Occipital Neuralgia in Chiari I Malformation: Two Different Events or Two Different Faces of the Same Event?

Occipital neuralgia (ON) is characterized by severe pain in the occipital region due to an irritation of the occipital nerves. Traumatic injuries, mass or vascular compression, and infective and inflammatory processes could cause ON. The dislocation of a nerve/muscle/tendon, as can happen in malformations such as the Chiari I malformation (CIM), also can be responsible.

Headache in immigrant patients: similarities and differences with Italian population.

Headache is one of the most common neurological diseases. It is well known that there are differences in the perception and in the management of pain in various populations. Immigrants represent a growing portion between neurology outpatients.

Association of RAMP1 rs7590387 with the risk of migraine transformation into medication overuse headache.

Objectives/background: We herein investigated the role of polymorphisms in calcitonin gene-related peptide (CGRP)-related genes looking at the association of rs3781719 (T > C) in the calcitonin gene-related polypeptide-alpha (CALCA) gene and of rs3754701 (T > A) and rs7590387 (C > G) at the receptor activity modifying 1 (RAMP1) locus with triptan response in patients with migraine without aura (MwoA). In addition, their role was evaluated as risk factors for transformation of episodic migraine into medication overuse headache (MOH). The CGRP has a central role in the pathogenesis of migraine; however, little information is currently available concerning the role of polymorphisms in CGRP-related genes as determinants of clinical response to anti-migraine drugs or as risk factors for migraine chronification.

Impaired visual inhibition in migraine with aura.

Objective: The pathophysiology of migraine with or without aura (MA, MO) is still a matter of debate. We thus studied patients with MA and MO by means of paired-pulse flash-visual evoked potentials (paired F-VEPs). This technique, recently revived, analyses the overall excitability of visual system as detected from the cortical occipital signal.

Lack of association between GRIA1 polymorphisms and haplotypes with migraine without aura or response to triptans.

The present study was designed to replicate previous findings reporting a significant association between the rs548294 polymorphism at the glutamate receptor subunit GluR1 gene (GRIA1) and migraine without aura, either as a single marker or in haplotype combination with rs2195450. In addition, the role of GRIA1 polymorphisms and haplotypes was evaluated in migraine patients without aura as predictive factors for consistency in headache response to triptans. Analysis of rs548294 and rs2195450 polymorphisms of GRIA1 was conducted by Real-time PCR allelic discrimination assay in 186 migraine patients without aura and 312 healthy controls, respectively.

An opposite-direction modulation of the COMT Val158Met polymorphism on the clinical response to intrathecal morphine and triptans.

Unlabelled: Genetic variation in the COMT gene is thought to have clinical implications for pain perception and pain treatment. In the present study, we first evaluated the association between COMT rs4680 and the analgesic response to intrathecal morphine in patients with chronic low back pain to provide confirmation of previously reported positive findings. Next, we assessed the relationship between rs4680 and headache response to triptans in 2 independent cohorts of migraine patients.

A case of cranial multinevritis: from the onset to the diagnosis of primary neurolymphomatosis.

Neurolymphomatosis (NL) is a rare peripheral or cranial neuropathy caused by non-Hodgkin's lymphoma (NHL). Diagnosis is often delayed and prognosis is poor. The authors described a woman in her 70s with a facial left peripheral palsy, complete right abducent palsy, left hypoacusia and balance deficit.

Moyamoya disease and arteriovenous fistula of the epiaortic vessels.

The association of Moyamoya disease (MD) with other cerebrovascular disease has been reported in literature but not the occurrence of MD and arteriovenous fistula of the epiaortic vessels. A 61-year old Italian woman was admitted to our Department because of the sudden onset of Broca's aphasia. At general examination she presented a murmur above the right clavicle.

The serotonin transporter gene polymorphism STin2 VNTR confers an increased risk of inconsistent response to triptans in migraine patients.

The aim of the present observational study was to assess the value of the C825T polymorphism of the beta-3 subunit of G proteins (GNB3) as well as of variants in the SLC6A4 gene (5HTTLPR and STin2 VNTR) and DRD2 gene (TaqI A and NcoI) as predictive markers for consistency in headache response to triptans in migraine patients. Consistent responders to triptans were defined as the migraineurs who experienced a > or =2 point reduction in a 4-point scale intensity of pain from 3 (severe) to 0 (absent) 2h after triptan administration, in at least two attacks out of the three. Genotyping was performed by PCR and PCR-RFLP on genomic DNA extracted from peripheral blood.

