scholarly journals Spontaneous remission of localized myasthenia gravis manifested as megaesophagus in a dog

2017 ◽  
Vol 61 (1) ◽  
pp. 36
Author(s):  
N. SOUBASIS (Ν. ΣΟΥΜΠΑΣΗΣ) ◽  
M. E. MYLONAKIS (Μ.Ε. ΜΥΛΩΝΑΚΗΣ) ◽  
M. N. PATSIKAS (Μ.Ν. ΠΑΤΣΙΚΑΣ) ◽  
Z. POLIZOPOULOU (Ζ. ΠΟΛΥΖΟΠΟΥΛΟΥ) ◽  
A. F. KOUTINAS (Α.Φ. ΚΟΥΤΙΝΑΣ)

A 2.5-year-old male German shepherd-cross dog was admitted for progressively deteriorating regurgitations ofone-month duration. Physical examination was unremarkable, apart from a poor body condition; also, neurological examination did not reveal any abnormality. In thoracic survey radiographs, a generalized esophageal dilation, consistent with megaesophagus, was well visualized. An increased nicotinic acetylcholine receptor antibody titer confirmed the diagnosis of acquired focal myasthenia gravis expressed as megaesophagus in this patient. The only help offered to the dog was a modification of its feeding habits (upright position, small and frequent meals). Within two months of the initial admission and prior to institution of specific treatment for myasthenia gravis, the dog experienced spontaneous and complete clinical remission and remained healthy for the next two years, eating again the normal way, but unfortunately was lost to follow-up. The favourable outcome of megaesophagus due to localized myasthenia gravis is discussed accordingly.

2018 ◽  
Vol 52 (4) ◽  
pp. 273 ◽  
Author(s):  
M. V. LIAPI (Μ.Β. ΛΙΑΠΗ) ◽  
M. N. SARIDOMICHELAKIS (Μ.Ν. ΣΑΡΙΔΟΜΙΧΕΛΑΚΗΣ) ◽  
A. F. KOUTINAS (Α. Φ. ΚΟΥΤΙΝΑΣ) ◽  
S. LEKKAS (Σ. ΛΕΚΚΑΣ)

Pemphigus folliaceus (PF), which is considered the most common autoimmune skin disease in the dog, is associated with the formation of autoantibodies directed against the desmosomes of the upper third of the epidermis, that result in loss of keratinocyte adhesion (acantholysis) and subsequent epidermal cleft formation. The clinical and histopathological findings, along with the response to treatment in 5 PF cases, either idiopathic (3/5) or secondary to trimethoprim-sulfonamides (1/5) and to allergic dermatitis (1/5), are presented and discussed, accordingly. All the 5 dogs exhibited hypotrichosis-alopecia (4/5), erythema (4/5), vesicles (1/5), pustules (5/5), crusts (5/5), epidermal collarettes (3/5), excoriations (1/5), erosions (3/5), ulcers (1/5), fissures (1/5), lichenification, thickening and folding of the skin (1/5), and footpad hyperkeratosis (1/5), that were pruritic in 3 of them. These lesions were located on the head (4/5), pinnae (4/5), limbs (3/5), body trunk (3/5) and the footpads (2/5). Diagnosis was based on the results of lesionai histopathology where the most striking finding was the presence of subcorneal pustules filled with normally looking neutrophils and acanthocytes. Moreover, pustule or crust cytology that was carried out in 3 cases, revealed clusters of acantholytic cells in 2 dogs. The treatment, that was instituted in 2/5 dogs and resulted in a complete resolution of skin lesions, was based on prednisolone in immunosuppressive dosage along with azathioprine (PF secondary to allergic dermatitis), or on a combination of dapsone, Oxytetracycline and vitamin E (idiopathic PF). The remaining 3 cases were left untreated, because one showed a spontaneous remission following withdrawal of the offending medication and the other 2 were lost to follow-up.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1493-1493
Author(s):  
Alan H. Bryce ◽  
Angela Dispenzieri ◽  
Robert A. Kyle ◽  
Martha Q. Lacy ◽  
Morie A. Gertz

