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  1. Fulltext Kawasaki Disease with Hepatobiliary Manifestations
    Suhaini SA, Azidin AH, Cheah CS, Lee Wei Li W, Khoo MS, Ismail NAS, et al.
    Medicina (Kaunas), 2022 Dec 12;58(12).
    PMID: 36557034 DOI: 10.3390/medicina58121833
    Background and Objectives: Kawasaki Disease (KD) incidence has been on the rise globally throughout the years, particularly in the Asia Pacific region. KD can be diagnosed based on several clinical criteria. Due to its systemic inflammatory nature, multi-organ involvement has been observed, making the diagnosis of KD more challenging. Notably, several studies have reported KD patients presenting with hepatobiliary abnormalities. Nonetheless, comprehensive data regarding the hepatobiliary manifestations of KD are limited in Malaysia, justifying a more in-depth study of the disease in this country. Thus, in this article, we aim to discuss KD patients in Malaysia with hepatobiliary manifestations. Materials and Methods: A total of six KD patients with hepatobiliary findings who presented at Hospital Canselor Tuanku Muhriz (HCTM) from 2004 to 2021 were selected and included. Variables including the initial presenting signs and symptoms, clinical progress, laboratory investigations such as liver function test (LFT), and ultrasound findings of hepatobiliary system were reviewed and analyzed. Results: Out of these six KD patients, there were two patients complicated with hepatitis and one patient with gallbladder hydrops. Different clinical features including jaundice (n = 3) and hepatomegaly (n = 4) were also observed. All patients received both aspirin and intravenous immunoglobulin (IVIG) as their first-line treatment and all of them responded well to IVIG. The majority of them (n = 5) had a complete recovery and did not have any cardiovascular and hepatobiliary sequelae. Conclusions: Despite KD mostly being diagnosed with the classical clinical criteria, patients with atypical presentations should always alert physicians of KD as one of the possible differential diagnoses. This study discovered that hepatobiliary manifestations in KD patients were not uncommon. More awareness on the epidemiology, diagnosis, and management of KD patients with hepatobiliary manifestations are required to allow for the initiation of prompt treatment, thus preventing further complications.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  2. Factors associated with outcomes of severe acute necrotizing encephalopathy: A multicentre experience in Malaysia
    Lee VWM, Khoo TB, Teh CM, Heng HS, Li L, Yusof YLM, et al.
    Dev Med Child Neurol, 2023 Sep;65(9):1256-1263.
    PMID: 36748407 DOI: 10.1111/dmcn.15536
    This case series compared clinical variables and various combinations of immunotherapy received with outcomes of patients with severe acute necrotizing encephalopathy (ANE). We performed a retrospective review of clinical variables, immunotherapy received, and outcomes (based on the modified Rankin Scale) in Malaysia between February 2019 and January 2020. Twenty-seven children (12 male), aged 7 months to 14 years (mean 4 years) at diagnosis were included. Of these, 23 had an ANE severity score of 5 to 9 out of 9 (high risk). Eleven patients received tocilizumab (four in combination with methylprednisolone [MTP], seven with MTP + intravenous immunoglobulin [IVIG]) and 16 did not (two received MTP alone, 14 received MTP + IVIG). Nine died. Among the survivors, six had good outcomes (modified Rankin Score 0-2) at 6 months follow-up. All patients who received tocilizumab in combination with MTP + IVIG survived. Twenty children received first immunotherapy within 48 hours of admission. No significant association was found between the timing of first immunotherapy with outcomes. Those with brainstem dysfunction (p = 0.016) were observed to have poorer outcomes. This study showed a trend towards better survival when those with severe ANE were treated with tocilizumab in combination with MTP + IVIG. However, larger studies will be needed to determine the effect of this regime on the long-term outcomes.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  3. Fulltext Immunodeficiency and Thymoma: A Case Report on Good Syndrome, a Diagnosis Frequently Missed and Forgotten
    Tang ASO, Loh WH, Wong QY, Yeo ST, Ng WL, Teoh PI, et al.
