1. Epidemiology
The lifetime prevalence of CSH varies depending on age, sex, comorbidities, features of the cold stimulus, and type of exposure.
The general lifetime prevalence ranges from 15% to 37%
[1][2][3]. In the pediatric population, the lifetime prevalence is higher than in the general population and it is reported in the literature as approximately between 40.6% and 79%
[4][5]. Zierz et al. in 2016
[6] compared the lifetime prevalence of CSH in 283 students (10–14-year-old), their parents (401), and 41 teachers with a self-completion questionnaire and found prevalence levels of 62% in the student population and 31% in the adult population. They assume that this might be related to (1) behavioral learning to avoid pain-provoking habits, (2) an age-dependent increased neuronal (e.g., trigeminal) stability against cold stimuli, or (3) the smaller anatomical structure in children, which predisposes them to a quicker local cooling and activation of specific receptors. In the same study
[6], they found that, in the student group, there was no age effect on the prevalence of CSH. Instead, Fuh et al.
[4] found that the prevalence increased with the grade of school in a study based on patients aged 13 to 15 years. This trend was also found in each gender.
According to most of the studies, there is no significant gender-specific prevalence of CSH in children and adolescents
[5][6], while Fuh et al.
[4] found that boys have a significantly higher prevalence of ice-cream headaches than girls (43.4% vs. 37.5%). In contrast, women in the adult groups have a significantly higher prevalence of CSH than men
[1][6][7].
In most of the studies, children and adolescents with other headaches, as adults, have a significantly higher prevalence of CSH, whereas the absence of any previous headache does not result in an increased prevalence of CSH
[1][4][6][7]. Otherwise, Bird et al.
[8] found a prevalence of 27% in migraine patients and 40% in the non-cephalalgic population. This fact could be explained by the distribution of the population of that study because the non-cephalalgic population was mostly composed of students, who generally are younger than the general one.
Comparing patients with a previous history of migraine with those with a previous tension-type headache (TTH), CSH has a higher prevalence in migraineurs (55.2–73.7%)
[4][7] than in patients with TTH (23–45.5%)
[1][4]. In one single study, it was considered CSH in patients with a previous history of stabbing headache (SH): the prevalence of CSH in this population was about 94%
[9].
A previous history of head injury could also be considered as a predisposing factor for CSH when comparing the prevalence of children with (47.3%) to those without (39.2%)
[4].
Family history may play a role in CSH. In fact, Ziers et al.
[6] found that children had a significant increased risk for CSH when the mother (odds ratio [OR] 10.7) or father (odds ratio [OR] 8.4) had CSH
[10]. Other headaches in parents were not significantly associated with CSH in students
[6].
2. Clinical Features
2.1. Type of Pain, Intensity, and Frequency
CSH is characterized by pain described as sharp, intense, and stabbing
[1][9][11][12]. However, throbbing pain is also reported in patients with a previous history of migraine
[1][7].
The intensity of CSH is referred to mainly as “mild”
[4] and “moderate”
[1], and is less commonly reported as a headache of “severe intensity”
[1][4].
Zierz et al.
[6] focused on the frequency of recurrence of CSH with respect to stimulus exposure in a group of students by using a self-administered questionnaire: 21% of cases felt pain every time they were exposed to a cold stimulus, 14% every second to fourth time, and 65% rarely. Differently, Fuh et al.
[4] found that 1.4% of their student population always referred CSH after a cold stimulus, 4.3% often, 37.8% sometimes, and 56.6% rarely. Therefore, there are no homogenous findings regarding the frequency of CSH recurrence in the pediatric population.
2.2. Localization
According to the IHS classification, the localization of CSH is typically bilateral and mid-frontal, although the pain can be temporal, frontal, or retro-orbital (especially in CSH due to ingestion or inhalation). It can also be unilateral, lateralized to the side of the usual migraine headaches in those who have unilateral headaches
[11].
In studies on pediatric populations, the localization of CSH is more common in front of the vertex than behind it. Selekler
[9] conducted a cold test for an SH sufferers group. He found that the distribution areas in the head of SH are different from the distribution of those of CSH, and this difference was statistically significant. In particular, 45% of SH cases usually had pain in front of the vertex and 55% behind the vertex; after the cold test, 94% of cases reported pain in front of the vertex and/or on the vertex and 6% behind the vertex.
Specifically, the frontal area of the head
[13], the frontotemporal
[12], and the temporal one
[4][7] are identified as predominant localizations of pain. In a minority of cases, occipital and parietal localizations have been reported
[1][4][7]. The pain is mostly bilateral
[1][4][6], but in CSH attributed to ingestion or inhalation of a cold stimulus, the pain can be unilateral to the side of the stimulus on the palate
[8].
In the study conducted by Selekler et al. in 2004
[7], the pain induced by ice was seen most frequently in the temporal areas in migraineurs (57.1%) than in TTH patients (25%) and it has often been referred to as the part of the head afflicted by the patient’s customary headache. This difference between the groups was statistically significant.
Zierz A.M. et al.
[6] found that bilateral CSH was more frequent than unilateral in students without other previous headaches (61 vs. 39%), while for students with other headaches, bilateral and unilateral CSH were distributed equally (49 vs. 51%).
2.3. Latency after the Stimulus and Duration of the Headache
The onset of the pain is documented as an early onset
[8], typically within 20–30 s after the cold stimulus
[1][4][7], but also frequently between 30 and 60–70 s
[1][4][5][6][7]. However, De Oliveira et al.
