Trigeminal neuralgia as the first clinical manifestation of anti-hu paraneoplastic syndrome induced by a borderline ovarian mucinous tumor.

Paraneoplastic neurologic syndrome (PNS) is an uncommon manifestation of cancer that is not caused by the tumor or metastasis. Trigeminal neuralgia (TN) is an initial symptom of this disease, but it has rarely been reported in the literature. Here, we report the case of a 76-year-old woman who presented with classic TN, followed by limbic encephalitis due to an underlying ovarian intestinal-type mucinous borderline tumor, with the presence of anti-Hu antibodies. She recovered quickly after removal of the tumor and was essentially free of symptoms 2 weeks after surgery. Because PNS precedes the tumor in approximately 60% of cases, its rapid detection and treatment are crucial. Therefore, we propose that PNS be considered during the management of TN when brain imaging is normal, as it is followed by other central and/or peripheral neurological manifestations as well as the presence of systemic symptoms such as anemia, fatigability, loss of appetite, or weight loss.

Multiple Large Tumefactive MS Plaques in a Young Man: A Diagnostic Enigma and Therapeutic Challenge.

Tumefactive demyelinating lesion is defined as large solitary demyelinating lesion with imaging characteristics mimicking neoplasm. These atypical features include size more than 2 cm, mass effect, edema, and/or ring enhancement. Distinguishing tumefactive lesions from other etiologies of intracranial space occupying lesions is essential to avoid inadvertent surgical or toxic chemotherapeutic intervention. Symptoms are generally atypical for multiple sclerosis (MS) and usually related to the pressure of a focal mass lesion without a history of MS. The clinical presentation and MRI appearance of these lesions often lead to biopsy. Here, we present a young man with fulminating neurological symptoms and multiple large tumefactive lesions on either hemisphere. Since patient and parents were not agreed on brain biopsy, a course of steroid therapy was commenced which ended to considerable improvement and confirmed the diagnosis of tumefactive MS. Thirteen months later, he experienced another relapse when his treatment was continued by weekly intramuscular injection of interferon b1a (Avonex). Two further MRIs showed shrinkage of tumefactive plaques and resolution of edema in the periphery of lesions.

Barking seizure: acute episodes of barking in a 75-year-old previously healthy man.

A 75-year-old right-handed man was admitted to our emergency department complaining of recurrent episodes of involuntary 'barking' within the past 12h. The episodes had occurred after an initial two-minute attack from sleep involving tonic contraction of the upper extremities and jaw locking. By the time of admission, the patient had had a total of at least 7-10 'barking' episodes, each lasting 30-45 s. Seven months prior to his current admission, the patient had had a minor ischemic stroke causing mild left paresis, which had resolved completely. His awake EEG revealed a normal background pattern interrupted by runs of two per second slow waves mixed with low-voltage spikes in the left temporal lobe with a left mid-temporal emphasis. The patient was diagnosed with recurrent simple partial seizures, and treatment with intravenous valproic acid was initiated. He was discharged four days later without having experienced any further barking episodes. Atypical presentations of the epileptic seizures have been described in the literature, but ictal barking is very rare manifestation of epilepsy.

Frequency distribution of the first clinical symptoms in the Iranian population with multiple sclerosis.

BACKGROUND: Initial symptoms of multiple sclerosis (MS) may be varied and nonspecific. We tried to find the frequency distribution of the first clinical symptoms in Iranian patients with MS. METHODS: In a case series study, 1130 patients with definite diagnosis of MS who had been referred to three referral university hospitals of Tehran, Iran, were enrolled. The patients' medical records were reviewed for neurological history to find the first symptom at presentation. RESULTS: 884 (78.2%) patients were female and 246 (21.8%) were male. The mean ± SD age of patients was 31.4 ± 9.1 years. The most common initial symptoms were motor in 492 (43.5%), ocular in 366 (32.4%), cerebellar in 91 (8.1%), sensory in 76 (6.7%), cranial nerve involvement in 51 (4.5%), and fatigue in 23 (2%) patients. There was no difference between female and male patients in first clinical symptoms (P > 0.05). CONCLUSION: The motor symptoms were the most common finding at presentation in the Iranian population with MS. Complementary studies with larger sample sizes are needed to increase the external validity.

Venous thrombosis in multiple sclerosis patients after high-dose intravenous methylprednisolone: the preventive effect of enoxaparin.