    Am J Case Rep, 2021 Mar 13;22:e928659.
    PMID: 33712551 DOI: 10.12659/AJCR.928659
    BACKGROUND Good syndrome (thymoma with immunodeficiency) is a frequently missed and forgotten entity. It is a rare cause of combined B and T cell immunodeficiency in adults. To date, fewer than 200 patients with Good syndrome have been reported in the literature. CASE REPORT We report a case of type AB Masaoka-Koga stage I thymoma which predated the evidence of immune dysregulation by 5 years, manifesting as bilateral cytomegalovirus retinitis, multiple bouts of pneumonia, and bronchiectasis in a HIV-seronegative 55-year-old man. Intravitreal ganciclovir was administered in addition to intravenous systemic ganciclovir, which resulted in severe neutropenic sepsis. A thorough immunodeficiency workup confirmed the presence of hypogammaglobulinemia with complete absence of B cells and reduced CD4/CD8 ratio. The patient responded well to monthly intravenous immunoglobulin replacement therapy, with no further episodes of infection since then. The immunoglobulin level doubled after 1 year of treatment. However, as the patient refused further intravitreal and CMV-targeted treatment, his vision did not recover. CONCLUSIONS Clinicians should be aware that thymoma can precede the onset of immunodeficiency. Clinical suspicion should be heightened in at-risk patients who present with multiple bouts of infection, particularly in thymoma cases with adult-onset immune dysfunction. It is of paramount importance to follow up those patients with annual clinical reviews and immunodeficiency screening.
    Matched MeSH terms: Immunoglobulins, Intravenous
  4. Epidemiology, clinical characteristics, and immediate outcome of Kawasaki disease: a population-based study from a tropical country
    Mat Bah MN, Alias EY, Razak H, Sapian MH, Foo FH, Abdullah N
    Eur J Pediatr, 2021 Aug;180(8):2599-2606.
    PMID: 34086103 DOI: 10.1007/s00431-021-04135-7
    Data on Kawasaki disease from tropical countries are scarce. Hence, this population-based study aims to determine the epidemiology, clinical characteristics, and outcome of Kawasaki disease in children enrolled in the Kawasaki disease registry between 2006 and 2019 in Southern Malaysia. Diagnosis of Kawasaki disease was made using standard criteria. Primary outcome measure was a coronary artery aneurysm. Multivariable logistic regression was used to analyze the associated risk factors for coronary artery aneurysm. There were 661 Kawasaki disease, with 27% incomplete and 11% atypical presentations. Male-to-female ratio was 2:1, and median age of diagnosis was 1.4 years (interquartile range 9 to 32 months). Incidence in children of less than 5 years was 14.8 (95% confidence interval [CI]: 13.6 to 16.0) per 100,000 population, higher in males (19/100,000) and Chinese (22/100,000), with a gradual increase from 5.7/100,000 in 2006 to 19.6/100,000 in 2019, p < 0.001. Incidence in children between 5 and 9 years old was 1.3 (95% CI: 0.9 to 1.6) per 100,000 population and remained stable over time. There was a seasonal pattern with peak incidence during the rainy season. Out of 625 intravenous immunoglobulins (IVIG)-treated Kawasaki disease, 7.4% were resistant, and 9% had coronary artery aneurysms. Atypical presentation, male sex, late diagnosis, and IVIG resistance were independent risk factors for coronary artery aneurysms.Conclusions: Despite the tropical climate, Kawasaki disease epidemiology is similar to non-tropic regions with seasonal patterns and a rising incidence. Atypical presentation, male sex, late diagnosis, and IVIG resistance were significantly associated with coronary artery aneurysms. What is Known: • Kawasaki disease predominantly occurs in males, children less than 5 years old, and the Asian population. • Male sex, late diagnosis, incomplete Kawasaki disease, and IVIG resistance were associated with coronary artery aneurysms. What is New: • In multi-ethnic Asian countries such as Malaysia, ethnic Chinese have a higher prevalence of Kawasaki disease compared to other ethnicities. • Kawasaki disease with atypical presentation can occur in both complete and incomplete Kawasaki disease and is significantly associated with a coronary artery aneurysm.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  5. A Systematic Review and Meta-regression Analysis on the Impact of Increasing IgG Trough Level on Infection Rates in Primary Immunodeficiency Patients on Intravenous IgG Therapy
    Lee JL, Mohamed Shah N, Makmor-Bakry M, Islahudin FH, Alias H, Noh LM, et al.
