Brachial Diplegia Research Articles

Lyme Encephalomyeloradiculitis Presenting with Brachial Diplegia (P9-10.001)

<h3>Objective:</h3> Lyme disease presenting with encephalomyelitis is rare. It often presents with non-localizing symptoms like confusion or personality changes. Focal symptoms can include hemiparesis, aphasia or ataxia. Acute brachial diplegia due to Lyme disease, although rare, has been previously reported. Our case offers additional insight into the underlying mechanisms of brachial diplegia. <h3>Background:</h3> A 73 year old man presented with mild upper thoracic back pain followed by progressive left greater than right arm weakness. Physical exam revealed a flicker of contraction in the left upper extremity and right thenar muscles. Mild proximal right arm weakness was noted. Lower extremity sensorimotor exam was normal. He was hyporeflexic in bilateral upper extremities and hyperreflexic in the lower extremities. He developed intermittent episodes of confusion, hyponatremia and a neurogenic bladder over the following week. His wife later reported he had a bulls-eye rash on his calf one month prior to presentation. <h3>Design/Methods:</h3> A work up for compressive myelopathy and cerebrovascular etiologies was negative. EMG revealed a demyelinating, predominantly motor neuropathy limited to the upper extremities, prompting treatment with IVIg. Serum Lyme PCR was positive. Cerebrospinal fluid showed lymphocytic pleocytosis, elevated protein and positive Lyme antibody. Diffuse nerve root edema was noted on brachial plexus MRI. <h3>Results:</h3> The patient was treated with intravenous ceftriaxone for 4 weeks. At his 4 week follow-up, left arm strength was at least 4/5 in all muscles. Right arm strength was normal proximally and at least 4/5 in all distal muscles. He reported paresthesia in his hands. He continues to require self-catheterization twice daily for ongoing urinary retention. <h3>Conclusions:</h3> This case illustrates that brachial diplegia in the setting of Lyme disease can be a result of radiculitis and proximal demyelination without significant motor axon loss. Radiculitis can precede encephalomyelitis. Early identification of the disease is crucial as treatment with antibiotics leads to marked improvement. <b>Disclosure:</b> Dr. Aschen has nothing to disclose. Dr. McLeod has nothing to disclose. Mary Kane-Brock has nothing to disclose. Daniel Brooks, MD has nothing to disclose. Dr. Desai has nothing to disclose.

Anterior Spinal Cord Ischemia due to an Acute Aortic Intramural Type B Hematoma

Anterior Spinal Cord Ischemia due to an Acute Aortic Intramural Type B Hematoma

Gender analysis of 'man-in-the-barrel' syndrome: is 'person-in-the-barrel' a more adequate term?

Gender bias is a known issue in healthcare and academia. We analysed the gender of patients reported in the literature with 'man-in-the-barrel' syndrome or equivalent semiological terms. A search in the PubMed database was conducted using the terms 'man-in-the-barrel', 'person-in-the-barrel', 'woman-in-the-barrel', 'brachial diplegia', 'flail arm' and 'cruciate paralysis'. All articles published between 1969 and 2020 containing a detailed description compatible with the original description of man-in-the-barrel syndrome were included. Ninety-five patients with a clinical picture compatible with man-in-the-barrel syndrome were included. Up to 33% of patients were female. Overall, the proportion of female patients with man-in-the-barrel syndrome was 25%. The term man-in-the-barrel may disenfranchise up to a third of women presenting with brachial diplegia with undesirable consequences. We propose a gender-neutral alternative such as 'person-in-the-barrel'.

Síndrome del hombre en el barril: manifestación atípica de la arteritis de células gigantes

«Man-in-the-barrel» syndrome refers to diplegia of the upper extremities in which mobility of the head and lower limbs is preserved. Brachial plexitis that presents as «man-in-the-barrel» syndrome is an unusual manifestation of giant cell arteritis. We report a case of C5-C6 plexitis as part of the clinical features of a patient with giant cell arteritis. A 70-year-old male with a two-month history of weight loss, headache, facial pain and jaw claudication, associated with a persistent elevation of acute phase reactants and bilateral brachial plexopathy, with no evidence of neck or brain injuries or occult neoplasm and with negative autoimmunity tests. Results of the biopsy study of the temporal artery were compatible with giant cell arteritis, and the positron emission tomography scan revealed extensive vascular involvement of the aorta and its branches. Although the typical clinical manifestations of giant cell arteritis are headache, jaw claudication, loss of sight, constitutional symptoms and polymyalgia rheumatica, its presence must be suspected in patients over the age of 50 who manifest alterations affecting the peripheral nerve, including brachial diplegia with no other demonstrable cause.