[1] found that 41% of the population with CSH started to feel pain between 1 and 8 min after the stimulus.
Concerning the headache duration, the diagnostic criteria define a short-lasting pain, ranging from a few seconds to a few minutes, with resolution within 10 min after the removal of the stimulus for ingestion or inhalation headaches, and within 30 min after the removal of the stimulus for external applications of cold stimulus headaches
[11].
Accordingly, the researchers found in all studies a short-lasting duration of the headache
[8] ranging from 30 s–1 min to 5 min
[1][4][6][12]. The short duration of the headache could be explained by the fact that the available studies on the pediatric populations deal with headaches attributed only to the ingestion of a cold stimulus, and not to an external application.
D. Mitchell
[13] reported a case of CSH with other associated symptoms, such as feeling sick and having difficulty seeing, after a consecutive consumption of three large ice creams. In this case, the duration of all symptoms was about an hour, but the duration of the headache was not specified.
2.4. Associated Symptoms
The presence of a cold stimulus applied on the head or over the palate and/or posterior pharyngeal wall could produce other symptoms in addition to headaches.
In the literature, lacrimation, rhinorrhea, conjunctival injection, flashing light dots, and loss of sensitivity in the face have been described in CSH cases
[14][15].
For CSH due to ingestion, a history of cold-induced toothache is more common than one of the cold-induced headaches, and palatal or pharyngeal application of ice cream provokes toothache more frequently than headaches
[8].
3. Pathogenetic Mechanisms
The pathogenetic mechanism of CSH is not completely understood yet. Local and cerebral vascular changes and direct stimulation of cold receptors are the two main hypotheses
[10].
In CSH due to ingestion of the cold stimulus, when the palate and/or posterior pharyngeal wall is exposed to a cold substance, this substance may trigger rapid constriction and dilation of vessels with activation of nociceptors of the vessel wall
[10].
H. Özyürek et al. measured the middle cerebral arterial (MCA) flow velocities before and while eating ice cream in two children: one with CSH, and one without it. They found a slight decrease in flow velocities in both the child with CSH and the healthy child
[12]. In another study conducted on adults, the MCA blood flow velocity was measured while eating ice cream: a decrease in the mean flow velocities was found in subjects who developed a headache, while no changes were observed in those without a headache. Therefore, it is postulated that a decrease in cerebral blood flow is probably secondary to vasoconstriction, which might be important in the development of ice-cream headaches
[16][17]. These studies did not explain how cerebral blood flow is mediated intracranially, other than it is hypothesized as a possible mechanism of an overreaction of a vasogenic reflex responding to a small drop in temperature of the carotid blood or a reflex response triggered by the sensation of cold in the palate or oropharynx
[17].
According to Hensel et al.
[18][19], instead, patients with a headache attack provoked by ice water ingestion had higher MCA mean flow velocity rates than those without headaches. Additionally, patients with a positive ice-cream headache history but negative headache provocation had a moderate mean flow velocity increase. They explain the mechanism of increased cerebral blood flow velocity by a reduction in cerebrovascular resistance secondary to trigeminal-parasympathetic activation. In addition, Mages et al. pointed out that lacrimation occurring during CSH indicates that the trigeminal-autonomic reflex participates in CSH
[14].
Burkhart et al. argued that CSH is felt to be a cutaneous sensory response; a reflex response triggered by the sensation of cold in the palate or oropharynx
[20].
In line with this, CSH triggered by direct stimulation to the palate (innervated by the trigeminal nerve) may differ from the additional stimulation from the pharynx and esophagus (innervated by the glossopharyngeal and vagus nerve) when swallowing
[14]. Therefore, the differences in CSH symptoms could be related to which cranial nerves are activated.
CSH is more common in migraineurs and is often referred to as the part of the head afflicted by the patient’s customary headache. This fact could suggest that there may be segmental disinhibition of central pain pathways in migraineurs, responsible for undue susceptibility to an afferent volley of impulses from the excitation of cold receptors in the oropharynx
[9]. Another explanation is that a specific portion of trigeminal pathways may be activated as a reflex for seconds or minutes by a sudden cooling of the pharynx or may discharge migraine headaches for hours. This suggests that hyperexcitability of trigeminal pathways persists between migraine attacks and that periodic discharge of these pathways could initiate migraine headaches
[7].
4. Treatment
There is no specific treatment for CSH, because of its short-lasting duration. Nevertheless, the triggering factors can be avoided.
For “ice-cream headache”, some authors suggested that the triggering factors (such as ice cream, ice water, and icy food) could be ingested very slowly, with attempts to minimize rapid exposure of the cold substance to the palate and to the posterior pharynx
[5]. Nevertheless, Fuh J. et al.
[4] found in their study that the intensity and duration of ice-cream headaches were not different among the students who abstained, decreased, or continued to eat ice cream during a cold test. In addition, they documented that younger students were more likely to decrease or abstain from ice cream because of headaches (from 46.6% at 13 yr. to 33.7% at 15 yr.).
Different studies suggest a different solution depending on the type of stimulus, for example: (a) for CSH due to diving, they suggested minimizing the exposure to cold water and therefore the pain by wearing a neoprene hood
[21]; (b) for CSH due to cryotherapy, it is suggested that 1 min of rubbing the face along the distribution of the nerves and vessels before the treatment reduces their hyperexcitability and could eliminate the pain-producing discharges in certain branches of the trigeminal nerve
[20].
This entry is adapted from the peer-reviewed paper 10.3390/life13040973