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Fresh fish products are exquisitely prone to be the source for food botulism because of primary contamination with Clostridium botulinum spores, lack of heat inactivation of C. botulinum spores, frequently used air-tight packaging, lack of preservatives, lack of heat inactivation of already produced botulinum toxin and because of substantial low temperature resistance of C. botulinum type E typically found in fish botulism. We can reporting a 25-year-old patient with food botulism presenting with an anticholinergic syndrome with mydriasis, accommodation difficulties, dryness of mouth, reduced sweating, constipation and reduced heart rate variance and only discrete involvement of striate muscles with ptosis, diplopia and generalised fatigue, all of which developed 12 h after the consumption of airtight sealed smoked salmon. The salmon was consumed 3 days after the 'use by' date had expired.
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PMID:[Botulism caused by consumption of smoked salmon]. 1566 67

We report herein the case of a 28-year-old man presenting with hyperglycemic chorea-ballism (HCB) in addition to mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). He was admitted to a local hospital due to weight loss, general fatigue and thirst. The patient had diabetes mellitus, with a blood glucose level of 738 mg/dl and HbA1c of 19.8%. Although insulin therapy improved hyperglycemia, he noticed involuntary movements in the right upper and lower limbs, which subsequently extended to the left side. The patient was thus transferred to our hospital. He displayed short stature (154 cm) and emaciation, and a maternal family history of diabetes mellitus was elicited. He had no history of stroke-like episode, headache, vomiting and seizure. Neurological examination revealed low intelligence (IQ 57), mild sensorineural deafness, and chorea-ballism in the extremities and head without ptosis or eye movement disturbance. Brain computed tomography (CT) demonstrated areas of high density, while T1-weighted magnetic resonance imaging (MRI) revealed extreme hyperintensity and T2-weighted MRI showed hyperintensity in bilateral caudate nuclei, putamina and globi pallidus. HCB was diagnosed. In, CSF, lactate level was increased to 43.9 mg/dl (n, 4-16), pyruvate level was 1.65 mg/dl (n, 0.3-0.9) and total protein concentration was 59 mg/dl. Histological examination of a biopsy sample from the biceps brachii muscle demonstrated ragged-red fibers. An A3243G point mutation in the tRNA(Leu(UUR)) gene was detected, indicating the presence of MELAS. Involuntary movements improved on treatment with haloperidol up to 4.5 mg/day. HCB usually appears in elderly individuals, and cases less than 40-years-old are very rare. The mitochondrial dysfunction in MELAS may accelerate development of HCB.
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PMID:[A case of MELAS presenting juvenile-onset hyperglycemic chorea-ballism]. 1611 32

A 42-year-old woman received a 6-month course of simvastatin (20 mg/d) for hypercholesterolemia. Despite an infection with fever, fatigue, myalgias, and lumbar pain, she continued to perform her regular sports activities. Neurologic examination revealed bilateral ptosis and slight upper limb weakness. Serum creatine kinase was 41,000 U/L. Needle electromyography was nonspecifically abnormal. Discontinuation of simvastatin and reduction of the sports activities was followed by a prompt continual lowering of the elevated muscle enzymes to normal values over a 2-week period. The patient's infection, regular sports activity despite the infection, and a suspected mitochondrial defect were regarded as triggers of rhabdomyolysis.
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PMID:Rhabdomyolysis from Simvastatin triggered by infection and muscle exertion. 1663 54

Every year, many women all over the world will undergo a hysterectomy, the removal of their uterus. The majority of hysterectomies are performed to treat conditions such as fibroids, heavy bleeding, endometriosis, adenomyosis and prolapse. A hysterectomy is not often a procedure that needs to be performed urgently, except in the case of cancer. Therefore, a woman considering the procedure should take time to investigate all her options, including other possible treatments. Deciding whether to have a hysterectomy can be a difficult and emotional process. Signs of depression may include severe and prolonged feelings of sadness and hopelessness; diminished interest in activities; significant weight loss or gain; insomnia; fatigue; and thoughts of death or suicide. Every person reacts differently, and reactions are a combination of emotional and physical responses. We still have much to learn about the effects of hysterectomy on sexual function. We investigated many studies published in different journals relative to this subject and we compare their results. Women are more likely to report improved sexual functioning after the surgery when their symptoms have been alleviated. A new hysterectomy procedure that 'spares' abdominal ligaments and nerves is quicker and results in less blood loss and shorter hospital stays and seems to respect the tissues more, without affecting the sexuality of the women.
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PMID:The effect of hysterectomy on sexuality and psychological changes. 1654 13