    J Clin Immunol, 2020 07;40(5):682-698.
    PMID: 32417999 DOI: 10.1007/s10875-020-00788-5
    PURPOSE: We conducted a systematic review and meta-regression analysis to evaluate the impact of increasing immunoglobulin G (IgG) trough levels on the clinical outcomes in patients with PID receiving intravenous immunoglobulin G (IVIG) treatment.

    METHODS: Systematic search was conducted in PubMed and Cochrane. Other relevant articles were searched by reviewing the references of the reviewed article. All clinical trials with documented IgG trough levels and clinical outcome of interest in patients receiving IVIG treatment were eligible to be included in this review. Meta-regression analysis was conducted using Comprehensive Meta-analysis Software. Additional sensitivity analyses were undertaken to evaluate the robustness of the overall results.

    RESULTS: Twenty-eight clinical studies with 1218 patients reported from year 2001 to 2018 were included. The mean IVIG dose used ranges from 387 to 560 mg/kg every 3 to 4 weekly, and mean IgG trough obtained ranges from 660 to 1280 mg/dL. Random-effects meta-regression slope shows that IgG trough level increases significantly by 73 mg/dL with every increase of 100 mg/kg dose of IVIG (p 

    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  6. Fulltext Different IVIG glycoforms affect in vitro inhibition of anti-ganglioside antibody-mediated complement deposition
    Sudo M, Yamaguchi Y, Späth PJ, Matsumoto-Morita K, Ong BK, Shahrizaila N, et al.
    PLoS One, 2014;9(9):e107772.
    PMID: 25259950 DOI: 10.1371/journal.pone.0107772
    Intravenous immunoglobulin (IVIG) is the first line treatment for Guillain-Barré syndrome and multifocal motor neuropathy, which are caused by anti-ganglioside antibody-mediated complement-dependent cytotoxicity. IVIG has many potential mechanisms of action, and sialylation of the IgG Fc portion reportedly has an anti-inflammatory effect in antibody-dependent cell-mediated cytotoxicity models. We investigated the effects of different IVIG glycoforms on the inhibition of antibody-mediated complement-dependent cytotoxicity. Deglycosylated, degalactosylated, galactosylated and sialylated IgG were prepared from IVIG following treatment with glycosidases and glycosyltransferases. Sera from patients with Guillain-Barré syndrome, Miller Fisher syndrome and multifocal motor neuropathy associated with anti-ganglioside antibodies were used. Inhibition of complement deposition subsequent to IgG or IgM autoantibody binding to ganglioside, GM1 or GQ1b was assessed on microtiter plates. Sialylated and galactosylated IVIGs more effectively inhibited C3 deposition than original IVIG or enzyme-treated IVIGs (agalactosylated and deglycosylated IVIGs). Therefore, sialylated and galactosylated IVIGs may be more effective than conventional IVIG in the treatment of complement-dependent autoimmune diseases.
    Matched MeSH terms: Immunoglobulins, Intravenous/immunology*; Immunoglobulins, Intravenous/metabolism; Immunoglobulins, Intravenous/pharmacology
  7. What are the effects of intravenous immunoglobulins on seizures and quality of life of people with epilepsy? A Cochrane Review summary with commentary
    Ahmad Fauzi A, Patrick Engkasan J
    Dev Med Child Neurol, 2021 05;63(5):501-502.
