Neuralgic amyotrophy na and idiopathic brachial plexus neuropathy refer to a syndrome consisting of acute, severe, and transient pain in the shoulder, arm, or both, followed by flaccid weakness in the same area and less prominent or absent sensory symptoms 2, 4, 6, 15, 18, 21, 23. Monomelic amyotrophy mma is a rare disease that causes muscle weakness in the upper extremities. Fourteen patients with neuralgic amyotrophy were examined using high resolution ultrasound. Mri findings and steroid therapy for neuralgic amyotrophy. Neuroimaging of classic neuralgic amyotrophy doris liebasamal, md,1 suren jengojan, md, 2gregor kasprian, md, christian wober, md, 1 and gerd bodner, md2 1department of neurology, medical university of vienna, wahringer g urtel 1820 1090 vienna, austria 2department of radiology and imageguided therapy, medical university of vienna, vienna, austria. Hnpp is associated with deletion or abnormal structure of the pmp22 gene on 17p12p11. Neuroscience in medicine, chapter ulnar deficiencies, 2008, page 209, murray m, figure basic peripheral. Brachial neuritis bn, also known as neuralgic amyotrophy, is a rare syndrome of unknown etiology affecting mainly the lower motor neurons of the brachial plexus andor individual nerves or nerve branches.
Neuralgic amyotrophy triggered by hepatitis e virus. Social and physical disabilities decreased mobility. The ventral grey matter of the spinal cord contains the alpha motor neuron. Hereditary neuralgic amyotrophy by julie weber on prezi. Parsonageturner syndrome, also known as acute brachial neuropathy and neuralgic amyotrophy, is a syndrome of unknown cause. The neuralgic amyotrophy may be of difficult diagnosis, due to phenotypic variability, with different initial presentations upper plexus multiple mononeuropathy, lumbosacral involvement, distal reached, phrenic involvement. Neuralgic amyotrophy parsonageturner syndrome or brachial plexus neuritis is an uncommon syndrome whose cause is unknown. Turner syndrome or brachial plexus neuritis is a distinct peripheral nervous. Jeroen jj van eijk1,3 md, jan t groothuis2 md phd, nens van alfen3 md phd 1 department of neurology, jeroen bosch hospital, shertogenbosch, the netherlands 2 department of rehabilitation, donders centre for neuroscience, radboud university medical. The condition failed to respond to the usual remedies used in the treatment of reiters disease, buteventually disappeared withprednisone. Releases a chemical neurotransmitter when an action potential. Neuralgic amyotrophy is not uncommon in adults but is relatively rare in children. The exact cause is not known, therefore it is known by numerous different names such as.
The network of nerves involved in hereditary neuralgic amyotrophy. To assess the incidence of classic neuralgic amyotrophy na in a primary care setting. Neuralgic amyotrophy is more common in males and on the right side. A neurons bushy, branching extensions that receive messages and conduct impulses toward the cell body. Mildly dysmorphic facial features including hypotelorism, long nasal bridge and upslanting palpebral fissures are present in affected people in some pedigrees with this. Treatment for idiopathic and hereditary neuralgic amyotrophy brachial neuritis source. In about half of the cases it is associated with a mutation of the sept9 gene 17q25. Bn usually is characterized by the acute onset of excruciating unilateral shoulder pain, followed by flaccid paralysis of shoulder and pa. Diabetic and nondiabetic lumbosacral radiculoplexus. Nerve biopsy may reveal the above, plus features of the specific disease. Hereditary neuralgic amyotrophy hna is a neuralgic disorder that is characterized by nerve damage and muscle atrophy, preceded by severe pain.
Patient 1 was a 7yearold girl who developed a right leg paralysis after an epileptic seizure. Lumbosacral radiculoplexus neuropathy lrpn originally described in diabetic patients is a distinct clinical condition characterized by debilitating pain, weakness and atrophy most commonly affecting the proximal thigh muscles asymmetrically. Neuralgic amyotrophy is a distinct clinical syndrome with acute severe pain and patchy paresis in the shoulder and arm region. Specifically, monomelic amyotrophy causes weakness and loss of muscle mass in the arms and fingers. Notalgia paresthetica following neuralgic amyotrophy. To determine the clinical phenotype and outcome in hepatitis e virusassociated neuralgic amyotrophy hevna. Neuralgic amyotrophy is an uncommon condition affecting the shoulder and upper arm. Clinical vignette proceedings of ucla healthcare volume 21 2017 post navigation setting. The suprascapular and axillary nerves and corresponding muscles are affected most frequently. A century after the first description of neuralgic amyotrophy na, its pathophysiology remains unknown. Sadly, doctors and scientists have not found a cure for hna. Hereditary neuralgic amyotrophy hna is a rare genetic disorder characterized by recurrent episodes of severe pain in the shoulder and arm. Hna is very rare but very dangerous at the same time.
