Jacopo Fantini

SUNCT-like syndrome attributed to varicella-zoster virus meningoencephalitis

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare trigeminal autonomic cephalalgia (TAC) characterized by strictly unilateral neuralgiform paroxysmal orbital, supraorbital or temporal stabbing pain of 1–600 s duration, and accompanied by ipsilateral cranial autonomic features, such as lacrimation and conjunctival injection [1]. Secondary causes of SUNCT-like attacks have been increasingly recognized, particularly lesions involving the posterior fossa and the pituitary gland or single reports associated to viral meningitis [2–4]. Here we report the first case of SUNCT-like syndrome secondary to varicellazoster virus (VZV) meningoencephalitis with microbiological and histological confirmation. A 72-year-old male suffering from diabetes and hypertension, with no past history of headache, was admitted to our institution owing to acute development of fever and persistent, pressing headache affecting the right temporal region, without cranial autonomic features or neurovegetative symptoms. Physical examination and brain computed tomography at the admission were unremarkable. White blood cells count was 12.480/mm (89 % neutrophils) and serum C-reactive protein level 42.6 mg/L. Initially the patient was treated with cefotaxime and sodium dipyrone but after a few days he started to complain of stereotyped, unilateral severe headache attacks in the right forehead, with sharp, lancinating pain associated with conjunctival injections and intense dacryorrhoea. Pain paroxysm duration ranged from 10 to 60 s. Rarely, attacks followed his neck motion. Pain paroxysms occurred from 20 to 40 times per day. Common analgesics, including indomethacin, and prednisone were ineffective. Introduction of gabapentin 900 mg/day progressively decreased the frequency of attacks to 1–2/day. One week later, high fever, nausea and right facial nerve palsy suddenly ensued. A brain magnetic resonance imaging (MRI) was performed, which showed unspecific scattered white matter gliotic areas. Blood tests revealed IgM and IgG antibody titers against VZV. A lumbar puncture demonstrated: 80 leukocytes/mm (100 % monocytes), protein level 78 mg/dL, and glucose level 113 mg/dL. Cerebrospinal fluid quantitative polymerase chain reaction (PCR) was positive for VZV DNA and a 10-day intravenous 30 mg/kg/ day acyclovir cycle was administered. Afterwards, a second lumbar puncture showed a reduction in leukocyte count and VZV PCR was negative. Two weeks later, the patient developed left sided hemiplegia, dysphagia, and dysphonia. Brain MRI detected right paramedian ponto-mesencephalic and left hippocampal acute ischemic lesions and a right ponto-bulbar meningeal thickening. Computed tomographic angiography of intraand extracranial vessels and transesophageal echocardiogram excluded large vessel and cardiologic aetiologies. Anti-platelet treatment with acetylsalicylic acid 300 mg/day was started. After 10 days of apparently stable general conditions, the patient suddenly died. Necropsy showed multiple foci of lymphocytic meningoencephalitis, a right pontine cavitated infarction and myocarditis. Fatal arrhythmia secondary to myocarditis was considered the cause of death. The International Classification of Headache Disorders indicates that a new headache occurring in relation to an infection should be coded as a secondary headache induced by that infection. A. Granato M. Belluzzo J. Fantini (&) M. Zorzon N. Koscica Department of Medical, Surgical and Health Sciences, Neurologic Clinic, Headache Centre, University of Trieste, Trieste, Italy e-mail: jacopo.fantini@gmail.com

How far away from having an effective treatment option for progressive multiple sclerosis are we

The availability of numerous disease-modifying drugs for relapsing-remitting (RR) multiple sclerosis (MS) is one of the greatest successes of the last 30 years of neurology history. Unfortunately, this story has a dark side, represented by progressive forms of the disease, still without an effective treatment. A total of 15% of MS patients show a progression form onset, while secondary progression is developed by about 80–90% of RR patients after long-standing disease. Clinical differences between primary progressive (PP) and secondary progressive (SP) forms are well known; nevertheless, these two entities share so many pathological mechanisms and characteristics [1] that seem warranted considering them as part of the same spectrum. The prominence of neurodegeneration over inflammation, with microglial activation, chronic axonal demyelination mediated by oxidative injury, and axonal mitochondrial dysfunction, translates in high disability levels that have deep consequences on patients’ quality of life and give rise to significant familiar, social, and economic burden. It is easy to understand why finding an answer to such a disabling disease represents a priority for scientific community.

