1/33. "Pseudo-conduction block" in vasculitic neuropathy. The predominant electrophysiologic feature of vasculitic mononeuropathy multiplex is axonal loss. Electrophysiologic findings interpreted as conduction block have, however, also been reported to occur in neuropathy secondary to necrotizing vasculitis. We report 3 patients with mononeuropathy multiplex and biopsy proven vasculitis in whom eight nerves met criteria for conduction block. In each circumstance, serial study demonstrated conversion of the electrophysiologic findings to those most consistent with severe axonal loss. "Conduction block" in vasculitic mononeuropathy multiplex is secondary to focal axonal conduction failure presumably related to infarctive axonal injury. The term conduction block should be used with caution in this disorder and only if serial studies demonstrate findings consistent with this electrophysiologic diagnosis. ( info) |
2/33. Localized neurological necrotizing vasculitides. Three cases with isolated mononeuritis multiplex. Localized vasculitic neuropathies are increasingly reported. We describe 3 cases of peripheral neuropathy with necrotizing vasculitis confined to nerves and muscles without systemic involvement. These neuropathies were severe and relapsing, in contrast to a usually benign prognosis. Our cases appear to be isolated vasculitic neuropathies, with vasculitis strictly limited to the peripheral neuromuscular system without nonspecific clinical and/or biological systemic involvement. ( info) |
3/33. Sensory perineuritis presented as a mononeuritis multiplex associated with livedo vasculitis. We report a 52-year-old patient affected by a mononeuritis multiplex and livedo vasculitis. sural nerve biopsy showed mild thickening of the perineurium, vascular alterations with inflammatory cell infiltration in the perineurium, and remarkable loss of large and small myelinated fibers. A therapeutic trial of acetylsalicylic acid, danazol, and dipyridamole controlled the skin lesion without improvement of nerve conduction studies. ( info) |
4/33. Peroneal mononeuropathy in pediatric Hodgkin's disease. A 12-year-old boy with Hodgkin's disease developed left peroneal nerve palsy during combination therapy with chemotherapy and low-dose irradiation. The palsy occurred twice; around 1-2 weeks after the second administration of vincristine in the second and third COPP (cyclophosphamide, vincristine, prednisolone, and procarbazine) regimens. Without any treatment, the peroneal neuropathy completely resolved clinically three months and electromyographically six months after the onset. He used to play television games for more than 6 hours a day with the legs crossed while sitting on the bedside. Compared to adult patients, little is known about the relationship between peroneal neuropathy and systemic malignant diseases in pediatric patients. This case shows for the first time that habitual leg crossing during potentially neurotoxic chemotherapy could induce peroneal mononeuropathy in a pediatric cancer patient. ( info) |
5/33. Intraneural nerve metastasis with multiple mononeuropathies. Although cancer is a frequent condition, neoplastic involvement of the peripheral nervous system is rare. The mechanisms are heterogeneous and include lesions within the cerebrospinal fluid (CSF) space, local invasion (e.g. brachial plexus), compression, rarely direct infiltration, perineurial spread and even rarer intranerval metastasis. A 47-year-old woman had been treated for a carcinoid 10 years earlier and had received axillar irradiation. At presentation she suffered from weakness of the biceps brachii and was experiencing pain radiating from the axilla into the forearm and thumb. MR scans of the brachial plexus were negative and her symptoms were primarily considered to stem from a postradiation brachial plexopathy, Because of increasing pain, the brachial plexus was explored and a metastasis in the left musculocutaneous nerve was resected. Several months later, numbness and pain appeared in the ulnar nerve and another intrafascicular metastasis in the ulnar nerve was discovered. Resection with preservation of remaining fascicles was performed. This rare case report demonstrates that multiple mononeuropathies, resembling multiplex neuropathy, may be caused by intranerval metastasis. ( info) |
