clinical review. Cephalalgia 29: 898-905.
6. Angus-Leppan H, Guiloff RJ (2016) Familial limb pain and migraine: 8-year follow-up of four generations. Cephalalgia 36: 1086-1093.
7. Bernstein C, Burstein R (2012) Sensitization of the trigeminovascular pathway: perspective and implications to migraine pathophysiology. J Clin Neurol 8: 89-99.