Can We Speak of Lack of Habituation in Visual Snow

We read with interest the article by Unal-Cevik et al reporting a case of visual snow (VS) in a patient with a pre-existing migraine with aura (MWA). The authors recorded a subjective improvement of the patient’s VS symptoms after treatment with lamotrigine (LTG) and the effect was objectively assessed by the evaluation of repetitive pattern reversal visual evoked potentials (rVEPs). At baseline, the patient showed a potentiation response, defined as an increase in amplitude of rVEPs between the first and the last block of 100 stimuli, which partially recovered after treatment with LTG. In our opinion, the authors proposed a valid and feasible approach to explore the pathophysiology and therapeutic responsiveness of VS, but we feel that there are some methodological concerns that need to be discussed. First, as the authors correctly stated, “the consequences of cortical hyperexcitability due to comorbid migraine cannot be eliminated,” and this is a crucial point. Indeed the lack of habituation, or even potentiation, is recognized as a characteristic feature of interictal migraine, especially with aura and in VS a high prevalence migraine (30-60%) has already been reported. Therefore, we believe that the potentiation effect on rVEPs that the authors described cannot be unequivocally attributed to VS but rather seems to be related to the underlying MWA. We suggest that this finding should be more prudently confirmed on a larger number of patients, possibly trying to make a distinction between the subgroups of VS with or without comorbid migraine. If we were able to unveil some differences between those two groups we could ascertain whether a state of cortical hyperexcitability is truly VS related or just migraine related. Second, taking in to account the ongoing controversy about the validity of rVEPs habituation paradigms in migraine, we suggest that in a single subject study design the reduction of the potentiation effect after LTG treatment should be interpreted with caution. Changes of few millivolts (mV) between blocks could be explained, for example, by an intrinsic sampling variability, fluctuations in subject’s attention, or concomitant treatments. Perhaps if we were able to demonstrate the restoration of a physiologic habituation response – at a group level – we could have a more solid clue to affirm that a normalization of cortical excitability levels has occurred. Third, LTG has been proven to be effective in the prophylaxis of MWA. Its mechanism of action is still only partially understood and presumably involves the modulation of the glutamatergic transmission, which may be altered in VS as well as in MWA. Some patients with VS reported some improvement of VS after receiving LTG but it is still unclear whether LTG ameliorates VS acting on both VS and migraine pathophysiology or if it has a specific effect on VS. In this specific case, a concomitant effect on MWA should be hypothesized and this observation is supported by the patient’s objective reduction of headache attacks and by the evidence of an increased likelihood of having additional visual symptoms and tinnitus in VS with comorbid migraine. We reaffirm that VS should be considered as a distinct clinical entity although the overlaps with migraine pathophysiology are relevant. Neurophysiological investigations are safe, non-invasive, Abbreviations: LTG, lamotrigine; mV, millivolts; MWA, migraine with aura; rVEPs, repetitive visual evoked potentials; VS, visual snow. Conflict of Interest: The authors declare that there is no conflict of interest.

Hemicrania continua with visual aura successfully treated with a combination of indomethacin and topiramate

Hemicrania continua (HC) is a rare syndrome characterized by continuous unilateral pain of moderate intensity with superimposed exacerbations of severe pain with migrainous (throbbing quality, presence of abnormal sensitivity to light and sound, nausea and vomiting) and autonomic features (conjunctival injection, lacrimation, nasal congestion, rhinorrhoea, ptosis, or eyelid oedema). HC is peculiarly responsive to indomethacin. Visual or sensitive auras associated with HC have been reported as very rare atypical feature. Here, we report a case of HC with visual aura successfully treated with a combination of indomethacin and topiramate. A 31-year old Caucasian man, with unremarkable medical history, who had never suffered from headache in his life was referred to our Headache Centre. He had no familiarity for headache or other relevant diseases. He complained of daily, strictly unilateral, right-sided headache which started 1 month before. Headache was continuous, of moderate intensity, localized in the right temporal-orbital region, radiating to the ipsilateral hemiface, associated with two exacerbations per day of severe throbbing pain lasting about 2 h, that was accompanied with nausea, photophobia and ipsilateral autonomic symptoms, such as ptosis, conjunctival injection and tearing. The exacerbations were always preceded by gradual appearance of spots of flickering light in the peripheral right visual hemi-field, lasting about 10 min, then slowly disappearing. The patient took acetaminophen 1,000 mg p.o. and ibuprofen 600 mg p.o. with no effect. Physical examination during exacerbation revealed right eyelid oedema, partial ptosis, and conjunctival injection. Secondary headaches were excluded by head CT scan, CT angiography, and cerebral MRI. The diagnosis of probable HC with visual aura was made and the patient was treated with indomethacin 25 mg t.i.d. in association with topiramate, which was slowly titrated to the final dose of 75 mg/ day. Two months later, indomethacin was stopped, while topiramate was continued. Visual symptoms and the continuous moderate headache disappeared in a few days. In the first month of treatment, severe attacks with autonomic symptoms decreased to one per week. In the following 2 months, headache frequency decreased to one moderate attack per month. Subsequently, the patient had no recurrence of symptoms in the next 6-month period. He reported no therapy side effects. In the International Classification of Headache Disorders, HC has been recently included in the group of the trigeminal autonomic cephalalgias (TACs), on the basis that the pain is usually lateralized, and the cranial parasympathetic autonomic features are ipsilateral to the headache [1]. Brain imaging studies show significant overlaps between HC and the other TACs, in particular in relation to the activation of the posterior hypothalamic gray matter. The clinical and pathogenetic relationship between HC and migraine is rarely described and poorly understood. Our patient had typical HC and the aura met the International Headache Society criteria for migrainous aura [1], however, HC and migraine do not seem to be linked, since the patient had never suffered of headaches or experienced J. Fantini (&) N. Koscica M. Zorzon M. Belluzzo A. Granato Department of Medical, Surgical and Health Sciences, Neurologic Clinic, Headache Centre, University of Trieste, Strada di Fiume 447, 34149 Trieste, Italy e-mail: jacopo.fantini@gmail.com

