Stiff Person Syndrome and Encephalitis with GAD Antibodies with Severe Anterograde Amnesia in an Adolescent: A Case Study and Literature Review

2021 ◽  
Author(s):  
Léa Herbulot ◽  
Chloé Bost ◽  
Agnès Viguier ◽  
Nathalie Faure-Marie ◽  
Eloïse Baudou ◽  
...  

AbstractAntiglutamic acid decarboxylase (GAD65) encephalitis is rare and few pediatric cases have been reported, with variable clinical presentations. A 14-year-old female adolescent was managed in our department. She had been treated for several months for drug-resistant temporal lobe epilepsy and gradually presented major anterograde amnesia with confusion. Upon her arrival at the University Hospital Centre, she showed a classical form of stiff person syndrome. The brain magnetic resonance imaging showed bitemporal hyperintensities and hypertrophy of the amygdala. The blood and cerebrospinal fluid were positive for GAD65 antibodies. At 2 years of immunosuppressive treatment and rehabilitation, the course showed partial improvement of the memory and neuropsychiatric impairment, and epilepsy that continued to be active. GAD65 antibodies are associated with various neurological syndromes, and this presentation combining limbic encephalitis and stiff person syndrome is the first pediatric form published to date; there are also few cases described in adults.

2005 ◽  
Vol 11 (3) ◽  
pp. 470-477
Author(s):  
M. Yahyaoui

Neurosyphilis accounts for 56%-70% of all visceral syphilis and is a complication in 5%-10% of cases of untreated syphilis. The aim of this study was to evaluate the epidemiological aspects and clinical presentations of neurosyphilis in Morocco through a series of 201 patients attending the Centre for Neurological Services at the university hospital in Rabat between 1986 and 1997. The mean age of the patients was 41.26 [SD 9.23] years [range:17-70 years] ; the majority [91%] were male. The incidence of neurosyphilis in Morocco is high. From 31 cases per year in 1985, it has fallen since 1990 to reach 10 cases in 1997. Among the different clinical presentations recorded, chronic meningoencepahalitis was the commonest, followed by meningovasculitis, tabes dorsalis and optic atrophy


2020 ◽  
Vol 16 (4) ◽  
pp. 343-345 ◽  
Author(s):  
Mohamed-Anis Souissi ◽  
Syrine Bellakhal ◽  
Emna Gharbi ◽  
Istabrak Abdelkefi ◽  
Mohamed-Hedi Douggui

Background: Stiff Person Syndrome (SPS) is a rare autoimmune neurological disorder which is often misdiagnosed. We report here a case of SPS with a long diagnosis delay. Case: A 36-year-old man presented with an 11-year history of progressive stiffness and painful spasms of his both legs, with recent worsening of his condition over the last year resulting in a considerable difficulty of standing up and walking. As the patient developed phobic symptoms, he was considered as having a psychiatric illness and treated with antianxiety and antidepressant drugs. As no real improvement was observed, the patient was referred to internal medicine. Neurological examination showed paraspinal, abdominal and lower limbs muscle contraction with lumbar rigidity. These symptoms were associated to adrenergic symptoms: profuse sweating, tachycardia and high bloodpressure. Initial routine investigations revealed high blood glucose level. Polygraphic electromyographic (EMG) evaluation from paraspinal and leg muscles showed continuous motor unit activity in agonist and antagonist muscle. Electroencephalography and brain magnetic resonance imaging were normal. Immunologic tests according to radio immune assay technique revealed high level of serum anti-glutamic acid decarboxylase (anti-GAD65) antibodies. Diagnosis of autoimmune SPS was retained based on clinical, electrophysiological, and immunological findings. Pregabalin at the dose of 150 mg, three times a day was prescribed with satisfying response. Conclusion: SPS is supported by an autoimmune pathogenesis and anti-GAD antibodies seems to be very helpful when SPS is clinically suspected. Treatment of SPS is a challenge, given the scarcity of the syndrome and the absence of established recommendations.


2021 ◽  
Vol 15 (06) ◽  
pp. 892-896
Author(s):  
Tomislava Skuhala ◽  
Dalibor Vukelić ◽  
Boško Desnica ◽  
Mirjana Balen-Topić ◽  
Andrija Stanimirović ◽  
...  