    PMID: 33559130 DOI: 10.1111/dmcn.14835
    Matched MeSH terms: Immunoglobulins, Intravenous
  8. Fulltext High dose intravenous immunoglobulin for systemic lupus erythematosus with lupus nephritis and associated studies - case report
    Lim SK, Tan SY
    JUMMEC, 2007;10(2):51-56.
    MyJurnal
    Systemic lupus erythematosus (SLE) is one of the commonest systemic autoimmune diseases that can present with variable clinical manifestations. Intravenous Immunoglobulin (IVIG) has been used as a salvage therapy for severe lupus with encouraging results though there is yet randomised trial to support the usage. This report highlights the efficacy and safety of high dose IVIG in SLE patients with multi-organ involvement particularly lupus nephritis. We also reviewed the literature on the usage of IVIG for lupus nephritis. However, more studies are needed to further clarify the optimal therapeutic dosage and regime for IVIG and to identify the group of patients who might benefit the most from this expensive therapy.
    Matched MeSH terms: Immunoglobulins, Intravenous
  9. Fulltext Diabetic neuropathic cachexia in a young woman
    Yusof NA, Idris NS, Zin FM
    Korean J Fam Med, 2018 Nov 30.
    PMID: 30497111 DOI: 10.4082/kjfm.17.0127
    Profound weight loss with painful symmetrical peripheral neuropathy in diabetic patients was first described as diabetic neuropathic cachexia more than 4 decades ago. It is a distinct type of diabetic peripheral neuropathy that occurs in the absence of other microvascular and autonomic complications of diabetes. The mechanism and precipitating cause are unknown. It was reported to have good prognosis with spontaneous recovery within months to 2 years. However, it was frequently missed by clinicians because the profound weight loss is the most outstanding complaint, rather than the pain, numbness, or weakness. This often leads to extensive investigation to exclude more sinister causes of weight loss, particularly malignancy. We report a case of a young woman with well-controlled diabetes who presented with profound unintentional weight loss (26 kg), symmetrical debilitating thigh pain, and clinical signs of peripheral neuropathy. As the disease entity may mimic an inflammatory demyelinating cause of neuropathy, she was treated with a trial of intravenous immunoglobulin, which failed to give any significant benefit. However, she recovered after 6 months without any specific treatment, other than an antidepressant for the neuropathic pain and ongoing rehabilitation.
    Matched MeSH terms: Immunoglobulins, Intravenous
  10. Delayed diagnosis of Kawasaki disease in Malaysia: Who is at risk and what is the outcome
    Mat Bah MN, Alias EY, Sapian MH, Abdullah N
    Pediatr Int, 2022 Jan;64(1):e15162.
    PMID: 35789141 DOI: 10.1111/ped.15162
    BACKGROUND: Data on the delayed diagnosis of (KD) is limited. This study aimed to determine the prevalence and trend of delayed diagnosis over time and identify the associated risk factors in Malaysia.

    METHODS: This retrospective, observational, population-based study involved all children with KD registered in Johor Kawasaki Clinical Registry over 10 years (January 2010 and December 2019). The prevalence of delayed diagnosis and its trend over time was calculated. Multivariable binary logistic regression was used to identify the independent risk factors for delayed diagnosis.

    RESULTS: There were 556 cases of KD, with 28% having incomplete criteria, 11% atypical presentation, while 10% developed a coronary aneurysm. The overall prevalence of delayed diagnosis was 9.9% (95% confidence interval (CI): 7.6-12.7%). There was a statistically significant decrease in delayed diagnosis over time (P = 0.008), with the latest rate of 4.6%. The majority of delayed diagnoses were due to failure to diagnose the disease during the initial consultation. Independent risk factors for delayed diagnosis were children older than 1 year, diagnosis before 2015, atypical presentation, and incomplete KD, with adjusted odds ratios (ORs) of 2.7, 2.3, 4.3, and 3.6, respectively. Compared to early diagnosis of KD, delayed diagnosis was significantly associated with coronary aneurysms (27.3% vs. 8.2%, P < 0.001, OR 4.2, [95% CI: 2.1-8.3]).