Abstract Background: Cryoglobulinemia(CG) is a relatively uncommon disorder which has been recognized as a syndrome for nearly forty years. The further classification into types I, II, and III was established thirty years ago, with Type II consisting of a monoclonal immunoglobulin possessing activity towards a polyclonal component. In the interim, many disease associations have been described and various therapies tried, but clinical trials remain rare, and the natural history, causes, and pathways of CG require further investigation. Methods: We examined the records of all patients seen at the Mayo Clinic, Rochester, with Type II CG between 3/2/1994 and 11/27/2000 using a prospectively held dysproteinemia database. Results: Of 66 total patients 30 were male(45%) and 36 were female(55%) with mean ages of 52.7±11.0 and 60.9±12.6, respectively. 63 patients were Caucasian, with one each of African, Hispanic, and other. 34 patients(52%) presented with purpura, 16 with edema(24%), 12 with neuropathy(18%), 12 with fatigue, 10 with arthralgia(15%), 8 with weight loss(12%), 5 with livido reticularis(8%), 4 with leg ulcers(6%), and 2 with infarcted extremities(2%). Of our patients, 13 had Type II CG (20%) unassociated with infection or lymphoma. 38 patients(58%) had hepatitis C(HCV), of whom 12 underwent genotyping with 10 having genotype 1a or 1b and 2 having genotype 2a or 2b. No patients had isolated Hepatitis B(HBV) though 8 HCV patients were coinfected with HBV. One patient each had CMV and EBV. 7 patients(10%) had Sjogren’s disease, and 1 had scleroderma. 11 patients (17%) had a lymphoproliferative disorder (LPD), and 5 more went on to develop LPD. Of these, 7 patients had a B-cell LPD, 4 had a MALT lymphoma, 4 had a non specific LPD, one had a lymphoplasmacytic lymphoma, and one had Gaucher’s thrombocytopenia. Significant renal disease developed in 23 patients(35%), with 12 being membranoproliferative glomerulonephritis (18%) and 3 being mesangioproliferative glomerulonephritis. Pertinent laboratory finding included a mean cryocrit of 7.6%±12.5, a positive rheumatoid factor in 36 out of 36, and hypocomplementemia in 33 of 41(80%). Liver function testing was reflective of the patient’s hepatitis status. Serum electrophoresis in 48 patients revealed the following patterns: 22 were normal(46%), 15 had a γ peak(31%), 5 polyclonal patterns(10%), 5 hypogammaglobulinemia, and one a β-γ peak. Of 32 samples where a monoclonal antibody was isolated 29 were IgM (91%), and 3 IgG (9%). The light chains were 88% κ, with 12% λ. Treatment was generally guided by the comorbidities, and many (30%) required no specific treatment for Type II CG. In the 38 HCV patients, 17 received interferon with or without ribavirin and 18 were deemed not to need treatment, while 2 received steroids alone and 1 was lost to follow up. In 28 patients without HCV 20 received steroids(71%), while 5 received other treatments and 3 were untreated. Of 46 patients who received any treatment, 27 (59%) achieved no response or had disease progression and required a change in therapy. To date, 8 patients have died, with none of deaths specifically attributable to complications of cryoglobulinemia. Conclusion: Type II CG is a non fatal disease most frequently associated with HCV. Optimal disease specific treatment is as yet undefined, and is generally directed at the underlying comorbidity when possible. Not all cases require treatment and many can be followed symptomatically.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 871.2-872
Author(s):  
E. Luchikhina ◽  
D. Karateev ◽  
A. Novikov ◽  
G. Lukina ◽  
E. Aleksandrova ◽  
...  

