Atraumatic spinal needles for lumbar puncture have been shown to dramatically decrease the risk of postdural puncture headache. Although the use of these needles is standard practice among anesthesiologists, they have not been adopted by other medical specialties. This may lead to unnecessary morbidity among patients undergoing lumbar puncture.
Vocal cord paralysis may occur in isolation or as part of a constellation of findings in neurological disease. We investigated the pathophysiology of these disorders with laryngeal electromyography (LEMG). We studied 44 patients with idiopathic vocal cord paralysis, confirmed by laryngoscopy. The major LEMG finding, present in 29 vocal cords, was evidence of active or chronic denervation in the thyroarytenoid (TA), indicating recurrent laryngeal neuropathy. Denervation of the cricothyroid (CT) in 5 patients indicated superior laryngeal neuropathy. TA and CT involvement in 1 patient indicated proximal laryngeal or vagus nerve damage. LEMG was performed in 48 patients with other laryngeal disorders. LEMG proved to be a safe and effective procedure in the diagnosis of laryngeal neuropathy as distinguished from supranuclear and mechanical disorders of the larynx.
Dermatomyositis sine dermatitis (DMSD) is a rare autoimmune disease. It's distinguished from classical dermatomyositis (DM) by a lack of skin involvement. DM is known to have a variety of extramuscular manifestations, including interstitial lung disease, myocarditis, and dysphagia. However, peripheral nervous system involvement in DM termed "Neuromyositis" is less often encountered. Neuromyositis diagnosis is controversial due to its rarity, unknown mechanism, and heterogeneity of nerve pathology findings. A 53-year-old woman presented to our hospital following a fall. Six months prior to presentation, she had a sensory disturbance in her right foot. On admission, she had a right foot drop that progressed to right then left lower extremity weakness. A biopsy of the superficial peroneal nerve and long peroneal muscle showed large fiber nerve axonal loss, CD20 B-cell and CD4 T-cell predominant inflammatory infiltrate involving the perimysial connective tissue of the muscle, as well as myocyte hypertrophy and fibrosis with type I fiber predominance. These findings were compatible with dermatomyositis with neuropathic features. Electrophysiological studies of lower extremities revealed severe widespread axonal dysfunction, as evidenced by decreased tibial compound muscle action potential (CMAP), no peroneal motor responses, absent sural sensory nerve action potential (SNAP), and extensive active denervation throughout the left lower extremity. Three months later, she developed bilateral upper extremity weakness. A biopsy of the deltoid muscle that was done eight months after admission showed CD20 B-cell and CD4 T-cell predominant inflammatory infiltrates involving the perimysial connective tissue. These findings were pathologically similar to the first biopsy. Subsequently, a repeat electromyography (EMG) of upper extremities revealed myopathic changes with normal nerve conductions. She ultimately became quadriplegic and ventilator-dependent nine months after admission. She never exhibited any skin findings throughout her course of illness. An extensive imaging and laboratory workup did not reveal any occult malignancy, inflammation, or nutritional deficiency. Our patient did not respond to steroids or intravenous immunoglobulin (IVIg) and ultimately passed away. The clinical, pathological, and electrophysiological features suggested the presence of neuromyositis. This case illustrates the importance of recognizing peripheral nervous system involvement as a significant and yet underreported extramuscular manifestation of DM. There are currently no formal management guidelines for neuromyositis.
Marchiafava-Bignami disease (MBD) is a rare pathological condition affecting the corpus callosum (CC), characterized by progressive demyelination and necrosis. While usually found in patients with chronic alcoholism, it has rarely been characterized in non-alcoholics. We describe a trauma patient with an unknown mechanism of injury, who was found to have MBD after remaining comatose for a prolonged period of time. Magnetic resonance imaging (MRI) demonstrated restricted diffusion involving the genu, body, and splenium of the CC. The patient eventually awoke but was non-communicative and uncomprehending prior to discharge to a nursing facility. We reviewed the literature and report here the first case of MBD encountered in a trauma patient. In conclusion, MBD is an extremely rare condition in non-alcoholic patients, and the use of MRI is crucial for its identification.
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