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology which affects most frequently the hilar lymph nodes and lungs. Symptomatic involvement of the central nervous system may develop in patients diagnosed with sarcoidosis or it may be the initial manifestation of the disease. This is a case report of 48-year old female patient admitted to our clinic for evaluation of working ability. The patient had a total of 24 years of service and occupational exposure and she has been employed as supplies procurement officer. On admission, she complained of the following discomforts: eye-lid pain, intellectual fatigue, psychic uneasiness, forgetfulness, dyspnea and productive cough. Neurological findings indicated the presence of the right eye ophthalmoplegia, psychoorganic syndrome and neurosarcoidosis. Ophthalmological examination evidenced bilateral ptosis and presence of anisocoria. Magnetic resonance imaging revealed discrete focal lesions of the pons (paracentral left) and parietal corona radiata of the left hemisphere. Based on performed examinations and diagnostics procedures, final evaluation of patient's working ability concluded that the patient was not capable of psychic strains and jobs associated with material accountability.
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PMID:[Work ability evaluation in neurosarcoidosis--a case report]. 1697 13

Myasthenia gravis (MG) is a chronic, autoimmune disease involving neuromuscular junctions. It is frequently associated with symptoms such as loss of muscle strength, difficulty in respiration and swallowing, diplopia and ptosis. All chronic diseases, including MG, may have psychiatric consequences in terms of coping and adaptation. Psychiatric morbidity usually appears as anxiety disorders, such as panic disorder and generalised anxiety disorder, and as depressive disorders. However, there are very few data on the prevalence and aetiology of such psychiatric symptoms in patients with MG, and those available in the literature are generally from old studies with poor methodology. The interaction between MG and psychiatric disorders needs to be appreciated, especially in the primary care setting, since the symptoms may overlap. MG may be under-recognised initially because the psychiatric symptoms may coincide with those of the actual disease, such as fatigue, lack of energy and shortness of breath. On the other hand, co-morbid psychiatric symptoms that appear during the course of the illness may be misdiagnosed as true myasthenic symptoms; thus, leading to unnecessary drug treatment. Differentiation of the aetiology of these symptoms might alter the treatment choice and, therefore, affect the treatment success rate and patients' well-being. Psychiatric treatments must be carefully planned because of the risk of aggravating the underlying neurological disease. Even though there appears to be an intricate relationship between MG and psychiatric symptoms, there is very limited information on this subject. As such, prospective, randomised, controlled pharmaco/psychotherapy studies are needed to better direct the management of patients and, thus, improve quality of life during the course of the illness.
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PMID:Mood and anxiety disorders in patients with myasthenia gravis: aetiology, diagnosis and treatment. 1752 Dec 27

Glycogen storage disease type Ib (GSD Ib, OMIM 232220) is an inborn disorder of glucose metabolism, caused by mutations in the G6PT gene, encoding a glucose 6-phosphate transporter (G6PT). GSD Ib is mainly associated with fasting hypoglycaemia and hepatomegaly. Most GSD Ib patients also show neutropenia and neutrophil dysfunction and therefore are at risk of developing severe infections and inflammatory bowel disease (IBD). An increased risk for autoimmune disorders, such as thyroid autoimmunity and Crohn-like disease, has also been demonstrated, but no systematic study on the prevalence of autoimmune disorders in GSD Ib patients has ever been performed. We describe a 25-year-old patient affected by GSD Ib who developed 'seronegative' myasthenia gravis (MG), presenting with bilateral eyelid ptosis, diplopia, dysarthria, severe dysphagia, dyspnoea and fatigue. The repetitive stimulation of peripheral nerves test showed signs of exhaustion of neuromuscular transmission, particularly evident in the cranial area. Even in the absence of identifiable anti-acetylcholine receptor antibodies, seronegative MG is considered an autoimmune disorder and may be related to the disturbed immune function observed in GSD Ib patients.
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PMID:Myasthenia gravis in a patient affected by glycogen storage disease type Ib: a further manifestation of an increased risk for autoimmune disorders? 1843 26

