Spinal cord compression is a rare presentation of non-Hodgkin lymphoma. Extradural location at onset is a rare but devastating event in pediatric oncology. The authors describe a girl with acute spinal cord compression due to epidural non-Hodgkin lymphoma, emphasizing the encouraging perspective for a complete recovery in children with this condition. A 5-year-old girl presented with pain followed by progressive hyposthenia and paraplegia after a trauma. CT scan and MRI showed homogeneous tissue extending from T2 to L4, occupying the entire vertebral canal and extending to the para- and peri-vertebral soft parts. Emergency surgical debulking was carried out through T6-L1 laminectomy. The patient began chemotherapy (LMB 89 Protocol) and the tumor quickly disappeared. The patient is maintaining a complete remission 42 months after diagnosis. Significant results may be obtained with the chemotherapy treatment of epidural non-Hodgkin lymphoma when the disease is promptly diagnosed. Considering the effectiveness of chemotherapy, the authors believe that a neuro-surgical approach should be employed only when rapid worsening of symptoms is observed or for diagnostic purpose.
Abstract-Objectives:To investigate the clinical pictures of patients with recurrent thunderclap headaches of unknown etiology and to field-test two relevant International Classification of Headache Disorders, 2nd edition (ICHD-II) criteria, i.e., primary thunderclap headache (Code 4.6) and benign (or reversible) angiopathy of the CNS (Code 6.7.3). Methods: We prospectively recruited patients presenting with idiopathic recurrent thunderclap headaches from a hospital-based headache clinic. Detailed histories, neurologic examinations, and MRIs and magnetic resonance angiographies (MRAs) were performed in all patients to exclude secondary causes. Patients with cerebral vasoconstriction received serial MRA follow-up. Results: Fifty-six consecutive patients (51 female/5 male, mean age 49.6 Ϯ 9.8 [range 22 to 76] years) were enrolled. Segmental vasoconstriction (or benign CNS angiopathy) was found in 22 patients (39%). Thunderclap headache recurred in all patients with a median frequency of 0.7 times per day for a median period of 14 days (range 6 to 86 days). The median duration for each single attack was 3 hours. Most patients (84%) reported at least one trigger. Nimodipine effectively aborted further attacks in 83% of the treated patients. Headache attacks subsided within 3 months. Four patients (7%) developed ischemic complications. Patients with and without vasoconstriction based on MRA images were similar regarding demographics and headache profile. Except for the duration criterion, our patients generally mapped well into the proposed ICHD-II criteria. Conclusions: This study suggests that the two diagnostic entities proposed by the ICHD-II may present different spectra of the same disorder. The distinct headache profile may help physicians quickly recognize this disabling headache disorder with risk of stroke and provide timely treatment.
IntroduzioneNonostante i progressi compiuti nella terapia farmacologica, chirurgica ed endovascolare, ancora oggi l'approccio terapeutico all'insufficienza vertebro-basilare da patologia steno-occlusiva delle arterie vertebrali non è ancora ben codificato 2,5 . Ciò non solo per le relative difficoltà del riconoscimento clinico e dell'inquadramento diagnostico neuroradiologico (nell'ambito del quale il ricorso all'angiografia digitale si impone pur in epoca angio-TC e angio-RM), ma anche per la mancanza dell'impulso alla ricerca di lesioni "chirurgiche" 2,3 . Le tecniche chirurgiche disponibili (endarterectomia dell'arteria vertebrale prossimale, by-pass tra arterie carotide comune o succlavia ed arteria vertebrale, tra arteria occipitale ed arterie vertebrali o cerebellare postero-inferiore, ecc), infatti, sono di difficile esecuzione e gravate da alta percentuale di complicanze neurologiche e polmonari legate anche alla toracotomia 5 . Proprio per questi motivi, addirittura, non è possibile definire con certezza la frequenza dell'insufficienza vertebro-basilare nella popolazione. Secondo i risultati di un recente studio, tuttavia, tra il 10 ed il 20% dei pazienti con diagnosi clinica precoce di presunto stroke acuto del circolo cerebrale anteriore può avere un infarto del circolo cerebrale posteriore 1 .Il progresso dei materiali e della tecnica endovascolare ha consentito, invece, il trattamento della patologia steno-occlusiva delle arterie vertebrali mediante posizionamento percutaneo di stent coronarici senza ("primario") o dopo ("secondario") angioplastica 3,6-8,10 . Le indicazioni proposte da Chastain et Al 3 , in particolare, includono il trattamento di:-stenosi sintomatiche (TIA o stroke minor del circolo posteriore) significative (>60%) di entrambe le arterie vertebrali, -stenosi sintomatica significativa dell'arteria vertebrale dominante, -lesioni significative di un'arteria vertebrale non dominante quando la sintomatologia derivi da deficit dell'arteria cerebellare postero-inferiore omolaterale e -stenosi significativa in paziente asintomatico che necessita di compenso emodinamico (ad esempio per concomitante occlusione carotidea).Gli scopi del presente lavoro sono la discussione e l'estensione delle suddette indicazioni e la descrizione delle opzioni tecniche attuali tramite la presentazione dei risultati ottenuti in 34 pazienti trattati con stenting delle arterie vertebrali per patologia steno-occlusiva. Materiali e MetodiPazienti e Trattamenti. Nel periodo tra Novembre 1997 e Ottobre 2001 sono stati eseguiti 36 trattamenti in 34 pazienti (25 maschi, 9 femmine), di età compresa tra 34 e 81 anni (media: 66,3). La sintomatologia consisteva in vertigini, disturbi dell'e-
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