6/33. Hereditary neuropathy with liability to pressure palsies mimicking multifocal compression neuropathy. Hereditary neuropathy with liability to pressure palsies (HNPP) is a recurrent disorder of the peripheral nervous system characterized by reversible episodes of sensorimotor deficits after neural compression injuries. Also known as tomaculous neuropathy, HNPP is further characterized ultrastructurally by multiple focal thickenings (tomacula) of peripheral myelin and has an autosomal dominant inheritance. The neuropathology of HNPP includes a partial deletion encoding the peripheral myelin protein 22 (PMP-22) gene on chromosome 17, resulting in underexpression of PMP-22. We describe multiple compression mononeuropathies in an individual with HNPP and report neuropathologic findings in 2 clinically asymptomatic family members. diagnosis was confirmed using pulsed-field gel electrophoresis. We believe that this diagnosis is clinically underappreciated by hand surgeons and should be considered in the differential diagnosis of patients with atypical presentations of compression neuropathies. ( info) |
7/33. gasoline sniffing multifocal neuropathy. The polyneuropathy caused by chronic gasoline inhalation is reported to be a gradually progressive, symmetric, sensorimotor polyneuropathy. We report unleaded gasoline sniffing by a female 14 years of age that precipitated peripheral neuropathy. In contrast with the previously reported presentation of peripheral neuropathy in gasoline inhalation, our patient developed multiple mononeuropathies superimposed on a background of sensorimotor polyneuropathy. The patient illustrates that gasoline sniffing neuropathy may present with acute multiple mononeuropathies resembling mononeuritis multiplex, possibly related to increased peripheral nerve susceptibility to pressure in the setting of neurotoxic components of gasoline. The presence of tetraethyl lead, which is no longer present in modern gasoline mixtures, is apparently not a necessary factor in the development of gasoline sniffer's neuropathy. ( info) |
8/33. Idiopathic bilateral diaphragmatic paralysis. A 41-year-old man complained of subacute onset of dyspnea and pain in the neck and chest. He was diagnosed with bilateral diaphragmatic paralysis, based on clinical inspection of the breathing pattern and transdiaphragmatic pressure recording, and was trained to use a portable bi-level positive airway pressure apparatus (BiPAP). Needle electromyography showed profuse fibrillation potentials and positive waves in the diaphragm, more abundant on the right than left side, and no response to phrenic nerve stimulation. Other muscles were not involved. Follow-up examinations, performed at 9 and 12 months after onset of paralysis, demonstrated a slow but progressive improvement of the patient's respiratory function, together with the appearance of reinnervation potentials in the diaphragm, and polyphasic, long-latency responses to phrenic nerve stimulation. The subacute onset of the paralysis associated with local pain, and its subsequent recovery, suggest bilateral proximal lesions in the phrenic nerves. In the absence of traumatic or metabolic causes, these findings suggest that the phrenic nerve can be a target in idiopathic neuritis. ( info) |
9/33. Spinal accessory mononeuropathy following posterior fossa decompression surgery. Isolated injury of the spinal accessory nerve is a well-recognized complication of surgeries involving the posterior triangle of the neck. The procedures most commonly implicated are lymph node biopsy and carotid endarterectomy. We present a patient with isolated injury to the spinal accessory nerve, localized proximal to the innervation of the sternocleidomastoid muscle, which was noted following suboccipital decompression for an arnold-chiari malformation. To our knowledge, this association has not been previously reported. ( info) |
10/33. Confluent peripheral multiple mononeuropathy associated to acute hepatitis B: a case report. A thirty three year-old, male patient was admitted at the Hospital of the Sao Paulo University School of medicine, at the city of Sao Paulo, brazil, with complaint of pains, tingling and decreased sensibility in the right hand for the last four months. This had progressed to the left hand, left foot and right foot, in addition to a difficulty of flexing and stretching in the left foot. Tests were positive for HBeAg, IgM anti-HBc and HBsAg, thus characterizing the condition of acute hepatitis B. The ALT serum level was 15 times above the upper normal limit. blood glucose, cerebral spinal fluid, antinuclear antibodies (ANA) and anti-hiv and anti-HCV serum tests were either normal or negative. Electroneuromyography disclosed severe peripheral neuropathy with an axon prevalence and signs of denervation; nerve biopsy disclosed intense vasculitis. The diagnosis of multiple confluent mononeuropathy associated to acute hepatitis B was done. This association is not often reported in international literature and its probable cause is the direct action of the hepatitis b virus on the nerves or a vasculitis of the vasa nervorum brought about by deposits of immune complexes. ( info) |