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424: Amyloid myopathy: An unusual but important cause of muscle weakness
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Abstracts / Journal of Clinical Neuroscience 15 (2008) 337–369
Betaferon, particularly in the case where it had been continued for two years, and subsequent echocardiograms in each case showed left ventricular size and function returned to normal over five to twelve months. Each patient has subsequently remained off interferon treatment with no ongoing cardiac symptoms, although still on medical therapy. Dilated cardiomyopathies are mainly idiopathic, probably the end result of myocardial damage produced by a variety of toxic, metabolic, or infectious agents. A causal link between reversible cardiomyopathy and beta-interferon treatment is likely in these cases, and possible mechanisms are considered. doi:10.1016/j.jocn.2007.07.036
424: Amyloid myopathy: An unusual but important cause of muscle weakness John D.E. Parratt a, Min Xia-Wang a, Steve Reddel b, John D. Pollard a; a Institute of Clinical Neurosciences, University of Sydney, Australia; b Department of Neurology, Concord Repatriation Hospital, Australia Purpose: Unlike neuropathy secondary to amyloid deposition, myopathy is infrequently described. We report the clinical, electrophysiological and pathological findings of two patients with distinct forms of this disease. We review the literature to determine the common presentations of amyloid myopathy and possible clues to the diagnosis. Case reports: A 58-year-old man with smouldering myeloma, presented with progressive, proximal muscle weakness over 12 months. Severe weakness of deltoid, triceps, biceps and iliopsoas was confirmed in the absence of reflex or sensory changes. Another man, aged 62, without haematological history described fatigue, weakness and prominent myalgia on exertion and relieved by rest. Exertional dyspnoea developed. Neurological examination was normal with no weakness or fatiguability. Significant myopathic changes were absent on both patients’ EMG. The first patient’s biopsy revealed variation in fiber size, scattered necrotic fibers and rims of eosinophilic, amorphous material surrounding abnormal fibers and thickened blood vessels. The second patient’s biopsy was architecturally normal with eosinophilic deposits in small, thickened vessels and interfascicular connective tissue. Congo red staining was positive in both cases, localising to the deposits. Results and discussion: The mean onset age of amyloid myopathy is 60 years. Approximately 90% of patients report proximal weakness. This may be associated with macroglossia and muscle pseudohypertrophy (35%). Dysphagia is present in 25% of patients. Muscle claudication, described by our second patient, is rare but congestive cardiac failure relatively frequent (15%). CK is often normal. EMG may demonstrate inflammatory, myopathic
changes. Congo red staining of muscle confirms the diagnosis. A monoclonal protein is often found by serum/ urine immunoelectrophoresis. Plasma cell dyscrasias or multiple myeloma are identified as the cause of amyloid myopathy in over 50% of patients. In middle-aged patients with progressive myopathy or muscle pain of unclear cause, amyloid should be considered with muscle biopsy as the definite test.
doi:10.1016/j.jocn.2007.07.037
425: Abnormalities on sagittal T2 weighted MRI of the spine in spontaneous intracranial hypotension Walid Matar, John G.L. Morris, Victor S.C. Fung; Westmead Hospital, Australia Purpose: To demonstrate the utility of sagittal MRI of the spine in the diagnosis of spontaneous intracranial hypotension. Spontaneous (‘idiopathic’) intracranial hypotension is under-diagnosed. It is caused by spontaneous spinal CSF leaks. These leaks can be detected by various imaging techniques, but the most sensitive of these is not known. We report on the MRI findings in 2 patients recently diagnosed with spontaneous intracranial hypotension. Methods: Case reports Case 1: 28-year-old female who presented to the emergency department with a sudden onset, severe, posturally dependant headache. She had been lifting a heavy fridge the day before. Case 2: 35-year-old female who presented to the emergency department with severe, posturally dependant headache, as well as nausea and vomiting. Results: A clinical diagnosis of headache secondary to intracranial hypotension was made in both patients. CT of the brain and CSF examination was normal, although CSF pressure was not measured. MRI of the brain with gadolinium showed dural thickening and enhancement but no other abnormalities. Sagittal T2 weighted MRI of the spine in case 1 showed a dorsal extradural fluid collection from C7 to L1 consistent with a CSF leak, and in case 2 a dorsal extradural fluid collection from C3 to L4. Conclusions: Extradural fluid collections identified on sagittal T2 weighted MRI of the spine can assist in the diagnosis of spontaneous intracranial hypotension. It remains unclear whether such collections are the direct result of spinal CSF leakage, or alternatively are subdural transudates secondary to hydrostatic pressure changes related to decreased CSF volume (the Monroe-Kellie Hypothesis).