    CONCLUSIONS: One-tenth of cases of KD were diagnosed late, but it has improved over time. Children > 1 year, presenting with atypical presentation, and incomplete criteria are associated with late diagnosis.

    Matched MeSH terms: Immunoglobulins, Intravenous
  11. The role of immunotherapy in Guillain-Barré syndrome: understanding the mechanism of action
    Shahrizaila N, Yuki N
    Expert Opin Pharmacother, 2011 Jul;12(10):1551-60.
    PMID: 21473704 DOI: 10.1517/14656566.2011.564160
    Guillain-Barré syndrome (GBS) is the most frequent cause of acute flaccid paralysis and, despite treatment, there continues to be an associated mortality and severe disability ranging from 9 to 17%. This article reviews the rationale behind the existing immunotherapy in GBS and discusses the future direction that work in this area should follow.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  12. Periodic lateralized epileptiform discharges in neuropsychiatric lupus: association with cerebritis in magnetic resonance imaging and resolution after intravenous immunoglobulin
    Lim KS, Cheong KL, Tan CT
    Lupus, 2010 May;19(6):748-52.
    PMID: 20133346 DOI: 10.1177/0961203309351539
    A 13-year-old girl with a known diagnosis of systemic lupus erythematosus presented with seizures and psychosis. An electroencephalogram (EEG) revealed continuous, non-evolving periodic lateralized epileptiform discharges (PLEDs) in the left temporal region, which did not resolve with benzodiazepine. A magnetic resonance imaging (MRI) brain scan demonstrated a focal hyperintensity in the left medial temporal and left occipital lobes, left thalamus and bilateral cerebellar white matter, with evidence of vasculitis in the magnetic resonance angiography. Intravenous immunoglobulin was given because of failed steroid therapy, which resulted in a full resolution of clinical, EEG and MRI abnormalities. Lupus cerebritis should be considered as a possible aetiology in PLEDs, and immunoglobulin can be effective in neuropsychiatric lupus.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  13. Fulltext Intravenous immunoglobulin in the treatment of acute disseminated encephalomyelitis
    Kanaheswari Y, Baizura J, Zulfiqar A
    Med J Malaysia, 2004 Mar;59(1):103-7.
    PMID: 15535344
    We describe a case of acute disseminated encephalomyelitis in a child. This case is unusual in that the illness was characterised by recurrent episodes rather than a monophasic course and that the choice of treatment was intravenous immunoglobulin over corticosteroids. The rapid and remarkable recovery is highlighted and a review of the treatment for this rare condition is discussed.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  14. Fulltext Intravenous immunoglobulin in the therapeutic armamentarium of systemic lupus erythematosus: a systematic review and meta-analysis
    Sakthiswary R, D'Cruz D
    Medicine (Baltimore), 2014 Oct;93(16):e86.
