Ipsilateral cluster headache and chronic paroxysmal hemicrania: two case reports

We report two patients with ipsilateral attacks of cluster headache and chronic paroxysmal hemicrania. The first patient, a 33-year-old man, started having attacks of chronic cluster headache at the age of 27. At 33, they were replaced by typical attacks of ipsilateral chronic paroxysmal hemicrania which showed a dramatic improvement with indomethacin 150 mg daily. After two days of complete remission, cluster headache attacks reappeared and persisted until verapamil, 360 mg a day, was added to indomethacin. The second patient, a 45-year-old man, first developed attacks of episodic cluster headache at the age of 35. At 44, he experienced ipsilateral typical attacks of chronic paroxysmal hemicrania, and two months later attacks of cluster headache. Under verapamil 240 mg daily, attacks of cluster headache disappeared, but those of chronic paroxysmal hemicrania increased in frequency until indomethacin 150 mg daily was added. These observations suggest a close relationship but not a similarity between cluster headache and chronic paraoxysmal hemicrania, and show the practical therapeutic interest of maintaining this distinction.     

Long-term follow-up of patients with persistent/recurrent,isolated haematuria: A Hungarian multicentre study

A retrospective multicentre study of 341 children with persistent/recurrent, isolated haematuria is described. The haematuria was isolated for at least 6 months at the beginning of observation. The duration of follow-up was 2–5 years in 201, 5–10 years in 119, 10–15 years in 19, and over 15 years in 2 cases. Of these patients 47.8% became symptom-free. In 18.4% the haematuria remained isolated; in 13.8% it was combined with proteinuria over 250 mg/day more than 2 years later. The occurrence of associated proteinuria increased progressively with time. It was 8.6% between the 3rd and 5th years, and 37.0% after the 5th year. Renal biopsy was performed because of the symptoms of glomerular disease in 47 cases at an average time of 12 months following the appearance of proteinuria. Proteinuria appeared after a 2–5, 5–10, 10–15 and more than 15 years follow-up period in 16, 23, 6, and 2 patients respectively; 14 of them had Alport's nephropathy. The percentage of more serious azotaemia was 1.7 (creatinine clearance: 10–50 ml/min per 1.73 m²) and 0.3 (creatinine clearance: < 10 ml/min per 1.73 m²). Mortality was 0.58%. Most of the patients who developed severe azotaemia had persistent microscopic haematuria at the beginning. The prevalence of hypertension was only 1.2%. The time of its appearance was above 5 years in 2 and below 5 years in 2 cases. All these patients had chronic glomerulonephritis. The haematuria was associated with hypercalciuria in 19.9%. In 14.3% of the overall group of patients urolithiasis developed 2–15 years after onset. All of these had hypercalciuria. Our findings suggest that symptoms of isolated haematuria may last for a longterm period and need systematic control. When proteinuria and/or hypertension is associated with haematuria a worse prognosis can be expected.Participating paediatric hospitals and university departments: Second Department of Paediatrics, I. Semmelweis Medical University of Budapest (M. Visy); Department of Paediatrics, University Medical School of Pécs (V. Jászai); Department of Paediatrics, A. Szent-Györgyi Medical University of Szeged (I. Haszon, S. Túri); County Children's Hospital, Miskolc (Á. Vissy); P. Heim Children's Hospital, Budapest (Z. Czirbesz); County Children's Hospital, Györ (Zs. Szelid); Buda-Children's Hospital, Budapest (I. Ferkis); I. Apáthy Hospital, Budapest (J. Kisbán); János Hospital, Budapest (I. Marosváry); Hospital of Hungarian State Railway, Budapest (J. Fehér); L. Madarász Hospital, Budapest (F. Kalmár); South Pest Hospital, Budapest (G. Halász); County Children's Hospital, Pécs (E. Kolman); County Children's Hospital, Gyula (P. Sipos); County Children's Hospital, Szolnok (I. Jaksics); County Children's Hospital, Debrecen (Á. Miskolczi); County Children's Hospital, Tatabánya (I. Kiss); County Children's Hospital, Eger (M. Frank, E. Ladányi); County Children's Hospital, Nyíregyháza (E. Bujdosó); County Children's Hospital, Szombathely (M. Andics); Kerepestarcsa Hospital, Budapest (M. Marcell); Komárom Hospital, Komárom (J. Kecskés)     

MRI and neurological complications of adult T-cell leukemia/lymphoma.

