Behcet Disease: Long-term Follow-up of Three Children and Review of the Literature

PEDIATRICS ◽  
1989 ◽  
Vol 83 (6) ◽  
pp. 986-992
Author(s):  
Yardena Rakover ◽  
Hanna Adar ◽  
Itamar Tal ◽  
Yaron Lang ◽  
Amos Kedar

Behcet disease is rare in children. There are only two reports of Behcet disease in childhood, describing seven patients. Three pediatric patients are described, in whom the age of onset ranged from 6 to 11 years. Aphthous stomatitis and arthritis were present in all of the patients; genital ulcers, iridocylitis, erythema nodosum, and CNS involvement were present in two patients. Other manifestations included Stevens-Johnson-like eruption, fever of unknown origin, and testicular involvement. All of the patients responded to glucocorticoids; two were also treated with colchicine and one was treated with chlorambucil. In two patients, follow-up of more than 10 years was done, with complete cure in one patient and benign course of illness in the other. Because of the rarity of the disease in childhood and the difficulty in making the diagnosis, there is not enough awareness by pediatricians concerning this disease.

2000 ◽  
Vol 39 (12) ◽  
pp. 715-717 ◽  
Author(s):  
Julie-An M. Talano ◽  
Ben Z. Katz

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Shuntaro Oribe ◽  
Takafumi Toyohara ◽  
Eikan Mishima ◽  
Takehiro Suzuki ◽  
Koichi Kikuchi ◽  
...  

Abstract Background Fibromuscular dysplasia (FMD) often causes renal artery stenosis with renovascular hypertension. Recent clinical outcomes encourage percutaneous transluminal renal angioplasty (PTRA) to treat FMD; however, the necessary follow-up period remains unclear. Moreover, previous studies have not revealed the difference in the period until recurrence between two major types of FMD—multifocal and focal. Case presentation We describe two patients with multifocal FMD who developed hypertension during their teenage years and had recurrence of FMD > 10 years after PTRA. We further examined the types of FMD and age of onset in 26 patients who underwent PTRA. The period until recurrence of multifocal FMD was longer than that of focal FMD. Moreover, patients with early-onset multifocal FMD are likely to have a delayed recurrence after PTRA compared to other types. Conclusions Our report suggests that patients with multifocal FMD, especially those with onset at an early age, may need long-term follow-up for at least ≥ 10 years.


Cephalalgia ◽  
1994 ◽  
Vol 14 (6) ◽  
pp. 458-460 ◽  
Author(s):  
G Lanzi ◽  
U Balottin ◽  
E Fazzi ◽  
M Tagliasacchi ◽  
M Manfrin ◽  
...  

We examined clinical aspects of Benign Paroxysmal Vertigo (BPV) in infancy and its most frequent differential diagnosis, in particular analogies and differences with forms of “migrainous vertigo” (MV) of later onset. During a long-term follow-up of 7 cases of BPV, diagnosed according to the Basser criteria, 5 of 7 BPV cases spontaneously resolved and 6 of 7 patients later developed migraine and other migraine-related symptoms. This course differs from that described for MV only in the age of onset of headache and in the chronological relationship with vertigo. The authors suggest that BPV can be interpreted as a migraine precursor and MV as a migraine equivalent.


Author(s):  
Shabeer Ahmad Paul ◽  
◽  
Chandrakanta Patra ◽  
Gouranga Prasad Mondal ◽  
Ramesh Bhattacharyya ◽  
...  

Pure autonomic failure is considered to be a degenerative condition of the autonomic nervous resulting from accumulation of alpha-synuclein in autonomic ganglia [1,2]. Lack of CNS involvement distinguishes this disorder from other synucleopathies with CNS involvement like MSA, DLB and PD. However, long term follow-up of PAF patients has shown that some of these cases convert to one of these disorders most commonly MSA [3]. Also, recent data, albeit limited in nature has shown the association of REM sleep disorder in PAF patients challenging the previous notion that PAF is a disease restricted to autonomic nervous system. This data has led to the discovery of a clinical syndrome with a position in the middle of the spectrum of these disorders with PAF at one end of the spectrum and MSA, DLB and PD at the other end. We hereby present a case report of one such patient with PAF associated with RBD but without any other CNS manifestations.


Neurosurgery ◽  
2007 ◽  
Vol 60 (5) ◽  
pp. E953-E955 ◽  
Author(s):  
Elias Dagnew ◽  
Lauren A. Langford ◽  
Frederick F. Lang ◽  
Franco DeMonte

Abstract OBJECTIVE The pineal region is a rare intracranial site for metastasis. We report three patients initially considered to have metastatic papillary adenocarcinoma to the pineal region. On review, these papillary, keratin-positive neoplasms meet the criteria for papillary tumor of the pineal region (PTPR). CLINICAL PRESENTATION These neoplasms occurred in three women (age range, 37–55 yr). Imaging studies demonstrated well-circumscribed lesions in the pineal region. All patients presented with obstructive hydrocephalus and symptoms attributable to hydrocephalus and tectal compression. INTERVENTION All three patients underwent near total microsurgical resection of the pineal region neoplasm, followed by adjuvant radiotherapy. The two patients with long-term follow-up (56–60 mo) have remained clinically stable without evidence of local or distant recurrence. The first two patients were initially diagnosed as having papillary metastatic carcinoma of unknown origin. The third patient was treated after the recent description of PTPR and met the histopathological diagnostic criteria. Retrospective pathological review of the previous two patients resulted in designation as PTPR. CONCLUSION The morphological features of the tumors in our series, along with the clinical presentations, are similar to those in the original description of the PTPR. Our findings agree with the original hypothesis that the cells composing the PTPR are similar to ependymal cells of the subcommissural organ, thus furthering the hypothesis that the PTPR derives from a specialized ependymocyte associated with the subcommissural organ. The two patients with long-term follow-up (56–60 mo) have remained clinically stable without evidence of local or distant recurrence.


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