doi:10.1016/j.jocn.2007.07.038
Abstracts / Journal of Clinical Neuroscience 15 (2008) 337–369
Betaferon, particularly in the case where it had been continued for two years, and subsequent echocardiograms in each case showed left ventricular size and function returned to normal over five to twelve months. Each patient has subsequently remained off interferon treatment with no ongoing cardiac symptoms, although still on medical therapy. Dilated cardiomyopathies are mainly idiopathic, probably the end result of myocardial damage produced by a variety of toxic, metabolic, or infectious agents. A causal link between reversible cardiomyopathy and beta-interferon treatment is likely in these cases, and possible mechanisms are considered. doi:10.1016/j.jocn.2007.07.036
424: Amyloid myopathy: An unusual but important cause of muscle weakness John D.E. Parratt a, Min Xia-Wang a, Steve Reddel b, John D. Pollard a; a Institute of Clinical Neurosciences, University of Sydney, Australia; b Department of Neurology, Concord Repatriation Hospital, Australia Purpose: Unlike neuropathy secondary to amyloid deposition, myopathy is infrequently described. We report the clinical, electrophysiological and pathological findings of two patients with distinct forms of this disease. We review the literature to determine the common presentations of amyloid myopathy and possible clues to the diagnosis. Case reports: A 58-year-old man with smouldering myeloma, presented with progressive, proximal muscle weakness over 12 months. Severe weakness of deltoid, triceps, biceps and iliopsoas was confirmed in the absence of reflex or sensory changes. Another man, aged 62, without haematological history described fatigue, weakness and prominent myalgia on exertion and relieved by rest. Exertional dyspnoea developed. Neurological examination was normal with no weakness or fatiguability. Significant myopathic changes were absent on both patients’ EMG. The first patient’s biopsy revealed variation in fiber size, scattered necrotic fibers and rims of eosinophilic, amorphous material surrounding abnormal fibers and thickened blood vessels. The second patient’s biopsy was architecturally normal with eosinophilic deposits in small, thickened vessels and interfascicular connective tissue. Congo red staining was positive in both cases, localising to the deposits. Results and discussion: The mean onset age of amyloid myopathy is 60 years. Approximately 90% of patients report proximal weakness. This may be associated with macroglossia and muscle pseudohypertrophy (35%). Dysphagia is present in 25% of patients. Muscle claudication, described by our second patient, is rare but congestive cardiac failure relatively frequent (15%). CK is often normal. EMG may demonstrate inflammatory, myopathic
changes. Congo red staining of muscle confirms the diagnosis. A monoclonal protein is often found by serum/ urine immunoelectrophoresis. Plasma cell dyscrasias or multiple myeloma are identified as the cause of amyloid myopathy in over 50% of patients. In middle-aged patients with progressive myopathy or muscle pain of unclear cause, amyloid should be considered with muscle biopsy as the definite test.
doi:10.1016/j.jocn.2007.07.037
425: Abnormalities on sagittal T2 weighted MRI of the spine in spontaneous intracranial hypotension Walid Matar, John G.L. Morris, Victor S.C. Fung; Westmead Hospital, Australia Purpose: To demonstrate the utility of sagittal MRI of the spine in the diagnosis of spontaneous intracranial hypotension. Spontaneous (‘idiopathic’) intracranial hypotension is under-diagnosed. It is caused by spontaneous spinal CSF leaks. These leaks can be detected by various imaging techniques, but the most sensitive of these is not known. We report on the MRI findings in 2 patients recently diagnosed with spontaneous intracranial hypotension. Methods: Case reports Case 1: 28-year-old female who presented to the emergency department with a sudden onset, severe, posturally dependant headache. She had been lifting a heavy fridge the day before. Case 2: 35-year-old female who presented to the emergency department with severe, posturally dependant headache, as well as nausea and vomiting. Results: A clinical diagnosis of headache secondary to intracranial hypotension was made in both patients. CT of the brain and CSF examination was normal, although CSF pressure was not measured. MRI of the brain with gadolinium showed dural thickening and enhancement but no other abnormalities. Sagittal T2 weighted MRI of the spine in case 1 showed a dorsal extradural fluid collection from C7 to L1 consistent with a CSF leak, and in case 2 a dorsal extradural fluid collection from C3 to L4. Conclusions: Extradural fluid collections identified on sagittal T2 weighted MRI of the spine can assist in the diagnosis of spontaneous intracranial hypotension. It remains unclear whether such collections are the direct result of spinal CSF leakage, or alternatively are subdural transudates secondary to hydrostatic pressure changes related to decreased CSF volume (the Monroe-Kellie Hypothesis).
doi:10.1016/j.jocn.2007.07.038