Aim. This study was designed to examine the possible role of high-dose intravenous methylprednisolone (IVMP) in the development of venous thrombosis (VT). The cerebral one anecdotally had been reported in patients with relapsing remitting multiple sclerosis (RRMS) in acute attacks and the possible preventive role of enoxaparin. Material and Methods. From a pool of 520 patients, 388 patients with definite RRMS who fulfilled entry characteristics were selected and randomly received either a 5-day course of daily 1 gr IVMP or the aforementioned plus 5 days of daily subcutaneous 40 units of enoxaparin according to a predefined protocol. Results. Mean age, gender ratio, mean relapse rate, and EDSS were similar in both groups of patients (P > 0.05). Finally, 366 patients remained in the study. Of 188 patients treated with IVMP with 855 relapses, 5 developed VT (0.37% per patient per year and 0.58% per each course of IVMP) within 3 to 15 days of starting therapy. None of the 178 patients who experienced 809 relapses who received IVMP plus enoxaparin developed such complications. Conclusion. The study implies that high-dose IVMP in MS exacerbation may increase the risk of VT and prophylactic anticoagulant treatment in this setting is warranted.

Opticospinal multiple sclerosis in Iran.

Clinical course, magnetic resonance imaging (MRI) findings and cerebrospinal fluid data (CSF) on 20 patients among 520 with clinically definite multiple sclerosis (MS) according to the criteria of Poser et al., with opticospinal clinical presentation above 5 years and mean disease duration of 8+/-4.4 years were reviewed. The prevalence rate was 3.8%. The clinical course was relapsing-remitting (RR) for all patients. The mean age of onset was 24+/-8.2 years. The gender ratio was 2.3:1 female:male. First clinical presentation was spinal signs in 12 (60%), optic neuritis in 7 (35%) and simultaneous involvement of both eyes in 1 (5%) patient. No transverse myelitis (TM), sustained severe optic neuritis (SSON) or minor brain stem signs were recorded. No conversion to conventional MS (CMS) or secondary progressive MS (SPMS) was identified during the study period and no case had positive family history for the disease. The yearly number of attacks was 0.66+/-0.84, with mean Kurtzke expanded disability status scale (EDSS) of 2.5+/-1.2. All patients had 1 to 5 hemispheral T2 lesions in brain MRI, non-fulfilled Barkhof criteria for brain lesions. All had preventricular and 14 (70%) had juxtacortical lesions. None had gadolinium T1 enhanced lesions. No involvement of the brainstem or cerebellar structures was detected. Fifteen had 1 cervical lesion, five had 2 and four had 1 concomitant thoracic cord signal, all extending below three vertebral segments in sagittal planes with peripheral white matter location on the axial planes. Spinal fluid contained normal cell and protein with negative oligoclonal bands (OB).

Interferon Beta-1a and intravenous immunoglobulin treatment for multiple sclerosis in Iran.

The aim of the study was to evaluate the efficacy and safety of interferon beta-1a (Avonex) and intravenous immunoglobulin (IVIG) in clinical practice for the treatment of relapsing-remitting multiple sclerosis. Avonex is the most common disease-modifying therapy used in Iran due to its ease of administration. IVIG is also frequently used due to its alleged effectiveness and fewer side effects. Eighty patients were selected and prospectively monitored according to a predefined protocol. They were then randomized to receive either weekly intramuscular injections of Avonex or 0.4 g/kg monthly IVIG in a single blind fashion and following an attack of exacerbation which was treated with steroids. Basal relapse rate and Expanded Disability Status Scale (EDSS) were similar in both groups of patients (p > 0.4). Seventy-two patients remained in the study. The annual relapse rate consistently decreased from 0.95 +/- 0.41 to 0.60 +/- 0.67 (approximately 32%, p < 0.001) for 34 patients treated with Avonex and from 1.05 +/- 0.34 to 0.55 +/- 0.46 for 38 patients in the IVIG group (approximately 47%, p < 0.001). EDSS decreased by 0.4 units in IVIG-treated patients (p < 0.001) and remained stable (0.2 < p < 0.3) in the Avonex arm. This study confirms the relative efficacy of both treatments with better safety profile for IVIG in the studied Iranian population. However, the results are very preliminary ones, due to limited numbers of patients and only 12 months of treatment.

Multiple sclerosis: report on 200 cases from Iran.

Clinical findings of 200 patients in Iran with definite multiple sclerosis (MS) according to Poser et al.'s criteria and positive findings on magnetic resonance imaging (MRI) have been reviewed. The clinical course was relapsing-remitting (RR) for 88%, primary progressive (PP) for 7% and secondary progressive (SP) for 5% of cases. The mean age of onset was 27 +/- 7.4 years for the whole group and 37.1 +/- 8.8 years for PPMS. The gender ratio was 2.5:1 female:male. Involvement of the pyramidal system was the most common mode of presentation. Five per cent of patients had positive family history for the disease, 14% of patients had benign MS and 12% with disease duration longer than five years had an Expanded Disability Status Scale < or = 2. The optico-spinal form was not a common form of presentation in the group.