Myasthenia gravis (MG) is a chronic neuromuscular disease which leads to varying degrees of weakness in the skeletal muscles. Some of the symptoms of the disorder include weakness of the eye muscles, difficulty in swallowing and slurred speech. When only the muscles of the eyes are affected, the illness is termed ocular myasthenia, which is often characterized by abrupt onset of diplopia and ptosis of the eyelid. In most patients with ocular-onset MG, there is a progression to involvement of other muscle groups within the first two years (generalized myasthenia). In the case reported here, a 39-year-old male of Ecuadorian descent complained of difficulty seeing, double vision, dizziness, unsteady gait, difficulty maintaining balance and fatigue for the previous two days. Neurological examination was remarkable for total external ophthalmoplegia. There was no external bulbar muscle paralysis, motor weakness, muscle wasting, sensory deficits or sphincter dysfunction. His laboratory workup was significant for elevated acetylcholine receptor antibody. He was diagnosed with ocular MG after differential diagnoses were ruled out based on the onset and presentation of symptoms, the patient's age and a normal magnetic resonance imaging exam. No signs of generalized myasthenia were detected. His symptoms improved dramatically after treatment with Acetyl cholinesterase (AchE) inhibitors and steroids, regaining much of his ocular mobility and ability to walk without gait imbalance. At follow-up visits, the patient remained healthy with no evidence development of other myasthenic signs. This case is atypical since ocular MG does not normally occur in the absence of other myasthenic forms.
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PMID:An atypical course of myasthenia gravis. 1860 52

We present the case of a 40-year-old female patient with sudden onset of anisocoria and unilateral ptosis of the left eye. With the exception of several previous episodes of nausea and vomiting, mild headache and tiredness, combined with the early death of the patient's mother following aortic rupture, patient history and clinical condition showed no pathological findings. Following indicative findings on duplex sonography, a dissection of the left internal carotid artery from its origin to its distal section was detected on CT angiography of the brain vessels and the diagnosis of Horner syndrome due to internal carotid artery dissection was made. Since this condition is associated with serious embolic complications, prompt treatment following diagnosis is of utmost importance. Our patient was treated conservatively using PTT (partial thromboplastin time)-effective heparinisation. Regular checks including kidney ultrasound, blood pressure measurement, imaging and continuous therapy with acetylsalicylic acid are recommended.
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PMID:[Anisocoria and nausea]. 1893 3

Myasthenia gravis is an autoimmune disease characterized by weakness and fatigue of the skeletal muscles of the face and extremities. Pregnancy is associated with 19-41% of exacerbations. We present a 19-year-old woman who was seen at the neurology clinic 10 days after her last cesarean section with diplopia and trouble swallowing liquids and solids for the previous 3 months. On examination she had slurred speech, bilateral palpebral ptosis, and mild droop of her shoulders. An arterial blood sample showed a PCO2 of 50 mmHg and a PO2 of 70 mmHg. The tensilon test was positive, Intravenous methylprednisolone and immunoglobulin and oral pyridostigmine effectively relieved her myasthenic signs and symptoms. Five assays for acetylcholine or anti-striational antibodies in her serum were negative. A test for muscle-specific tyrosine kinase antibody was positive. In 10-20% of patients with myasthenia gravis no antibodies to acetylcholine receptors are detectable. Forty percent of this group has an antibody to muscle-specific tyrosine kinase.
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PMID:"Seronegative" anti-MUSK positive myasthenia gravis presenting during pregnancy. 1961 May 60


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