Background:A group of patients with recent onset of arthritis always presents a certain difficulty for the practitioner due to its marked heterogeneity. This especially concerns the patients with undifferentiated inflammatory arthritis (UIA).Objectives:To characterize the long-term nosological outcome of clinical and immunological subtypes of early UIA.Methods:A cohort study of patients with recent onset of arthritis with a duration of not more than 12 months (n = 761, 82.8% females, 55.2% RF +) was initiated in 2003. All patients were referred to rheumatologist from GP with suspicion on inflammatory arthritis. Inclusion criteria: age 18 years or more, at least 1 swollen joint at 1stvisit and/or positive squeeze test or morning stiffness for at least 30 minutes. 243 patients were initially classified as having UIA: 46 (18,9%) males, 197 (81,1%) females, age (median, quartiles)- 44 [29;53] years, duration of symptoms- 3 [2;5] months, swollen joints count (66/68)- 2 [1;4], tender joints count 3 [2;6], 85 (35%) rheumatoid factor (RF)+, 63 (25,9%) anti-CCP+. Patients with UIA were followed up for at least 10 years.Results:28 (11,5%) of patients were lost to follow up. Long-term observation was conducted in 215 patients. 22 (10,2%) of them developed persistent spontaneous (without use of DMARDs) remission. Other patients were finally classified as having: rheumatoid arthritis (RA) 138 (64,2%) and non-RA (25,6%). The spectrum of diagnoses in non-RA group included: ankylosing spondylitis, osteoarthritis, gout, psoriatic arthritis, polymyalgia rheumatica, systemic lupus, mixed connective tissue disease, sarcoidosis, paraneoplastic syndromes. The following characteristics at baseline positively correlated with the development of RA: arthritis of 3 or more joints (p=0,011), arthritis of hand (p=0,005), high ESR (p<0,001), high CRP (p=0,003), PF-positivity (p<0,001), anti-CCP-positivity (p<0,001), older age of onset (p=0,019), higher levels of RF IgM (p=0,027) and anti-CCP (p<0,001). Development of persistent spontaneous remission negatively correlated with polyarthritis (p=0,033), PF-positivity (p=0,034), anti-CCP-positivity (p=0,001). Positive seroconversion was observed: of RF in 10 (4,7%) patients, 8 developed RA, of anti-CCP – in 3 (1,4%) patients, all developed RA.Table.Outcome of different subtypes of patients with UA after 10 years of follow up (n=215).Subtypes of UAOutcomeP (Pearson chi-squared test)Non-RA (n=55)RA (n=138)Spontaneous remission (n=22)Mono- and oligo arthritis (n=140)40 (28,6%)81 (57,9%)19 (13,6%)0,016Polyarthritis (n=75)15 (20%)57 (76%)3 (4%)RF-negative AND anti-CCP-negative (n=90)34 (37,8%)41 (45,6%)15 (16,7%)<0,001RF+ OR anti-CCP+ OR low levels (<3 ULN) (n=92)20 (21,7%)65 (70,7%)7 (7,6%)High RF+ AND High anti-CCP+ (n=33)1 (3%)32 (97%)0Conclusion:Seronegative oligoarticular disease and highly seropositive disease are different subtypes of UIA. Combination of seronegativity and oligoarticular disease (n=52) associated with relatively rare development of RA (36,2%) and high proportion of spontaneous remission (22,4%). Patients who were highly positive (>3 ULN) for both RF and anti-CCP developed RA in 97% of cases and never remitted spontaneously.Disclosure of Interests:Elena Luchikhina Consultant of: Abbvie, Biocad, Sanofi, Celgene, Speakers bureau: Abbvie, Roche, Pfizer, Biocad, MSD, Sanofi, Johnson & Johnson, Glaxo, UCB, Celgene, Novartis, Dmitry Karateev Consultant of: Abbvie, Pfizer, Biocad, Sanofi, Novartis, Lilly, Speakers bureau: Abbvie, Roche, Pfizer, Biocad, MSD, Sanofi, Johnson & Johnson, Glaxo, UCB, Celgene, Novartis, Lilly, Bayer, Alexander Novikov: None declared, Galina Lukina Speakers bureau: Novartis, Pfizer, UCB, Abbvie, Biocad, MSD, Roche, Elena Aleksandrova: None declared, Natalia Demidova: None declared


F1000Research ◽  
2021 ◽  
Vol 9 ◽  
pp. 1045
Author(s):  
Syrine Mannoubi ◽  
Med Amin Mesrati ◽  
Ibn Hadj Amor Hassen ◽  
Taha Hasnaoui ◽  
Hiba Limem ◽  
...  