Kevin f sunshein, dpm neurogenx nervecenter of centerville 6474 centerville business pkwy centerville, oh 454592633 patient information. Afterstudyingtheliterature, webelievethatthis is only the second recorded case of neuralgic amyotrophy occurring in a patient with reiters disease associated with genital infection. While not much is known about this disorder, it has been characterized to be similar to parsonageturner syndrome in prognosis. What are the distinctive features of hevassociated. Incidence of neuralgic amyotrophy in a primary care. Nervous system chapter 8 questions and study guide. Parsonageturner syndrome brachial neuritis patchwork amyotrophy localised neuritis of the shoulder girdle seratus magnus. Ultrasonographic identification of nerve pathology in. Only 200 families are known to have it worldwide originating from people of european descent. Neuralgic amyotrophy na, also called parsonageturner syndrome, or brachial plexus neuritis, is an acute and painful neuropathy that involves mainly the upper brachial plexus. The condition manifests as a rare set of symptoms most likely resulting.
The syndrome is usually monophasic and preceded by significant weight loss at least more than 10 lbs. It was first reported after antitetanic serotherapy, by dyke, in 1918. Lower motor neurons are cells that help communicate information from the brain to the muscles that are involved in movement skeletal muscles. The disorder exhibits a broad range of clinical manifestations. Pain especially occurs with small fiber damage sensory fibers. Neuralgic amyotrophy parsonage turner syndrome or brachial plexus neuritis is an uncommon syndrome whose cause is unknown. Wyburnmason described 42 cases of brachial plexus neuritis in 1941, and spillane reported 46 cases of. We report the case of a patient who developed notalgia paresthetica during the recovery from a neuralgic amyotrophy.
To characterize the ultrasonographic findings on nerves in neuralgic amyotrophy. A 23yearold woman had a typical neuralgic amyotrophy severe shoulder pain, followed by a long thoracic nerve palsy. Hereditary neuralgic amyotrophy with predilection for the brachial plexus is an autosomal dominant disorder associated with recurrent, episodic, painful brachial neuropathies. Id of 3 glutamic acid residues 2x greater frequency of d allele in type 2 patients with peripheral neuropathy. The clinical phenotype was recently found to be more comprehensive and the longterm prognosis less optimistic than usually assumed for. Learn vocabulary, terms, and more with flashcards, games, and other study tools. The clinical manifestations and outcomes of neuralgic. Hereditary neuralgic amyotrophy is a disorder characterized by episodes of severe pain and muscle wasting amyotrophy in one or both shoulders and arms. Despite advances, cerebral angiography continues to be used for the examination of patients with cerebrovascular. Marys hospital, college of medicine, the catholic university of korea, seoul.
Ultrasonographic identification of nerve pathology in neuralgic amyotrophy. Hereditary neuralgic amyotrophy hna, also termed hereditary brachial plexus neuropathy, is a genetically heterogeneous condition most commonly caused by point mutations or duplications in the sept9 gene on chromosme 17q25. Despite the confusion in naming the condition it is a wellrecognised and wellidentified clinical entity. Although hereditary neuralgic amyotrophy with predilection for the brachial plexus has some similarities to hereditary neuropathy with liability to pressure palsies hnpp.
Neuralgic pain is felt along the path of one or more nerves and often has no obvious physical cause. A correct diagnosis is important because in spite of the. We recently encountered 2 cases of neuralgic amyotrophy in children. Neuralgic amyotrophy is an uncommon condition characterised by the acute onset of severe pain in the shoulder and arm, followed by weakness and atrophy of. During the year 2012 we registered all new cases of neck, shoulder or arm symptoms from two large primary. A threadlike extension of a neuron that carries nerve impulses away from the cell body. To date, there is little guidance on these patients therapeutic management, especially those for which neuralgic amyotrophy is. Clinical phenotype and outcome of hepatitis e virusassociated neuralgic amyotrophy van eijk jjj, dalton hr, ripellino p, et al. Incidence of neuralgic amyotrophy in a primary care setting. Pathology report by bako pathology services epidermal nerve fiber density analysis of patient ee physician. Hereditary neuralgic amyotrophy genetics home reference. Hereditary neuralgic amyotrophy is a type of nervous system disease that affects the group of nerves that control movement in the arms and shoulders called the brachial plexus. Pathology nerve fiber damage, usually axonal degeneration. In most cases, pain may persist for a few hours to a few weeks and is followed by wasting and weakness of the.