CLIPPERS and its mimics: evaluation of new criteria for the diagnosis of CLIPPERS.

OBJECTIVE: To evaluate the accuracy of the recently proposed diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). METHODS: We enrolled 42 patients with hindbrain punctate and/or linear enhancements (<3 mm in diameter) and tested the CLIPPERS criteria. RESULTS: After a median follow-up of 50 months (IQR 25-82), 13 out of 42 patients were CLIPPERS-mimics: systemic and central nervous system lymphomas (n=7), primary central nervous system angiitis (n=4) and autoimmune gliopathies (n=2). The sensitivity and specificity of the CLIPPERS criteria were 93% and 69%, respectively. Nodular enhancement ( ≥ 3 mm in diameter), considered as a red flag in CLIPPERS criteria, was present in 4 out of 13 CLIPPERS-mimics but also in 2 out of 29 patients with CLIPPERS, explaining the lack of sensitivity. Four out of 13 CLIPPERS-mimics who initially met the CLIPPERS criteria displayed red flags at the second attack with a median time of 5.5 months (min 3, max 18), explaining the lack of specificity. One of these four patients had antimyelin oligodendrocyte glycoprotein antibodies, and the three remaining patients relapsed despite a daily dose of prednisone/prednisolone ≥ 30 mg and a biopsy targeting atypical enhancing lesions revealed a lymphoma. CONCLUSIONS: Our study highlights that (1) nodular enhancement should be considered more as an unusual finding than a red flag excluding the diagnosis of CLIPPERS; (2) red flags may occur up to 18 months after disease onset; (3) as opposed to CLIPPERS-mimics, no relapse occurs when the daily dose of prednisone/prednisolone is ≥ 30 mg; and (4) brain biopsy should target an atypical enhancing lesion when non-invasive investigations remain inconclusive.

Varicella-zoster meningovasculitis in a multiple sclerosis patient treated with natalizumab.

BACKGROUND: Natalizumab is associated with the occasional occurrence of progressive multifocal leukoencephalopathy (PML). While natalizumab-associated PML is well described in multiple sclerosis (MS) patients, herpes and other infections have rarely been reported. OBJECTIVE: To report a case of varicella-zoster (VZV) meningovasculitis in a MS patient treated with natalizumab. METHODS: Case report. RESULTS: A 48-year-old woman diagnosed with MS in treatment with natalizumab (Expanded Disability Status Scale (EDSS): 4.0). After 72 infusions, she complained of a holocraneal headache and a new unsteady gait with diplopia (EDSS: 5.0). A brain and spinal cord magnetic resonance (MR) scan showed a multifocal leptomeningeal enhancing nodular lesions and an angiography revealed irregularity of the proximal segments of cerebral arteries. Testing for VZV DNA by polymerase chain reaction (PCR) amplification was positive in cerebrospinal fluid. Treatment with endovenous acyclovir was started. After clinical improvement (EDSS: 4.5), treatment with natalizumab was restarted associated with oral acyclovir as prophylaxis. CONCLUSION: Neurologists should be aware of other possible neuroinfections besides PML in MS patients under natalizumab.

[Radiological and surgical characteristics of a case of recurring spinal hydatidosis]. / Caracteristicas radiologicas y quirurgicas de un caso de hidatidosis espinal recurrente.

INTRODUCTION: Hydatidosis is an illness that is still to be found in our setting. The lungs and liver are the organs most frequently involved and it rarely extends to bones. Involvement of the spine is infrequent, but it can give rise to a high degree of disability and may recur despite apparently complete excision. Here we report a case of recurring spinal hydatidosis with radiological and surgical particularities. CASE REPORT: Our case involves a 63-year-old male living in a rural area of the northern part of Spain's central plateau. The patient had a history of surgery for spinal hydatidosis and was on treatment with mebendazole. In the months before his visit, the patient had experienced difficulty in walking and urgency incontinence. The examination revealed paraparesis that predominantly affected the right-hand side and the patient required bilateral support to be able to walk. A dorso-lumbar magnetic resonance scan showed post-surgery changes with pedicular instrumentation added later in another centre, and a hydatid cyst with involvement of the body of the D10 vertebra, epidural extension and compression of the spinal cord. The patient was submitted to a surgical procedure involving the extraction of the system of fixation and full macroscopic excision of the cyst, with partial resection of the body of the vertebra. Initially the patient displayed motor improvement and treatment with mebendazole was reintroduced in high doses. CONCLUSIONS: Spinal hydatidosis is a rare condition that, in spite of correct surgical and medical treatment, often recurs. Magnetic resonance scans show characteristic 'honeycomb' images, which are sometimes large and have their origins in the body of the vertebra. Although successive surgical interventions can become increasingly more difficult because of old instrumentation or the conservation of compromised vertebral bodies, aggressive excision is indicated while the patient still has useful neurological functioning.

Epicrania fugax with backward radiation: clinical characteristics of nine new cases.

Epicrania fugax (EF) is a novel syndrome, described as a paroxysmal and brief head pain, starting in posterior cranial regions and rapidly spreading forward ipsilateral eye, nose or forehead. Two patients with comparable clinical features stemming from frontal scalp to ipsilateral posterior regions have been recently described and proposed as backward radiation epicrania fugax (BREF). We report a new series of nine BREF and compare their clinical characteristics with 18 forward radiation EF (FREF). Since first description of BREF in February 2010 we have assessed nine patients (four males, five females) with this clinical picture at an outpatient headache office in a Tertiary Hospital. Comparison is established with 18 FREF patients (6 males, 12 females), attended since the publication of first series of EF in March 2008. We found no differences between BREF and FREF, respectively, in age at onset (43.4 ± 13.1 vs. 42.5 ± 17.7 years), female/male ratio (5/4 vs. 12/6), pain intensity (6.9 ± 2.1 vs. 6.8 ± 2.1 in a 0-10 visual analogical scale), duration (7.1 ± 4.9 vs. 5.7 ± 4.3 s) and frequency of episodes per day (7 ± 8.4 vs. 9.9 ± 15.4). Patients in BREF group presented less frequently interictal pain in stemming point (22.2 vs. 55.5%) and accompanying autonomic signs (33.3 vs. 55.5%), but without statistical significance in both the cases. This series reinforces the proposal of EF as a new headache variant or a new headache syndrome. Clinical picture of brief pain paroxysms starting in the anterior scalp and radiating backwards does not fit known headaches or neuralgias and might correspond to a reverse variant of EF, clinical characteristics of which are comparable to FREF.