Guillain-Barré Syndrome is an acquired acute autoimmune polyradiculoneuropathy that commonly presents with limb weakness and occasional cranial nerve, respiratory and autonomic involvement. Although the classic description of Guillain-Barré Syndrome is that of a demyelinating neuropathy with ascending weakness, predominant bilateral finger drop as presenting feature has rarely been reported. A characteristic pattern of weakness involving the extensor components of the fingers known as "finger drop sign" has been first described to be specific in acute motor axonal neuropathy form of Guillain-Barré Syndrome in the literature. We report a case of acute motor-sensory axonal neuropathy, which showed characteristic pattern of predominant finger extensor weakness, and provide a summary of all reported cases to date. While previous reports suggested that this is a sign that carries good prognosis, our case report suggested otherwise as the patient succumbed to respiratory and autonomic complications. Further studies are needed to evaluate the clinical significance of this peculiar sign.
Tuberculosis is a multi-system disease, but paranasal sinuses involvement is uncommon, and most cases occur secondary to pulmonary tuberculosis. Tuberculous granuloma of the paranasal sinuses is rare and only a few cases are reported. Due to non-specific ways of clinical presentations, diagnosis of paranasal tuberculosis can be missed, delayed or confused with other entities like malignancy. We present an uncommon case of tuberculous granuloma of the left maxillary antrum and ethmoid sinus in a 56-year-old man which mimicked trigeminal neuralgia and malignancy. Medial maxillectomy and anterior ethmoidectomy with clearance of the lesion followed by antituberculous treatment prevented permanent loss of his vision. Depending on the chief complaints, patients may present to various specialties. Early cross-referral is important for early diagnosis, appropriate management and prevention of complications.
Pulmonary epithelioid hemangioendothelioma is an uncommon lung malignancy of endothelial origin. Besides demonstrating unpredictable presentation features and prognosis, the paucity of established treatment guidelines remains a challenge in managing these patients. We present two patients. The first patient presented with chronic productive cough over 1-year duration. He was initially diagnosed and showed partial response to treatment for cardiac failure. A persistent right upper zone consolidation on chest radiograph prompted further investigations which revealed the diagnosis of pulmonary epithelioid hemangioendothelioma. The second patient presented with right-sided hemiparesis for 1-month duration. Initial computer tomography scan of the brain showed findings of distant metastatic foci. Subsequent investigations revealed pulmonary epithelioid hemangioendothelioma as the primary lesion. Both patients succumbed without any treatment due to rapid progression of the disease. We believe that pulmonary epithelioid hemangioendothelioma is undoubtedly rarely reported in south-east Asia region. In these two case reports, the patients were diagnosed in west and east Malaysia, respectively, in the same year (2015). Both cases highlight the increasing prevalence of pulmonary epithelioid hemangioendothelioma. We postulate that this could possibly be secondary to the advancement in diagnostic capabilities and improved healthcare facilities available in this region. Late presentation of pulmonary epithelioid hemangioendothelioma generally results in grave prognosis. Further investigations are required to elucidate the nature of progression and therapeutic options for patients with pulmonary epithelioid hemangioendothelioma.
The differentiation between a pseudo-pneumoperitoneum and true pneumoperitoneum on an initial chest radiograph is challenging but essential to clinical practice. The former is managed conservatively whereas the latter may require surgical intervention. Chilaiditi's sign describes a rare incidental radiological finding of gas filled bowel interpositioned between the right hemi-diaphragm and the liver, which is visible on a plain abdominal or chest radiograph. It is often misdiagnosed as a pneumoperitoneum. Correct diagnosis of Chilaiditi's sign in an asymptomatic patient can prevent unnecessary procedures. We have reported one incidental chest radiograph with Chilaiditi's sign in a patient presenting and treated for pneumonia. The report aims to illustrate the diagnostic dilemma experienced by clinicians in distinguishing a true versus pseudo-pneumoperitoneum on a chest radiograph.
Perivascular epithelioid cell tumour is a rare mesenchymal tumour with distinct immunohistochemical profile. While it is known to occur in various anatomical sites, the central nervous system had always been a protected site for primary or secondary perivascular epithelioid cell tumours. We describe a 61-year-old lady who presented with symptoms of raised intracranial pressure, 3 months after the resection of duodenal and thoracic tumours which were histologically consistent with perivascular epithelioid cell tumour. She was investigated and then subsequently subjected to resection of two metastatic intracranial lesions. The radiological, intraoperative as well as histopathological findings of the metastatic lesions are discussed. Metastatic perivascular epithelioid cell tumour of the brain is extremely rare. However, patients who are stratified as high risk for recurrence or metastases should undergo an early magnetic resonance imaging/computed tomography of the brain in addition to a whole-body positron emission tomography scan, to allow for early detection and management of these tumours.