Ventricular non-compaction (VNC) is a rare myocardium disorder, which can be both genetic and sporadic. A poor wall compaction process or an excessive trabeculae formation may be at the genesis of myocardial hypertrabeculation with multiple recesses. It is often complicated by ventricular dysfunction, arrhythmias and cardiac embolism. Herein we report a case of a 20-year-old male patient with no particular past medical history who was followed up at the cardiology department for dyspnea. Echocardiography showed reduced ejection fraction of the left ventricle with potential hypertrabeculation in the right ventricle, confirmed by cardiac MRI. The patient was not put under medication and was later lost to follow-up. He died few months later without a clear cause explaining death. A forensic autopsy was performed that attributed death to acute ventricle arrhythmia secondary to VNC, emphasizing the major role of an early and specific treatment to avoid such a fatal outcome.


F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 1045
Author(s):  
Syrine Mannoubi ◽  
Med Amin Mesrati ◽  
Ibn Hadj Amor Hassen ◽  
Taha Hasnaoui ◽  
Hiba Limem ◽  
...  

Ventricular non-compaction (VNC) is a rare myocardium disorder, which can be both genetic and sporadic. A poor wall compaction process or an excessive trabeculae formation may be at the genesis of myocardial hypertrabeculation with multiple recesses. It is often complicated by ventricular dysfunction, arrhythmias and cardiac embolism. Herein we report a case of a 20-year-old male patient with no particular past medical history who was followed up at the cardiology department for dyspnea. Echocardiography showed reduced ejection fraction of the left ventricle with potential hypertrabeculation in the right ventricle, confirmed by cardiac MRI. The patient was not put under medication and was later lost to follow-up. He died few months later without a clear cause explaining death. A forensic autopsy was performed that attributed death to acute ventricle arrhythmia secondary to VNC, emphasizing the major role of an early and specific treatment to avoid such a fatal outcome.


2009 ◽  
Vol 40 (12) ◽  
pp. 13
Author(s):  
ALAN ROCKOFF
Keyword(s):  

2013 ◽  
Author(s):  
Danielle M. Lespinasse ◽  
Kristen E. Medina ◽  
Stacey N. Maurer ◽  
Samantha A. Minski ◽  
Renee T. Degener ◽  
...  

1971 ◽  
Vol 10 (01) ◽  
pp. 39-46
Author(s):  
C. Alexandrou ◽  
E. Papadakis ◽  
E. Gyftaki ◽  
J. Darsinos

SummaryRadioisotope renograms were obtained in the upright and prone position in 9 normal subjects, in 5 patients with untreated essential hypertension and in 21 hypertensives under treatment, showing moderate postural hypotension.No significant renographic change were seen in the two positions in normal subjects and untreated hypertensives. Treated hypertensives with postural hypotension showed significant impairment of renal function in the upright position in 15 cases and no change in 6. Renal creatinine clearance was lower in the group that showed renographic changes. Renography in the upright position is suggested as a convenient test for early diagnosis and follow-up of the adverse effects of antihypertensive treatment.


2018 ◽  
Vol 1 (1) ◽  
pp. 1
Author(s):  
Wawan Mulyawan ◽  
Yudi Yuwono Wiwoho ◽  
Syaiful Ichwan

Background: Following surgical treatments for low back pain, lower extremity pain or neurologic symptoms would last or recur, this is defined as failed sack surgery syndrome (FBSS). FBSS usually occurs in 5-40% of these surgical patients. The most common cause is an epidural scar adhesion. Percutaneous epidural neuroplasty is the non-mechanical treatment for this condition. Previously, the use of hyaluronidase and hypertonic saline separately is commonly used for epidurolysis but the combination of hyaluronidase and hypertonic saline 3% has not been explored.Objective: To investigate the two-year outcomes of percutaneous epidural neuroplasty using a combination of hyaluronidase and hypertonic saline 3% in patients with FBSS.Methods: Twelve patients who experience low back pain, with or without radiculopathy, who have underwent lumbar spine surgery previously were assigned to the study. Parameters, such as the visual analogue scale scores for the back (VAS-B) and legs (VAS-L), and the Oswestry disability index (ODI), were recorded and compared between pretreatment, 1 week, 1 month, 3 months, 1 year and 2 years follow-up.Results: For all 12 patients, the postoperative VAS-B, VAS-L, and ODI were significantly different from the preoperative values in all follow-up periods: 1 month, 3 months, 1 year, and 2 years.Conclusion: Based off this study group, percutaneous epidural neuroplasty using a combination of hyaluronidase and hypertonic saline 3% has a favourable outcome in the 2 years follow-up


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