International Headache Society (IHS) code and diagnosis: 4.8 New daily-persistent headache (NDPH)

World Health Organization (WHO) code and diagnosis: G44.2 New daily-persistent headache (NDPH)

Short description: NDPH is a headache that is daily and unremitting from or almost from the moment of onset, typically in individuals without a prior headache history. NDPH may take either of two subforms: a self-limiting subform, which typically resolves without therapy within several months, and a refractory subform, which is resistant to aggressive treatment.

Previously used terms: chronic benign daily headache

New daily persistent headache (NDPH) was first described by Vanast in 1986 (14) as a benign form of chronic daily headache (CDH) that improved without therapy. In the headache specialist’s office NDPH is anything but benign and is felt to be one of the most treatment refractory of all headache conditions. NDPH has now been included in the revised International Classification of Headache Disorders (ICHD-II) as a separate entity (Table 103-1). As there have been only a few studies looking at the clinical characteristics and differential diagnosis of NDPH, these consensus criteria may need to be modified as more data on this syndrome are published. It appears that there are two subtypes of NDPH: a self-limited form, which typically goes away within several months without any therapy, and a refractory form, which remains resistant even to aggressive treatment. NDPH should not be difficult to diagnose in a physician’s office based on its profile of headache onset, which should be very precise, and most patients can pinpoint the exact date and some even the exact time their headache began.

Even though NDPH has probably been around for centuries, it has only recently been considered a separate diagnosis from chronic tension-type headache, medication-overuse headache, hemicrania continua, or chronic migraine (12). The prevalence of CDH from population-based studies in the United States, Asia, and Europe is about 4 to 5% (12). In those epidemiologic investigations, NDPH was rarely stratified out from the data and the used definition of NDPH varied slightly. Several studies have documented the prevalence of NDPH: Castillo et al. reported that 4.7% of a 2252 population sample from Spain have CDH, of which 0.1% had NDPH (2). Bigal et al.(1) noted that 10.8% of 638 patients with CDH in a headache specialty clinic had NDPH, while Koenig et al. found that 13% of a pediatric CDH population, surveyed from selected pediatric headache specialty clinics, had NDPH (4).

There are only three case series in the literature dedicated to describing the clinical characteristics of NDPH. Vanast(14) noted a female predominance in 45 patients (26 women and 19 men) and an earlier age of onset in women. Seventy-two percent of the patients stated the pain of NDPH was constant. Pain location was temporal in 9 of 45 patients, temporal plus other areas in 14 patients, occipital and extra sites in 20 patients, and holocranial in 5 patients. “Migrainous” associated symptoms were noted in a large percentage: nausea 55%, vomiting 12%, photophobia 34%, and phonophobia 37%.

Li and Rozen (5) published the largest study to date describing the syndrome of NDPH based on a retrospective chart review. Female:male gender ratio was 2.5:1. Age of onset ranged from 12 to 78 years with a peak age of onset in the second and third decade in women and the fifth decade in men. Eighty-two percent of patients were able to
pinpoint the exact day their headache started. Headache occurred in relation to an infection or flulike illness in 30%, extracranial surgery in 12%, and a stressful life event in 12%. A prior headache history was found in 38% (episodic migraine 19%, episodic tension-type headache 2%, and unspecific headache 17%). In 79%, the pain was continuous throughout the day. Pain intensity was moderate in 61%, while 21% experienced severe pain. Headache location was bilateral in 64% while almost 60% had some pain localized to the occipital-nuchal region, 44% experienced retro-orbital pain, and 18% holocranial pain. Headache quality was described as a throbbing sensation in 55% and pressurelike in 54%. Nausea occurred in 68%, photophobia in 66%, phonophobia in 61%, vomiting in 23%, osmophobia in 23%, and vertigo in 11%. Aura-type symptoms, including visual photopsias and seeing zigzag lines, were present in a small number of patients. Family history of headache was documented in 29%. Takase et al. (13) looked at the clinical characteristics of NDPH in 30 Japanese patients. There was a male predominance (17 men and 13 women), and age of onset ranged from 13 to 73 years. Headache onset was associated with a stressful life event in 20%, while the remainder could not identify a probable cause. Headache intensity was severe in all patients. Headache quality was a pressure or tightening sensation in 73%, pulsating in 10%, and both pressing and pulsating in 5%. Associated symptoms were rare with mild nausea occurring in 10 patients while only 1 patient had photophobia.