Posterior alien hand in a left-handed person.

Posterior alien hand syndrome usually involves the non-dominant hand with lesions usually in the right hemisphere. This is the first case in a left-handed person, involving the dominant hand.

Cluster-like headache and idiopathic intracranial hypertension: a case report.

Cluster headache (CH) is a well-defined primary headache syndrome, but cases of symptomatic headache with clinical features of CH have been previously reported. Idiopathic Intracranial Hypertension (IIH) is a secondary headache disorder characterized by headache and visual symptoms, without clinical, radiological or laboratory evidence of intracranial pathology. Both papilloedema and IIH-related headache are typically bilateral, however asymmetrical or even unilateral localizations are described in literature.

Cerebral venous thrombosis: a retrospective multicentre study of 48 patients.

The objective was to describe the clinical features and management of cerebral venous thrombosis (CVT) in non-selected centres. An observational study in 11 neurological departments in NW Italy was carried out from 1995 through 1999 on 38 female and 10 male patients. Mean age: 44.

Churg-Strauss syndrome associated with the leukotriene antagonist montelukast.

Churg-Strauss syndrome (CSS) is a disseminated small vessel vasculitis characterized by late-onset asthma, upper airways disease, eosinophilia and late neurological manifestations such as peripheral neuropathy. Recently, several cases of CSS have been reported in patients treated with leukotriene antagonists after weaning corticosteroids. We describe a case of CSS developed while the patient was receiving montelukast for asthma treatment, after corticosteroids withdrawal.

[Spasmus nutans. A re-emergent pathology?].

The authors report two cases of spasmus nutans (a clinical syndrome peculiar to infancy the complete form of which is characterised by nystagmus, rhythmic head movements and wryneck) in two extra-EEC infants. The following elements were present in both patients: scarce exposure to light, early introduction of whole cow's milk, rachitic stigmata, dark skin, poor socio-economic conditions and rapid and complete remission of symptoms following daily exposure to sunlight. It is important to distinguish spasmus nutans, a completely benign and self-restricting disease, from potentially severe conditions which may appear with similar symptoms.

[Are metoclopramide dystonias familial?].

Metoclopramide is an antiemetic drug; the effects on CNS (acute dystonic reaction, tardive dyskinesia, parkinsonism) occur in only 1 of 500 patients treated. Acute dystonic reactions are not apparently dose-dependent and suggest individual sensitivity to the drug (idiosyncrasia). We report 4 cases in 2 families (grandmother-grandchild; brother-sister).

[The diagnostic course in patients with hereditary ataxias and hereditary spastic paraparesis].

A postal questionnaire was sent to all patients affected by hereditary ataxias and hereditary spastic paraparesis resident in the province of Turin (Italy) to study their diagnostic process. A 61% response rate was obtained. The mean time interval between onset and diagnosis was 6 years (1 to 32 years).

Glutamate dehydrogenase (GDH) deficiency in different types of progressive hereditary cerebellar ataxia.

Leukocyte glutamate dehydrogenase (GDH) was studied in 29 patients affected by progressive cerebellar ataxia (PCA) and in 20 healthy controls. Eight GDH-deficient patients, with GDH activity 2 SD below mean value of controls, were identified. GDH deficiency did not identify a subgroup of PCA by characteristic pattern of inheritance and/or age of onset of disease.

Post-poliomyelitic motor neuron disease. Clinical aspects and its relation to typical motor neuron disease.

Eighteen cases of post-poliomyelitic motor neuron disease (PPMND) were found in a series of 869 subjects affected by motor neuron disease (MND). The mean age of onset of acute anterior poliomyelitis (AAP) was 43.6 months.

Disability and quality of life in hereditary ataxias: a self-administered postal questionnaire.

A postal questionnaire was sent to 151 patients affected by Friedreich's disease (FD) and other hereditary ataxias (OHA) to study their disability and quality of life. A 79.5% response rate was obtained.