Conflict of Interest: None
Thunderclap Headache as Initial Manifestation of Vogt-Koyanagi-Harada Disease
Article first published online: 14 SEP 2007
Headache: The Journal of Head and Face Pain
Volume 48, Issue 1, pages 153–155, January 2008
How to Cite
Cho, J. H., Ahn, J. Y., Byeon, S. H. and Huh, J. S. (2008), Thunderclap Headache as Initial Manifestation of Vogt-Koyanagi-Harada Disease. Headache: The Journal of Head and Face Pain, 48: 153–155. doi: 10.1111/j.1526-4610.2007.00913.x
- Issue published online: 20 DEC 2007
- Article first published online: 14 SEP 2007
- Accepted for publication June 29, 2007.
- thunderclap headache;
- Vogt-Koyanagi-Harada disease;
- pituitary apoplexy
A 59-year-old Asian woman suffered from a TCH followed by sudden, binocular blurred vision, mimicking pituitary apoplexy. The diagnostic workup (including fluorescein angiography, MRI of the brain, and CSF analysis) showed severe optic disc swelling and dye leakage of multiple faint hyperfluorescent spots at retinal pigment epithelium level, diffuse pachymeningeal hypertrophy, and monocytic pleocytosis, respectively. VKH disease should be considered in the differential diagnosis of patients presenting with a TCH followed by sudden, bilateral decreased visual acuity.
magnetic resonance imaging
non-steroidal anti-inflammatory drugs
optic coherence tomography
Rathke's cleft cyst
A 59-year-old Asian woman suffered from a TCH followed by sudden, binocular blurred vision, mimicking pituitary apoplexy. The diagnostic workup (including fluorescein angiography, MRI of the brain, and CSF analysis) showed severe optic disc swelling and dye leakage of multiple faint hyperfluorescent spots at retinal pigment epithelium level, diffuse pachymeningeal hypertrophy, and monocytic pleocytosis, respectively. All of these results met the diagnostic criteria for VKH disease. The presenting symptoms and signs resolved over a 4-day period following high-dose steroid therapy. VKH disease should be considered in the differential diagnosis of patients presenting with a TCH followed by sudden, bilateral decreased visual acuity.
A TCH is an acute and severe headache that reaches its maximum intensity at onset, and is likened to a “clap of thunder.” Although the term was first used to describe this type of headache as a presentation of an unruptured intracranial aneurysm,1 a number of other disorders are known to present with a TCH, including subarachnoid hemorrhage, cerebral venous sinus thrombosis, cervical artery dissection, spontaneous intracranial hypotension, pituitary apoplexy, retroclival hematoma, acute hypertensive crisis, reversible cerebral vasoconstriction syndrome, third ventricle colloid cyst, and intracranial infection.2 In all cases, an intracranial disease should always be ruled out by appropriate investigations. In some patients with a TCH and bilateral visual symptoms, the differential diagnosis should include pituitary apoplexy, retroclival hematoma, and acute hypertensive crisis.
VKH disease is a granulomatous inflammatory disorder affecting the eyes, auditory system, meninges, and skin. The target of attack seems to be antigens associated with melanocytes.3 VKH patients complain of neurological symptoms that are quickly followed by a decrease in vision (caused by choroiditis, frequently with exudative retinal detachments).4 Headache is present in 67% of VKH patients,5 but it is usually not the leading symptom. Here, we report a unique case and discuss pertinent diagnostic and therapeutic issues regarding a VKH patient who presented with a severe headache of sudden onset, followed by ocular symptoms.
A normotensive 59-year-old Asian woman with a past medical history significant for a 10-year duration of long-standing migraines presented to our emergency department with a 3 day history of a different type of headache, primarily fronto-parietal and bilateral. The patient had no history of head trauma or surgery. She characterized her long-standing migraines as a throbbing headache on the right temporal region with photophobia, which had been properly controlled in the past by NSAIDs, antidepressants, and minor tranquilizers. In this case, the new severe nonpulsating pain began suddenly at night, reaching its maximum in intensity at onset. The headache was worsened by movement, coughing, and straining, and was accompanied by photophobia, nausea, and vomiting. Blurred vision, which had begun following the first symptom, had been getting worse up until her hospital visit. The patient had also assumed it was just a stronger version of her previous migraine attacks and neglected her ocular problems, owing to never having experienced severe headache.