Two Cases of ‘Man-in-the-Barrel’ Syndrome Caused by Cerebral Hypoperfusion and Hypoxia

Background: Man-in-the-barrel syndrome (MIBS) is defined as brachial diplegia with normal strength in the legs. Most common cause of MIBS is borderzone ischemia resulting from cerebral hypoperfusion. Case Report: Our first patient was a 61-year-old woman who had cardiac arrest during medical procedure in a hospital. After resuscitation, she developed a comatous mental status. Our second patient was a 78-year-old man who was admitted with pneumonia. During hospitalization, he was discovered in an unconsciousness state. After emergent treatment, he developed a status myoclonus. After multiple antiepileptic drugs trials, his seizure attacks stopped. In both cases, mental status was recovered. However, motor weakness confined to bilateral upper extremities with preserved lower extremities strength persisted. Magnetic resonance image showed bilateral hyperintense lesions in arm-hands area of motor cortex. After supportive care, there was partial improvement in upper extremities strength. Conclusion: MIBS after cerebral hypoperfusion usually has poor outcome with high mortality rate. However, our cases had relatively good recovery. This might be due to prompt recognition, resuscitation, and treatment after hypoxic attack. J Neurocrit Care 2015;8(1):30-34

P11.09 * A NEUROLOGIC PRESENTATION OF INTRAVASCULAR LARGE B-CELL LYMPHOMA

INTRODUCTION: Intravascular large B-cell lymphoma is a rare and fatal disease that can have central nervous system manifestations. Given its rarity and the multiplicity of presentations, diagnosis is extremely difficult. We report a case that presented with neurological manifestations and which diagnosis was made ante mortem. CASE REPORT: A 74-year-old man, with a background of hypertension and dyslipidemia was referred to our neurology department with a history of speech difficulties and walking disturbances that rapidly evolved to an acute confusional sate. Physical examination disclosed an obvious executive dysfunction, global dysphasia and gait dyspraxia. Computed tomography scan of the brain showed a right frontal paramedian subcortical hiperdensity. Further characterization with brain MRI showed that this lesion was not expansive and did not enhanced with gadolinium. On laboratory analysis there was a mild lymphopenia and increased lactate dehydrogenase and ferritin levels; erythrocyte sedimentation rate was in the normal range and the immunological and virological tests were negative. Glucocorticoids (methylprednisolone 1g, intravenous, per day, during five days) were administered, leading to some improvement in the patient's symptoms. However, the control brain MRI disclosed lesion worsening, with bihemispheric extension. The cerebrospinal fluid study was unremarkable and the anti-neuronal antibodies were negative. Control laboratory analysis revealed an increase in serum C-reactive protein, erythrocyte sedimentation rate, lactate dehydrogenase (>900 IU/l), hepatic enzymes and B2-microglobulin levels. Electroencephalography was unremarkable and there was no evidence of emboligenous cardiopathy on the transesophageal echocardiogram. A computed tomography scan of the chest abdomen and pelvis was performed, with evidence of nodular thickening of both adrenal glands. An adrenal gland biopsy was undertaken, and the histological exam confirm the diagnosis of intravascular large B-cell lymphoma. Patient's condition rapidly worsened, evolving to depression of consciousness, cortical blindness, tetraparesis with brachial diplegia, and the patient died. CONCLUSION: The clinically heterogeneous spectrum of presentation and the lack of pathognomonic features or markers have made ante mortem clinical diagnosis of intravascular large B-cell lymphoma difficult and challenging. This report highlights the diagnostic challenge, the aggressive behavior and the devastating clinical course associated with this clinical entity.

Bilateral brachial plexus injury following acute carbon monoxide poisoning

BackgroundCarbon monoxide (CO) intoxication is a leading cause of severe neuropsychological impairments. Peripheral nerve injury has rarely been reported. It consists usually in a demyelinating polyneuropathy or mononeuropathy affecting mainly the lower limbs. Isolated involvement of both upper extremities has been described in only 4 patients related to root damage. We report the first case of bilateral brachial plexus injury following CO poisoning and review all previous CO-induced neuropathy described in literature.Case presentationAfter being unconscious for three hours, a 42 years old man experienced bilateral brachial weakness associated with edema of the face and the upper limbs. Neurological examination showed a brachial diplegia, distal vibratory, thermic and algic hypoesthesia, deep tendon areflexia in upper limbs. There was no sensory or motor deficit in lower extremities. No cognitive disturbances were detected. Creatine kinase was elevated. Electroneuromyogram patterns were compatible with the diagnosis of bilateral C5 D1 brachial axonal plexus injury predominant on the left side. Clinical course after hyperbaric oxygen therapy was marked by a complete recovery of neurological disorders.ConclusionPeripheral neuropathy is an unusual complication of CO intoxication. Bilateral brachial plexus impairment is exceptional. Various mechanisms have been implicated including nerve compression secondary to rhabdomyolysis, nerve ischemia due to hypoxia and direct nerve toxicity of carbon monoxide. Prognosis is commonly excellent without any sequelae.

