Livia N. Rossi

The Landry-Guillain-Barré syndrome: Complications, prognosis and natural history in 123 cases

One hundred and twenty-three patients, 68 males and 55 females in whom acute polyradiculitis Guillain-Barré appeared at an age of between 1,6 and 76 years were re-examined after 0.9 to 12.3 years. The initial symptoms, the signs during the acute phase, in particular the cranial nerve signs and central nervous signs, as well as findings in the cerebrospinal fluid and complications are described. Three patients died during the acute phase. At follow-up only 57% of the patients were completely cured. In 22% motor signs, mainly distal in the lower extremities were found. Only 6 of these patients however were handicapped. Twenty-two patients had loss of one or more tendon reflexes, in general the ankle jerk. Only 6 showed very slight central nervous system signs. Eleven of 55 follow-up cases had pathological findings in needle electromyography. A disturbance of conduction velocity or distal latency was also found in several adults and children without residual clinical signs. The time span between the maximum of the initial signs and the beginning of recovery seemed to be particularly long in patients who showed residual signs on follow-up. These patients also seemed frequently to have had a severe tetraparesis in the initial phase. We could not confirm the therapeutic effect of cortisone or ACTH in our patients: amongst the 30 adults treated with cortisone 6 (20%) had residual signs at follow-up, whilst 5 (25%) of the 22 untreated ones had similar findings.

A randomized trial of oral betamethasone to reduce ataxia symptoms in ataxia telangiectasia.

No controlled studies exist regarding the pharmaceutical reduction of ataxia symptoms in ataxia telangiectasia (A‐T). In a multicenter, double‐blind, randomized, placebo‐controlled crossover trial, oral betamethasone (BETA) and placebo were compared in terms of their reduction of ataxia symptoms as assessed with the International Cooperative Ataxia Rating Scale (ICARS). In this study of 13 A‐T children, betamethasone reduced the ICARS total score by a median of 13 points in the intent‐to‐treat population and 16 points in the per‐protocol population (ie, median percent decreases of ataxia symptoms of 28% and 31%, respectively). In conclusion, Oral betamethasone could be a promising therapy to relieve ataxia symptoms in A‐T patients; however, long‐term effectiveness and safety must be established. (Current Controlled Trials, number ISRCTN08774933.)

Obstetrical Lesions of the Brachial Plexus

34 subjects with 36 obstetrical paralysis of the brachial plexus were studied. The diagnosis was made immediately after birth in the great majority. 5 pareses of the lower plexus, however, were only r

Classification criteria and distinction between migraine and tension-type headache in children

The International Headache Society (IHS) classification system (1988) was developed primarily for headache disorders in adults and its validity for paediatric age is currently under discussion; in 1995 Seshia et al. proposed a revision of the criteria for migraine without aura to make diagnostic criteria more applicable to children. The purposes of the current study were to: (1) compare the IHS classification with the Seshia proposal, (2) compare the children affected by migraine without aura (MO) with the children affected by tension headache (TH) as defined by Seshia, for characteristics which are not included in the classification. The patients are a series of 320 children (mean age 9.9, SD 2.6 years; 144 males, 176 females) with recurrent or chronic headaches referred to a headache clinic in Milan, Italy. Using the Seshia criteria instead of the IHS criteria a higher number of children were included in the MO category: bilateral pain and family history of migraine were the most important factors which allowed a shift of children into this category. However, with the Seshia classification there was no reduction in the number of unclassifiable children. The reason why some children could not be classified was a short duration of attacks; the majority of unclassifiable children were 6 years old or less. No relevant difference was found between children with MO and children with TH for the following variables: occurrence of attacks in the afternoon or evening after school, reduction of attacks during school holidays, full‐time schooling, after‐school activities on school days, disordered daily life. On the contrary children with MO when compared with those with TH showed a higher number of precipitating factors and for the following factors a significant difference was found: exposure to TV or a computer, sleep deficiency, and strong emotions. Furthermore, children with MO showed a greater severity of attacks.

Osmophobia as an early marker of migraine: a follow-up study in juvenile patients.

Background: Osmophobia is frequent in children with migraine (20–35%) but can also occur in up to 14% of cases with tension-type headache (TTH). So far, the prognostic role of this symptom in children with primary headaches has never been evaluated. Methods: A longitudinal prospective study was conducted on 90 young patients with TTH (37 with osmophobia, 53 without osmophobia). We evaluated whether osmophobia could predict the diagnosis transformation from TTH to migraine after a 3-year follow-up. Results and Discussion: In our cases the rate of diagnosis change was significantly greater in cases with osmophobia (62%) than in those without (23%). Osmophobia persisted at a 3-year follow-up in the majority of our cases (85%) and it was found to be one of the major predictors for the development of migraine; other predictors of evolution to migraine were phonophobia, a probable rather than certain diagnosis of TTH and olfactory triggers (p < 0.05). Conclusion: Our data confirm that osmophobia has an important diagnostic and prognostic role in children with primary headaches and should be systematically investigated at diagnosis and during follow-up.

