Two Unusual Cases of Headache: Superficial Siderosis Coexisting with Migraine.

We hereby report two cases who had a prolonged history of headache which had characteristics of migraine; but subsequently, they developed focal neurological deficit to warrant detailed investigations, which revealed superficial siderosis. Coexistence of migraine and superficial siderosis is unique.

Patient 1- A 25-year-old female presented with a history of episodic headache, occurring 6–10 times per year, lasting 6–24 hours, mostly holoranial, pulsating type, moderate in intensity (5/10 in visual analog scale or VAS), and associated with photophobia, phonophobia, nausea, and occasionally vomiting for the last 14 years. She used to take acetaminophen for her headache. In the last 4 months, her headache had become severe in intensity (8/10 in VAS), and the frequency had increased to 3–4 times per month. Two days before attending our institute, she developed double vision while looking towards the right along with headache and vomiting. Headache intensity reached its peak after 1 h of onset. The clinical examination revealed right abducens palsy and gaze-evoked nystagmus while looking towards the left. Her brain MRI revealed superficial siderosis involving bilateral cerebellar sulci and fissures and right Sylvian fissure [Figure 1]. The MRI of the whole spine and cerebral angiography were normal. The CSF analysis and coagulation profile were normal. We prescribed her propranolol 40 mg/day PO and naproxen 500 mg PO as and when required. After 1 week, her headache and diplopia subsided, but the nystagmus persisted. Her headache reappeared after 1 month, and we increased the dose of propranolol to 60 mg/day. As there was no improvement, we stopped propranolol and started amitriptyline 25 mg/day PO along with deferiprone 500 mg PO twice daily. After 10 months, she became headache-free.

Figure 1

Brain MRI of both the patients. Patient 1 (left): T1 (a, b) and T2 (c, d) hypointensities with low signal in GRE (e, f) and SWI (g, h) sequences in the right Sylvian fissure and cerebellar sulci. Patient 2 (right): T1 (a, b) and T2 (c, d, e) hypointensities with low signal in GRE (f, g, h) sequence in the left parietal and cerebellar sulci

Patient 2- A 20-year-old female presented with a history of episodic headache, occurring 2–5 times per year, lasting 4–6 hours, mostly hemicranial (alternating sides), pulsating type, moderate in intensity (4/10 in VAS), and associated with photophobia, phonophobia, and occasionally nausea for the last 12 years. Her headache used to subside with rest, and she seldom needed analgesics. Six years ago, she developed an episode of headache associated with double vision while looking towards the left. Headache intensity reached its peak after 3–4 h, and she was admitted to the local hospital where a plain CT scan of the brain was done, which was normal. Headache and diplopia subsided after 2–3 days. Following this episode, she took propranolol 40 mg/day PO for 4 years. Three months ago her headache reappeared, and she started taking propranolol again, but there was no improvement. Her clinical examination was normal. The MRI of the brain revealed superficial siderosis involving cerebellar and cerebral sulci, more in the left parietal region [Figure 2]. The coagulation profile and CSF analysis were normal. The MRI of the whole spine and cerebral angiography were normal. We prescribed her amitriptyline 25 mg/day PO. Her headache subsided after 1 week, and she is symptom-free since then.

Figure 2

Cerebral angiography of both patients. Patient 1 (left): Normally visualized left internal carotid artery (ICA), middle cerebral artery (MCA), anterior cerebral artery (ACA) and their branches (a, b), right ICA, MCA, ACA and their branches (c, d), left vertebral artery, basilar artery, and both PCA (e, f); Normal venogram is seen in g and h. Patient 2 (right): Normally visualized right ICA, MCA, ACA and their branches (a, b), left ICA, MCA, ACA and their branches (c, d), left vertebral artery, basilar artery, and both PCA (e, f); Normal venogram with hypoplastic left transverse sinus is seen in g and h

Superficial siderosis is presumed to be caused by repeated small amounts of bleeding into the subarachnoid space resulting in iron and hemosiderin deposition in the leptomeningeal layers of the brain and the spinal cord.[12] It can occur following a head injury, subarachnoid hemorrhage (SAH), or any other intracranial hemorrhage and in reversible cerebral vasoconstriction syndrome (RCVS), primary CNS angiitis (PACNS), cerebral amyloid angiopathy (CAA), cavernoma, or coagulopathy.[1234] If no underlying cause is found, then it is labeled as idiopathic superficial siderosis.[1234] There are two principal patterns of superficial siderosis- cortical superficial siderosis (cSS) and infratentorial superficial siderosis (iSS).[2] cSS is restricted to the supratentorial region and can cause headache which is either acute in onset or episodic or thunderclap-like.[12345] iSS predominantly presents with sensorineural hearing loss, cerebellar ataxia, and/or myelopathy but supratentorial spread, and thereby headache can occur.[12] Iannaccone et al. have described a 33-year-old male diagnosed with superficial siderosis who experienced recurrent episodes of severe headache which were sudden in onset, stabbing, frontal in location, gradually increasing in severity, lasting 8–12 hours, and associated with nausea, vomiting, and photophobia since 8 years of age.[5] Migraine-like aura in the form of spreading paresthesia and/or positive visual phenomena with or without mild headache can occur in cSS associated with CAA, which is a disease of the elderly (>50 years of age).[6] Chowdhury et al. have described a 75-year-old male diagnosed with superficial siderosis without any evidence of CAA who presented with moderate intensity (6/10 in VAS), throbbing type, holocranial headache, associated with photophobia, phonophobia, vomiting, and dizziness for 2 years.[7] In this case, exacerbation of the headache by head movements, coughing and bending forward, and the presence of cognitive impairment, cerebellar signs, and sensorineural hearing loss suggested the diagnosis of a secondary headache.[7] However, a headache that has characteristics of migraine and no symptoms or signs to suggest any secondary cause has never been described in superficial siderosis. Both of our patients had a long-standing headache that fulfilled the International Classification of Headache Disorders (ICHD-3) criteria of migraine without aura.[6] However, they eventually developed abnormal neurological signs giving clues towards a secondary cause. There was no history of head injury, and MRI of the brain and spine did not show any evidence of cavernoma, CAA, SAH, or any other intracranial hemorrhage. A normal cerebral angiogram and normal CSF study make RCVS and PACNS unlikely. Both patients had a normal coagulation profile. Hence, our diagnosis was idiopathic superficial siderosis. As brain MRI was not done previously in both cases, it is not possible to determine whether superficial siderosis was present since the beginning, thereby mimicking migraine. In the absence of any published literature to support such a possibility and since in both the cases worsening of the pre-existing headache occurred in close temporal relation to the detection of superficial siderosis, we conclude that the previous headache was primary (migraine without aura) which was worsened by a new onset secondary headache attributable to superficial siderosis. To the best of our knowledge, literature falls short till date to unfurl such coexistence, which makes this unique association worthy of reporting.

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Conflicts of interest

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