A man with small vessel vasculitis presenting with brachial diplegia, multiple cranial mononeuropathies and severe orthostatic hypotension in diabetes mellitus: a case report

IntroductionWe report a rare case of fulminant vasculitic mononeuropathy resulting in brachial diplegia, with suspected brainstem and autonomic nervous system involvement in a patient with diabetes mellitus.Case presentationA 58-year-old Hispanic Caucasian man with diabetes mellitus presented with a 1-year history of progressive bilateral upper extremity weakness, orthostatic intolerance and progressive memory decline. Diagnostic evaluation including laboratory tests for progressive encephalopathies, systemic inflammatory and non-inflammatory neuropathies, cerebrospinal fluid analyses, electrodiagnostic studies, and nerve biopsy were performed. Clinical examination revealed moderate cognitive deficits on the Montreal Cognitive Assessment scale, bilateral facial weakness and weakness of bilateral shoulder girdle and intrinsic hand muscles. Cerebrospinal fluid analyses revealed elevated protein and an elevated immunoglobulin G synthesis rate, suggesting an immune-mediated process. Further laboratory work up was non-diagnostic. Electrodiagnostic studies demonstrated chronic asymmetric axonal mononeuropathies with ongoing denervation. A superficial radial nerve biopsy showed a chronic vasculitic neuropathy. Glucocorticosteroid treatment, symptomatic pharmacologic and supportive non-pharmacologic therapies resulted in improved clinical outcomes despite challenges with glycemic control.ConclusionsThis case report emphasizes the importance of a thorough evaluation of atypical or uncommon neuromuscular presentations in diabetic patients without etiological presumptions. This is necessary in order to promptly establish a diagnosis, initiate appropriate therapies and prevent irreversible nerve injury.

Subacute brachial diplegia associated with west nile virus myelitis

Brachial diplegia is a clinical term used to describe weakness restricted to the upper extremities. We report a case of brachial diplegia associated with West Nile virus infection. A 48-year-old man developed severe painless bilateral upper extremity weakness within a few weeks of a flu-like illness. Clinical examination revealed marked periscapular, shoulder girdle, and humeral muscle atrophy and bilateral scapular winging, with near symmetrical bilateral hypotonic upper extremity weakness. This was associated with clinical signs of an encephalomyelopathy without cognitive or sensory deficits. Electrophysiological studies demonstrated a subacute disorder of motor neurons, their axons or both, involving the cervical and thoracic myotomes, with ongoing denervation. Serological studies confirmed recent West Nile virus (WNV) infection. Gradual improvement occurred following conservative supportive therapies. Progressive brachial diplegia is a rare neuromuscular presentation of WNV neuroinvasive disease. This case report adds to the clinical spectrum of WNV-induced neurologic sequelae.

Acute Brachial Diplegia Due to Lyme Disease

to describe acute brachial diplegia as the initial manifestation of Lyme disease. bilateral, predominantly motor, cervical radiculoplexus neuropathy, the "dangling arm syndrome," has not been reported as a complication of acute Lyme infection. retrospective series of 5 patients from 2 tertiary neuromuscular centers. there were 4 men and 1 woman with an average age of 69 years. One recalled a tick bite, and preceding constitutional symptoms included headache (2) and fever, arthralgias, and fatigue in 1 patient each. Proximal arm weakness and acute pain developed within 3 weeks from onset; pain was bilateral in 3 patients and unilateral in 2 patients, and was described as severe throbbing. Arm weakness was bilateral at onset in 3 patients, and right sided in 2 patients followed by spread to the left arm within days. All the patients had weakness in the deltoid and biceps that was 3/5 or less (Medical Research Council scale), with variable weakness of the triceps and wrist extensors; 1 patient had a flail right arm and moderate (4/5) weakness of the proximal left arm muscles. Light touch was normal in the regions of weakness, and 1 patient had mildly reduced pin sensation over the forearm. Serum IgM Lyme titers were elevated in all the patients and were detected in the cerebrospinal fluid in 4 tested patients. The cerebrospinal fluid protein ranged between 135 and 176 mg/dL with lymphocytic pleocytosis (range, 42 to 270 cells). Electrodiagnostic studies showed normal median and ulnar motor potentials with asymmetrically reduced sensory amplitudes in the median (4), ulnar (3), and radial, and lateral antebrachial cutaneous potentials in 1 patient each. Two patients had acute denervation in the cervical or proximal arm muscles. There was full recovery after antibiotic therapy in 4 patients and considerable improvement in 1 patient after 2 months. acute brachial diplegia is a rare manifestation of acute Lyme infection and responds promptly to antibiotic therapy.