Diagnostic criteria for migraine and psychogenic headache in children

The headache histories obtained from 214 children were analysed by computer to see whether it was possible to identify and classify migraine, and to distinguish children with psychogenic headache. During headache attacks, most children had no or very few associated symptoms. For classification, 175 patients were divided into four homogeneous groups; the remaining 39 could not be grouped. An overlap between the different groups was found. Psychogenic headache emerged as a clearly definable syndrome, characterised by psychological problems and daily headache for a period of at least one month (10 patients). When the 214 patients were grouped according to the classification of the Headache Classification Committee of the International Headache Society, distinguishing those children with psychogenic headache was no longer possible.

Analysis of the International Classification of Headache Disorders for diagnosis of migraine and tension-type headache in children.

In 2004, the revised International Classification of Headache Disorders (ICHD‐II) was published. This study evaluates: (1) the results obtained from applying ICHD‐II to children with primary headaches to distinguish between migraine without aura (MO) and tension‐type headache (TTH); and (2) the results obtained from introducing modifications of the classification criteria for MO as suggested by various authors. There were 200 participants (93 males, 107 females; age range 3‐17y, mean 9y 8mo [SD 2y 7mo]). According to the ICHD‐II, MO compared with TTH was characterized by: higher intensity of pain; higher frequency of associated symptoms; and higher number of precipitating factors. The significant difference found between patients with MO/probable MO and those with TTH/probable TTH for the variables used in the ICHD‐II shows that these variables describe the two forms well. However, 15.5% of children proved to be unclassifiable, mainly because they could not give information for some criteria; other reasons for this were too short a duration of episodes and the possible overlap of criteria describing probable MO and probable TTH. The frequency of one variable, pulsating pain, significantly increased with age. Reduction of duration to 1 hour for MO produced a statistically non‐significant increase in the number of children with MO. Behaviour during attacks was found to be simple to apply in evaluating intensity and therefore was introduced as a new criterion. Severe intensity was related to MO, whereas moderate or low‐intensity was related to TTH.

BENIGN MIGRAINE‐LIKE SYNDROME WITH CSF PLEOCYTOSIS IN CHILDREN

Four children with symptoms consistent with complicated migraine had CSF pleocytosis, in three cases lymphocytic and in the fourth polymorphonuclear. In one case the CSF abnormality was found during two separate attacks of complicated migraine. On the basis of the 23 cases reported in the literature and these four personal cases, the authors conclude that, as a rule, pleocytosis is a secondary phenomenon of an attack of complicated migraine. However, in exceptional cases an infectious disease might produce both a migraine attack and CSF pleocytosis.

Maturation of Vestibular Responses: Preliminary Report

64 full‐term and three preterm, normal infants, appropriate for gestational age (AGA), were tested for the appearance of vestibular response to rotatory stimulation at a minimal postconceptional age of 44 weeks. A total of 56 positive responses was obtained. The majority of the full‐term AGA infants responded by the 45th postconceptional week (60 per cent of the 50 infants tested at that age). Greater duration of extra‐uteri ne life significantly accelerated the appearance of the response.

Survey on treatments for primary headaches in 13 specialized juvenile Headache Centers: The first multicenter Italian study

AIM The purpose of this retrospective multicenter study was to evaluate the use and the self-perceived efficacy and tolerability of pharmacological and non-pharmacological treatments in children and adolescents with primary headaches. METHODS Study of a cohort of children and adolescents diagnosed with primary headache, consecutively referred to 13 juvenile Italian Headache Centers. An ad hoc questionnaire was used for clinical data collection. RESULTS Among 706 patients with primary headaches included in the study, 637 cases with a single type of headache (migraine 76% - with and without aura in 10% and 67% respectively; tension-type headache 24%) were selected (mean age at clinical interview: 12 years). Acetaminophen and non-steroidal anti-inflammatory drugs (in particular ibuprofen) were commonly used to treat attacks, by 76% and 46% of cases respectively. Triptans were used overall by 6% of migraineurs and by 13% of adolescents with migraine, with better efficacy than acetaminophen and non-steroidal anti-inflammatory drugs. Preventive drugs were used by 19% of migraineurs and by 3% of subjects with tension-type headache. In migraineurs, flunarizine was the most frequently used drug (18%), followed by antiepileptic drugs (7%) and pizotifen (6%), while cyproheptadine, propanolol and amitriptyline were rarely used. Pizotifen showed the best perceived efficacy and tolerability. Melatonin and nutraceuticals were used by 10% and 32% of subjects, respectively, both for migraine and tension-type headache, with good results in terms of perceived efficacy and tolerability. Non-pharmacological preventive treatments (i.e. relaxation techniques, biofeedback, cognitive-behavioral therapy, acupuncture) were used only by 10% of cases (migraine 9%, tension-type headache 15%). DISCUSSION Non-steroidal anti-inflammatory drugs, especially ibuprofen, should be preferred to acetaminophen for acute attacks of migraine or tension-type headache, because they were usually more effective and well tolerated. Triptans could be used more frequently as first or almost second choice for treating migraine attack in adolescents. Non-pharmacological preventive treatments are recommended by some pediatric guidelines as first-line interventions for primary headaches and their use should be implemented in clinical practice. Prospective multicenter studies based on larger series are warranted to better understand the best treatment strategies for young people with primary headaches.