    PMID: 25310743 DOI: 10.1097/MD.0000000000000086
    Prepared from the plasma of thousands of blood donors, therapeutic intravenous immunoglobulin (IVIg) mostly consists of human polyspecific immunoglobulin G (IgG). The use of IVIg in systemic lupus erythematosus (SLE) is still considered experimental without any clear indications. The purpose of this systematic review is, therefore, to evaluate the available evidence to determine the therapeutic role of IVIg in SLE. A comprehensive, computerised search was performed in the MEDLINE (Pubmed), Scopus, EMBASE, and Cochrane controlled trials. The study eligibility criteria were randomized controlled trials, and prospective and retrospective observational studies that examined the efficacy of IVIg in adult patients with SLE who were considered the participants.IVIg therapy was the mode of intervention in these patients. Data abstracted included the study design, study population, changes in the disease activity scores (Systemic Lupus Erythematosus Disease Activity Index, Systemic Lupus Activity Measure, and Lupus Activity Index-Pregnancy), steroid dose, complement levels, autoantibodies, and renal function. Thereafter, data analysis established statistical procedures for meta-analysis. Thirteen studies (including 3 controlled and 10 observational) were eligible for inclusion. There was significant reduction in the SLE disease activity scores with IVIg therapy with a standard mean difference of 0.584 (P = 0.002, 95% confidence interval [CI] 0.221-0.947). In terms of rise in complement levels, the response rate was 30.9% (P = 0.001, 95 CI 22.1-41.3). The effects of IVIg on other clinical outcome measures including anti-double-stranded DNA, antinuclear antibody, average steroid dose, and renal function could not be determined because of the limited numbers of trials. The limitations of this review were lack of well-designed controlled trials with adequate sample size on the use of IVIg in SLE. In conclusion, the use of IVIg is associated with significant reduction in SLE disease activity and improvement in complement levels.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  15. Clinical and functional change in multifocal motor neuropathy treated with IVIg
    Chia PK, Hung SKY, Hiew FL
    J Clin Neurosci, 2019 Nov;69:114-119.
    PMID: 31416736 DOI: 10.1016/j.jocn.2019.08.016
    We determined the clinical progression, disability and outcome of 11 Multifocal Motor Neuropathy (MMN) patients from Malaysia. Mean patient age was 46.8 (SD 13.3), with mean disease duration of 108.0 months (SD 80.2). All reported unilateral limb weakness at onset. At diagnosis, after mean 49.9 months (SD 73.5) delay, 7 (63.6%) had more than 2 limbs involvement. Nine (90%) of 10 patients received induction IVIg dose of 2.0 gm/kg responded, demonstrated improvement in MRCSS of > 2 points or mRS score of > 1 point. We observed 38.5% drop in IVIg dose to mean 1.12 gm/kg/month after 12 months of treatment, and a further 34.8% drop upon 24th month treatment to mean dose of 0.73 gm/kg/month. This was in parallel with initial improvement in MRCSS and mRS, observed among 88.9% and 77.8% of the patients, and later further improvement (33.3%) or stabilization (66.7%) of mRS score toward 2nd year. During the same period, 50% of patients reported deterioration in ONLS, 33.3% in grip strength and 16.7% in MMN-RODS. Beyond 36th month, average annual IVIg dose increased at 0.12 gm/kg/year (SD 0.09) or 11.2%, up to the 84th months. Despite that, progressive deterioration was observed in term of number of limbs involvement, definite motor conduction blocks on electrophysiology study, and both clinical as well as functional scores. Although IVIg dose reduction for maintenance treatment in MMN is recommended, careful clinical assessment is required to prevent under-treatment. Use of reliable and responsive modern outcome measures is important to quantify clinically relevant change to guide therapy.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  16. A case report of refractory kawasaki disease
    Ng SH, Roshan S
    Med J Malaysia, 2018 12;73(6):410-412.
    PMID: 30647216
    Kawasaki disease is an autoimmune disease that commonly affects children below the age of 5 years. It is a vasculitic disease of unknown aetiology effecting the skin, eyes, lymph nodes and mucosal layer. Intravenous Immunoglobulin (IVIG) and aspirin therapy are the mainstay treatment however a number of cases have been shown to be refractory to this treatment. Evidence regarding approach and treatment for such cases is limited. This case report is to share our experience in the management of Refractive Kawasaki disease at a district level.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  17. Intravenous immunoglobulin may reduce relapse frequency in neuromyelitis optica
    Viswanathan S, Wong AH, Quek AM, Yuki N
    J Neuroimmunol, 2015 May 15;282:92-6.