Man-in-the-barrel syndrome after thoracoilium fusion

The authors report a case of man-in-the-barrel (MIB) syndrome occurring after an extensive revision involving thoracoilium instrumentation and fusion for iatrogenic and degenerative scoliosis, progressive kyphosis, and sagittal imbalance. Isolated brachial diplegia is a rare neurological finding often attributed to cerebral ischemia. It has not been previously reported in patients undergoing complex spine surgery. This 70-year-old woman, who had previously undergone T11-S1 fusion for lumbar stenosis and scoliosis, presented with increased difficulty walking and with back pain. She had junctional kyphosis and L5-S1 pseudarthrosis and required revision fusion extending from T-3 to the ilium. In the early postoperative period, she experienced a 30-minute episode of substantial hypotension. She developed delirium and isolated brachial diplegia, consistent with MIB syndrome. Multiple studies were performed to assess the origin of this brachial diplegia. There was no definitive radiological evidence of any causative lesion. After a few days, her cognitive function returned to normal and she regained the ability to move her arms. After several weeks of rehabilitation, she recovered completely. Man-in-the-barrel syndrome is a rare neurological entity. It can result from various mechanisms but most commonly seems to be related to ischemia and is potentially reversible.

Subclavian artery stenosis causing transient bilateral brachial diplegia: an unusual cause of anterior spinal artery syndrome

The author report a case of a 74-year-old man who had presented with transient bilateral brachial diplegia. Investigations led to the diagnosis and treatment of subclavian artery stenosis. There are no known published cases of subclavian artery stenosis associated with transient bilateral arm weakness, and the authors believe that a steal phenomenon leading to vertebrobasilar artery insufficiency and subsequent anterior spinal artery insufficiency may have caused these symptoms, which resolved after correction of the patient's stenosis.

Diplejía braquial como variante de esclerosis lateral amiotrófica (síndrome de Vulpian-Bernhart): descripción del primer caso mexicano y revisión de la bibliografía

Brachial diplegia refers to an atypical clinical variant of amyotrophic lateral sclerosis (ALS), which is defined as a lower motor neuron disease in the upper limbs, with no significant functional complications in other regions. A 65-year-old female who presented a clinical picture of brachial diplegia that slowly progressed over a period of 72 months. Neurophysiology studies revealed a chronic denervation process. Throughout a follow-up lasting one year the clinical course remained stable. Brachial diplegia in females is a rare form of presentation of ALS and they probably have a higher survival rate than males.

Reversible brachial diplegia in a case treated with cyclosporine

A 32-year-old woman with leukemia underwent allogenic bone marrow transplantation and was subsequently treated with cyclosporine A (CsA) and prednisone. Three weeks after initiation of treatment the patient presented with acute onset of brachial diplegia. NCS\E …

Brachial diplegia as a result of cervical cord injury

To report a case of brachial diplegia with normal lower limb power, after a cervical cord injury. Case report of a 63-year-old man who sustained a neck trauma following a fall. Department of Neurology, University Hospitals of Leicester, Royal Infirmary, Leicester, UK. The mechanism of the injury was probably one of hyperflexion resulting in central cervical cord contusion. Initial neurological examination revealed brachial diplegia, with posterior column sensory involvement of the lower limbs which had normal power. The patient was also in urinary retention but had no respiratory involvement. Magnetic resonance imaging (MRI) of the spinal cord showed a hyperintense signal in the central mid-cervical cord. Pure brachial diplegia represents a rare clinical presentation of cervical cord pathology, including trauma, of which clinicians should be aware.

Man‐in‐the‐Barrel Syndrome Associated with Closed Head Injury

A patient who sustained closed head injury demonstrated brachial diplegia and intact leg strength, a case of man-in-the-barrel syndrome. Extensive hemorrhagic contusion was seen in the inferior frontal lobes bilaterally. Although aptly descriptive, the syndrome is neither predictive of an exact anatomical substrate nor representative of a specific etiology.

Brachial diplegia as a sequel to cardio-respiratory arrest: ‘man-in-the-barrel syndrome’

Brachial diplegia as a sequel to cardio-respiratory arrest: ‘man-in-the-barrel syndrome’