Introduction: To review unusual actinomycosis cases that appeared as a diagnostic and therapeutic challenge at our institution and to present a literature review on the usual clinical presentations. Methodology: This retrospective review included all patients hospitalized for actinomycosis in a 10-year period at the University Hospital for Infectious Diseases “Dr. Fran Mihaljević”, Zagreb, Croatia. Results: A total of 15 patients were hospitalized during the observed period, 9 (60%) females and 6 (40%) males. The localizations of actinomycosis were: pelvis (5), lungs (3), blood stream (2), colon (1), penis (1), stomach (1), skin (1), cervicofacial region (1). We present four unusual cases: subcutaneous actinomycotic abscess, actinomycosis of the stomach with underlying non-Hodgkin lymphoma, sepsis due to Actinomyces neslundii originated from chronic asymptomatic periapical tooth abscesses and actinomycosis of the distal part of the penile shaft. Conclusions: Actinomycosis was a very rare clinical problem in our clinical practice (0.032% of all hospitalizations and 0.0034% of all outpatients) but among those cases classical clinical presentations were also very rare.


VASA ◽  
2004 ◽  
Vol 33 (2) ◽  
pp. 78-81 ◽  
Author(s):  
Thalhammer ◽  
Aschwanden ◽  
Jeanneret ◽  
Labs ◽  
Jäger

Background: Haemostatic puncture closure devices for rapid and effective hemostasis after arterial catheterisation are a comfortable alternative to manual compression. Implanting a collagen plug against the vessel wall may become responsible for other kind of vascular injuries i.e. thrombotic or stenotic lesions and peripheral embolisation. The aim of this paper is to report our clinically relevant vascular complications after Angio-Seal® and to discuss the results in the light of the current literature. Patients and methods: We report the symptomatic vascular complications in 17 of 7376 patients undergoing diagnostic or therapeutic catheterisation between May 2000 and March 2003 at the University Hospital Basel. Results: Most patients presented with ischaemic symptoms, arterial stenoses or occlusions and thrombotic lesions (n = 14), whereas pseudoaneurysms were extremely rare (n = 3). Most patients with ischaemic lesions underwent vascular surgery and all patients with a pseudoaneurysm were successfully treated by ultrasound-guided compression. Conclusions: Severe vascular complications after Angio-Seal® are rare, consistent with the current literature. There may be a shift from pseudoaneurysms to ischaemic lesions.


1993 ◽  
Vol 32 (05) ◽  
pp. 365-372 ◽  
Author(s):  
T. Timmeis ◽  
J. H. van Bemmel ◽  
E. M. van Mulligen

AbstractResults are presented of the user evaluation of an integrated medical workstation for support of clinical research. Twenty-seven users were recruited from medical and scientific staff of the University Hospital Dijkzigt, the Faculty of Medicine of the Erasmus University Rotterdam, and from other Dutch medical institutions; and all were given a written, self-contained tutorial. Subsequently, an experiment was done in which six clinical data analysis problems had to be solved and an evaluation form was filled out. The aim of this user evaluation was to obtain insight in the benefits of integration for support of clinical data analysis for clinicians and biomedical researchers. The problems were divided into two sets, with gradually more complex problems. In the first set users were guided in a stepwise fashion to solve the problems. In the second set each stepwise problem had an open counterpart. During the evaluation, the workstation continuously recorded the user’s actions. From these results significant differences became apparent between clinicians and non-clinicians for the correctness (means 54% and 81%, respectively, p = 0.04), completeness (means 64% and 88%, respectively, p = 0.01), and number of problems solved (means 67% and 90%, respectively, p = 0.02). These differences were absent for the stepwise problems. Physicians tend to skip more problems than biomedical researchers. No statistically significant differences were found between users with and without clinical data analysis experience, for correctness (means 74% and 72%, respectively, p = 0.95), and completeness (means 82% and 79%, respectively, p = 0.40). It appeared that various clinical research problems can be solved easily with support of the workstation; the results of this experiment can be used as guidance for the development of the successor of this prototype workstation and serve as a reference for the assessment of next versions.


1995 ◽  
Vol 34 (01/02) ◽  
pp. 75-78 ◽  
Author(s):  
R. D. Appel ◽  
O. Golaz ◽  
Ch. Pasquali ◽  
J.-C. Sanchez ◽  
A. Bairoch ◽  
...  

Abstract:The sharing of knowledge worldwide using hypermedia facilities and fast communication protocols (i.e., Mosaic and World Wide Web) provides a growth capacity with tremendous versatility and efficacy. The example of ExPASy, a molecular biology server developed at the University Hospital of Geneva, is striking. ExPASy provides hypermedia facilities to browse through several up-to-date biological and medical databases around the world and to link information from protein maps to genome information and diseases. Its extensive access is open through World Wide Web. Its concept could be extended to patient data including texts, laboratory data, relevant literature findings, sounds, images and movies. A new hypermedia culture is spreading very rapidly where the international fast transmission of documents is the central element. It is part of the emerging new “information society”.


2019 ◽  
Author(s):  
M Stättermayer ◽  
F Riedl ◽  
S Bernhofer ◽  
A Stättermayer ◽  
A Mayer ◽  
...  

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