Acute promyelocytic leukaemia consists of 7%-8% of cases of acute myeloid leukaemia. Extramedullary manifestations are rare and show distinct biological features. We describe a 22-year-old female of Malay ethnicity who presented with fever and a left axillary swelling for a week. The peripheral blood smear showed abnormal promyelocytes with faggot cells. PML-RAR-alpha t(15;17) (q22; q12) was detected by polymerase chain reaction. The left axillary swelling histology and immunohistochemical staining confirmed granulocytic sarcoma. She was induced with triple agents consisting of all-trans-retinoic-acid, arsenic trioxide and idarubicin. On day 14 of induction, she developed severe neutropenic sepsis in which she responded to ventilation and antimicrobials. She completed her induction, consolidation and maintenance therapy. Currently she is in molecular and morphological remission. Extramedullary disease in acute promyelocytic leukaemia usually has a severe clinical presentation. Granulocytic sarcoma may present as an early feature of acute promyelocytic leukaemia.
Anti-N-methyl-d-aspartate receptor encephalitis is characterized by the clinical manifestation of neuropsychiatric symptoms, predominantly affecting young adults, and frequently associated with neoplasms. It is the second most common cause of autoimmune and paraneoplastic encephalitis. Early diagnosis is often missed, as patients are commonly diagnosed with psychiatric illnesses and are treated with antipsychotics - which rarely gives complete resolution of symptoms. Herein, we discuss a patient with mixed clinical, imaging, electroencephalogram, and laboratory findings, with an eventual diagnosis of anti-N-methyl-d-aspartate receptor encephalitis requiring immunotherapy and operative intervention.
Kaposi's sarcoma is the most common AIDS-associated malignancy. Kaposi's sarcoma in the oral cavity and oropharynx present as a macular, papular, or nodular lesion on the palate, gingiva, or tongue which may look pink, reddish, or purplish. Kaposi's sarcoma of the tonsils is relatively less common compared with other sites in the oral cavity and oropharynx. We report a case of Kaposi's sarcoma of tonsil with profuse bleeding requiring emergency tracheostomy to protect the airway followed by tonsillectomy to control the bleeding. Our initial diagnosis was hemangioma or a pyogenic granuloma. The patient tested positive for a retroviral infection and the histopathology report was compatible with Kaposi's sarcoma. Antiretroviral therapy and radiotherapy were given after stabilizing the patient. Kaposi's sarcoma of tonsils is relatively uncommon and it is unusual to cause profuse bleeding. Various treatment options are available but there are no standard treatment protocols. Treatments options depend on the site, size, stage, and immune status of the patient.
Over the years, allopurinol has been widely used as the preferred choice of urate lowering therapy in patients with gout. However, its role in patients with renal impairment is limited; and adverse reactions are well documented. Febuxostat, a newer oral non-purine xanthine oxidase inhibitor has been proven in several trials to be more effective and tolerable compared to allopurinol and may be used in patients with renal impairment. Here, we describe a case of successful febuxostat desensitization in a patient with a history of allopurinol- and febuxostat-induced adverse cutaneous reaction, as well as the protocol utilized.
Bartter syndrome is a rare autosomal recessive disorder characterized by hypokalaemia. Hypokalaemia is defined as low serum potassium concentration ˂3.5 mmol/L, which may lead to arrhythmia and death if left untreated. The aim of this case report was to normalize serum potassium concentration without the need for intravenous intervention. A 5-month-old male of 2.7 kg body weight diagnosed with Bartter syndrome was admitted to the general paediatric ward with acute severe hypokalaemia and urinary tract infection. The main challenge was the inability to administer drugs through intravenous route due to compromised body size. Therefore, we shifted the route of administration to the nasogastric tube/oral route. A total of 2 mL of concentrated intravenous potassium chloride (4 mEq potassium) were dissolved in distilled water and administered through nasogastric tube. Serum potassium concentration was rapidly normalized, which culminated in patient discharge. In conclusion, shifting drug administration from intravenous to oral route in a paediatric patient with Bartter syndrome includes numerous advantages such as patient convenience, minimized risk of cannula-induced infection, and reduced nurse workload.
Umbilical cord-derived mesenchymal stem cells for regenerative therapy are a promising treatment option for chronic illnesses. Umbilical cord-derived mesenchymal stem cells offer several advantages over other sources, which makes them an attractive option in tissue repair and regeneration. This clinical study describes a 1-year follow-up on the safety and tolerance of umbilical cord-derived mesenchymal stem cell therapy on nine patients in Malaysia. Patients were assessed for adverse effects, and liver function tests were carried out on both pre- and post-treatments. Umbilical cord-derived mesenchymal stem cells' effectiveness and safety were assessed by follow-up evaluations. All nine patients responded positively towards umbilical cord-derived mesenchymal stem cell therapy, without any adverse effects. After umbilical cord-derived mesenchymal stem cell therapy, a significant improvement was observed in liver functioning test outcomes, as haematological parameters and tumour markers were stable. The present study concludes that umbilical cord-derived mesenchymal stem cell therapy is well tolerated by Malaysian patients; however, further clinical screening must be done over a large number of patients population.