Case Report: Coexistence of SUNCT and Hypnic Headache in the Same Patient

Short‐lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and hypnic headache (HH) are two exceedingly rare and distinctly classified primary headaches. The hypothalamus seems to be a crucial region involved in the pathophysiology of both conditions, but no cases of SUNCT and HH co‐occurrence have been described so far.

The reappearance of hemiplegic cluster headaches: A case report and review of the literature

Cluster headache (CH) is a rare and severe syndrome characterized by the recurrence of unilateral pain attacks, of short duration (15-180min), and associated with ipsilateral cranial autonomic symptoms. Although, not formally included in the International Classification of Headache Disorders, hemiplegic cluster headache (HCH) is an even more rare subtype of CH in which typical attacks can be accompanied by visual, sensory, and/or aphasic migrainous auras that have a variable propensity to evolve in reversible hemi-motor symptoms. After its first description in 2002, only few cases of HCH have been reported and many open questions about its prevalence and pathophysiology still need to be addressed. We describe a case of a 41-year old male that fulfilled the ICHD criteria for episodic CH who experienced atypical attacks characterized by concomitant acute onset of sensory aura, aphasia and hemi-motor symptoms. We also provide a concise review of the available literature and discuss the prevalence and the possible pathogenesis of CH with hemiplegic features.

Avoiding misdiagnosis: cystic calcified brain metastases of uterine cervical cancer mimicking neurocysticercosis

The radiological finding of multiple calcified brain lesions is atypical for brain metastases and in absence of a clear evidence of disseminated neoplastic disease the differential diagnosis may be difficult. Calcified brain metastases (CBM) are rarely encountered in clinical practice and they mostly arise from lung, breast and gastrointestinal primitive tumours. Only one case of uterine cervical carcinoma (UCC) with CBM has been reported so far. We describe the case of a 41-year-old Caucasian woman with a history of hysterectomy and bilateral salpingo-oophorectomy for UCC 3 years prior to observation and no evidence of neoplastic recurrence that developed cystic CBM. Owing to their peculiar radiological appearance, lesions were initially misidentified as neurocysticercosis, the most common parasitic infection of the central nervous system. We offer the reader some important teaching points for the differential diagnosis and discuss the rarity of our case.

O031. Physiotherapy treatment in chronic tension-type headache: an ongoing study

Methods We enrolled patients with cTTH (ICHD3-beta criteria) attending the Headache Centre of Trieste who preferred not to take pharmacological treatment. Patients were prophylaxis free in the last 3 months and were asked to fill in an ad hoc diary in a three-month baseline period. The physiotherapy group (PhG) underwent a threemonth combined protocol of postural advice, exercises and manual therapy. Intensity (NRS), frequency and duration of pain were analysed with GraphPad InStat 3.06. Cervical range of motion (CROM) was evaluated with a headgear provided with goniometer and spirit level. Neck pain was studied with the Neck Pain and Disability Scale I (NPDS) Questionnaire. A control group (CG) treated with amitriptyline for three months was compared with PhG.

EHMTI-0356. Hypnic headache in a patient with sunct: case report

Case description A 49 year-old healthy woman developed two rare primary headaches. At the age of 23 she experienced single painful stabs in the left orbital-temporal region lasting few seconds, every 6-7 months. In the subsequent 3 years the single pain attacks became longer in duration, they were accompanied by ipsilateral conjunctival injection and tearing, and frequency increased to 20 episodes/day for 5-10 consecutive days, with pain free intervals of 6-7 months. Brain MRI ruled out secondary headaches. In the following years, active periods reached the duration of 3 consecutive months, with remissions of 1 month. Six years ago, the patient was evaluated in our Headache Centre, a SUNCT (short-lasting unilateral headache with conjunctival injection and tearing) was diagnosed, and lamotrigine 100 mg/ day was effectively administered. The pain recurred after therapy discontinuation, so she was forced to continue lamotrigine. She was pain-free for further 2 years, then she began suffering from a new occipital bilateral pressing pain, moderate, sometimes with nausea, presenting exclusively during sleep and causing awakening. Attacks occurred usually at 4:00-5.30 a.m., lasted about 60 minutes and gradually became near daily. A diagnosis of hypnic headache was made and the patient was treated with caffeine 30 minutes before sleeping, without headache recurrence.