We report the assessment by MRI of a lumbosacral lymphomatous localization of adult T-cell leukemia/lymphoma (ATLL) with human T-cell lymphotropic virus-1 infection in an African patient. Brain MRI detected associated multifocal lesions of increased signal intensity in the subcortical white matter. These MRI abnormalities are compatible with reported necropsy findings in cases of ATLL with neurological complications.     

Transepithelial paracellular leakiness induced by chronic phorbol ester exposure correlates with polyp-like foci and redistribution of protein kinase C-alpha

Although exposure of LLC-PK1 epithelial cell sheets to phorbol esters (TPA) causes a near immediate and total decrease of transepithelial electrical resistance (TER), continuation of exposure for 3 to 4 days results in a tachyphylactic response as TER begins to return to control levels. Recovery of TER is maximal by 5 to 6 days, but reaches only 70 to 80% of control level. A reciprocal change in the transepithelial flux of D-mannitol indicates that the TER decrease is indicative of an increase in tight junction permeability. Exposure of cell sheets to TPA for several days also results in the appearance of multilayered polyp- like foci (PLFs) across the otherwise one cell layer thick cell sheets. The pattern of penetration of the electron dense dye, ruthenium red, from the apical surface, across the tight junction and into the lateral intercellular space indicates that the tight junctions of the cell sheet become uniformly leaky after acute exposure to TPA. However, when exposure is continued for several days, only the junctions of cells in the PLFs manifest leakiness. The decrease in TER following acute TPA exposure correlates with the translocation of protein kinase C-alpha (PKC alpha) into a membrane-associated compartment. With exposure of several days, only a trace of PKC alpha is visible by Western immunoblot, and this is in the membrane-associated compartment. Immunofluorescent microscopy indicates that the trace of PKC alpha seen in the Western immunoblots is ascribable distinctly to cells of the PLFs. Monolayer areas between PLFs show no discernible immunofluorescent signal. The data therefore indicate that tight junction barrier function may be restored in certain areas by the down regulation of PKC alpha from the membrane-associated compartment. Failure to down regulate may result in the paracellular leakiness and abnormal cell architecture of the PLFs. Possible implications of this model for in vivo epithelial tumor promotion are discussed.      

Acute abdominal attack of hereditary angioneurotic oedema associated with ultrasound abnormalities suggestive of acute hepatitis

Hereditary angioneurotic oedema (HANO) is an autosomal dominant disorder caused by a deficiency of the inhibitor protein Cl-esterase. Recurrent subcutaneous and/or submucosal oedema formation is a hallmark of this disease. HANO is a rare, but potentially life-threatening disorder with a mortality around 20-30%. Acute oedematous abdominal attacks of HANO can mimic a surgical emergency; this is exemplified by the case of a 14-y-old male patient with HANO admitted for such clinical manifestations. Conclusion: Diagnostic clues include ascites and abnormalities of hepatic structure visible with ultrasound during the oedematous attack. The importance of appropriate treatment is emphasized.     

Long-term follow up of chronic recurrent isolated hematuria

A retrospective multicentre study of 341 children with persistent/recurrent, isolated haematuria is described. The haematuria was isolated for at least half a year in the beginning of observation. 47.8% of the patients became symptom-free. In 18.4% the haematuria remained isolated, in 13.8% it was combined with greater than 250 mg/day proteinuria greater than 2 years later. The occurrence of associated proteinuria was 8.6% between the 3rd to fifth years, and 37.0% after the 5th years. 14 cases had Alport's nephropathy. The percentage of more serious azotaemia was 1.7 (Ccreat: 10-50 ml/min/1.73 m2) and 0.3 (Ccreat: less than 10 ml/min/1.73 m2). Mortality was 0.58%, rate of hypertension 1.2%. Most of the patients who developed severe azotaemia, had persistent microscopic haematuria in the beginning. The haematuria was associated with hypercalciuria in 19.9%. In 14.3% of the overall group of patients urolithiasis developed 2-15 years after onset. All of them had hypercalciuria. Our findings suggest that symptoms of isolated haematuria may last for a long-term period and need systematic control. When proteinuria and/or hypertension associates to haematuria a worse prognosis can be expected.