    PMID: 25903734 DOI: 10.1016/j.jneuroim.2015.03.021
    To evaluate the use of intravenous immunoglobulin (IVIG) in preventing relapses in patients with neuromyelitis optica (NMO) and its spectrum disorders (NMOSDs).
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  18. Fulltext Myasthenic Crisis as the First Presentation of Myasthenia Gravis: A Case Report
    Payus AO, Leow Wen Hsiang J, Leong JQ, Ibrahim A, Raymond AA
    Am J Case Rep, 2021 Jan 20;22:e928419.
    PMID: 33468985 DOI: 10.12659/AJCR.928419
    BACKGROUND Myasthenic crisis is a condition characterized by the sudden onset of myasthenic weakness involving the respiratory muscles and requires ventilatory support to prevent death. This is a case report of respiratory failure in a 43-year-old man as the first presentation of myasthenia gravis. CASE REPORT A 43-year-old man with underlying hypertension and a lacunar stroke with good muscle-power recovery presented with severe community-acquired pneumonia, complicated with respiratory failure requiring invasive ventilatory support. He responded well to the intravenous antibiotic therapy and after 1 week of treatment, he was hemodynamically stable and his septic parameters improved. However, he persistently failed to maintain adequate spontaneous respiratory effort after the removal of the ventilatory support and had to be reintubated multiple times. There was no other identifiable cause for the worsening respiratory failure. He had no clinical features or muscle weakness suggestive of myasthenia gravis. However, his blood test was positive for serum anti-acetylcholine receptor antibodies and repetitive nerve stimulation tests showed the characteristic decremental response of compound muscle action potential amplitude, in keeping with the diagnosis of myasthenia gravis. He responded well to intravenous immunoglobulin and was discharged with anticholinesterase inhibitors and long-term immunosuppression therapy. CONCLUSIONS This report demonstrates that when patients are admitted to the hospital with acute respiratory failure without any underlying pulmonary disease and with weakness of the respiratory muscles, the diagnosis of myasthenia gravis presenting with a myasthenic crisis should be considered.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  19. Forme fruste anti-GQ1b-negative Miller Fischer syndrome masquerading as posterior circulation stroke
    Sim BNH, Joseph JP
    J R Coll Physicians Edinb, 2019 Dec;49(4):304-306.
    PMID: 31808458 DOI: 10.4997/JRCPE.2019.411
    Miller Fischer syndrome (MFS) is a variant of Guillain-Barré syndrome first described in 1956 and is characterised by the clinical triad of ophthalmoplegia, ataxia and areflexia. However, since its discovery, forme fruste and overlapping syndrome have been described. A forme fruste of MFS implies an attenuated form where not all of the clinical triad are present. In this report, a case of MFS is highlighted that was mistakenly treated as posterior circulation stroke, as well as the challenges faced in reaching the correct diagnosis and hence the appropriate treatment.
    Matched MeSH terms: Immunoglobulins, Intravenous/administration & dosage*
  20. Septic miscarriage with toxic shock syndrome and disseminated intravascular coagulation (DIC): The role of surgery, recombinant activated factor VII and intravenous immunoglobulin (IVIG)
    Tan LN, Mariappa G, Voon HY, Suharjono H
    Med J Malaysia, 2017 12;72(6):380-381.
    PMID: 29308782 MyJurnal
    Severe sepsis with multi-organ failure is associated with a high mortality rate. This case report highlights the challenges and modalities available in the management of a lady with refractory shock and disseminated intravascular coagulation (DIC) due to toxic shock syndrome (TSS) from genital tract sepsis. Early surgical intervention to remove the source of infection, the use of recombinant activated factor VII to treat intractable disseminated intravascular coagulation and intravenous immunoglobulin to neutralise the circulating exotoxins, have been employed and shown to drastically improve outcomes.
    Matched MeSH terms: Immunoglobulins, Intravenous/administration & dosage*
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