The patient was afebrile and didn't show cutaneous manifestations like alopecia, poliosis, and vitiligo on physical examination. Neurological examination revealed nonspecific abnormalities, with the exception of neck stiffness, somnolence, and blurred vision. Her pupillary reflex and hearing were intact. On ophthalmologic examination, her visual acuity was decreased (left, 20/125; right, 20/400) and fundus photography after pupillary dilatation showed severe optic disc swelling with mild retinal pigment epithelium mottling in both eyes. Slit lamp examination demonstrated many cells in anterior chamber of both eyes, compatible with iridocyclitis. Fluorescein angiography disclosed severe optic disc swelling and dye leakage of multiple faint hyperfluorescent spots at retinal pigment epithelilum level (Fig. 1). A head CT scan was interpreted as normal. Orbital MRI revealed diffuse pachymeningeal hypertrophy and diffuse choroidal thickening bilaterally, with strong enhancement (Fig. 2A). Whole brain MRI showed Rathke's cleft cyst (RCC), which was determined to be too small to compress the optic nerve and increase intracranial pressure (Fig. 2B). On the third day, CSF findings were as follows: red blood cells, 2; white blood cells, 48 (100% monocyte); glucose level, 61 mg/dL, protein level, 46.1 mg/dL. Based upon the ophthalmological and neurological findings, VKH disease was diagnosed. The patient received systemic methylprednisolone (1000 mg/day) for 3 days and was then switched to oral prednisolone (50 mg/day) as a maintenance dose for 1 month. On the fourth day, the headache resolved, and her visual acuity was normal. On the tenth day, a follow-up CSF analysis revealed no pleocytosis and a normal protein level (30.2 mg/dL). Oral steroids were tapered during the following 4 months. During a 12-month follow-up, there were 2 moderate ophthalmological relapses without the redeveloping TCH, which resolved after local steroid treatment.
This patient suffered an unusual illness commencing with a TCH with bilateral decreased visual acuity. A TCH is a very rare presentation of VKH disease. Usually, VKH patients present soon after the onset of the disease, and may complain of auditory and neurologic manifestations followed by the onset of decreased vision (with exudative retinal detachments and optic disk hyperemia). When the patient presents at disease onset, manifestations of meningeal involvement include those findings commonly referred to as “meningismus”: malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back, or a combination of these features. Because many patients with uveitis will admit to headache upon direct questioning, headache alone is insufficient to fulfill VKH disease diagnostic criteria. In these clinical settings, documentation of cerebrospinal fluid pleocytosis is needed to establish the diagnosis.6 The new diagnostic criteria are based on the concept that VKH disease is a single entity with very different clinical manifestations depending on the stage at which the patient is examined.4 The patient with VKH disease does not manifest all possible signs of the condition.5 For this reason, the criteria are designed to allow for division of “definite” VKH disease into “complete” and “incomplete” categories based on the spectrum of manifestations seen. The presenting manifestations of our patient fulfilled the criteria of “incomplete” VKH disease because of no integumentary findings.
If a TCH from aseptic meningoencephalitis occurs acutely and is combined with the ocular symptoms of VKH disease (as in this case), the patient's symptoms may be interpreted as the onset of pituitary apoplexy. Pituitary apoplexy is an uncommon clinical syndrome usually characterized by acute headache, ophthalmoplegia, diminished visual acuity, and altered mental status in any combinations, and is caused by the sudden infarction or hemorrhage of the pituitary gland (invariably harboring an adenoma). Brain MRI in our case showed RCC on the pituitary fossa. Headaches, particularly frontal episodic headaches, are a common and characteristic symptom of RCC and are more frequent in patients with high signal-intensity cyst on T1-weighted MRI image.7 Some patients presenting with sudden episodic headaches may mimic those with pituitary apoplexy.7 In a retrospective review of our case, patient had low signal-intensity cyst on T1 weighted MRI image with frontoparietal-area headache not localized only to the frontal area. Moreover, the patient's headache showed rapid response to the steroid therapy in keeping step with amelioration of other manifestations of VKH. From these reasons, we suspected that the presenting TCH in our case did not correlate with the RCC. Careful ophthalmologic evaluation and CSF analysis are mandatory for diagnosis in this unusual clinical situation.
The treatment of choice varies in different parts of the world. Usually, the aim of therapy for VKH disease is to suppress the initial intraocular inflammation with early and aggressive use of systemic corticosteroids. Cytotoxic and/or immunosuppressive agents are needed in refractory cases.8 Systemic corticosteroid therapy is generally accepted as the standard of care for initial therapy, but there is no consensus regarding the optimal route and dosage of administration. In our case, meningoencephalic and visual symptoms dramatically resolved following high-dose corticosteroid therapy.
In conclusion, VKH disease should be considered in the differential diagnosis of patients presenting with a TCH followed by sudden decreased bilateral visual acuity. Early diagnosis and prompt treatment are the keys to success.