Distal Upper Extremity Research Articles

Case Report: Injectable Pentazocine Abuse Leading to Necrotizing Soft Tissue Infection and Florid Osteomyelitis

A 20-year-old right-hand dominant female presented to the emergency department with a significant ulcerative lesion overlying the dorsum of her left hand with exposed bone. She had arrived in the USA the previous day from Nigeria and was brought to our institution for evaluation. Her only significant medical history included migraine headaches and malaria, the latter of which had been recently treated. Approximately 1 year prior, she had been diagnosed with migraine headaches by her physician and was prescribed intravenous pentazocine (Talwin) for symptomatic relief. She became dependent on the medication and began self-administration, primarily injecting into her distal upper extremities. According to her history, she began to develop superficial cutaneous ulcers on the dorsum of her hands bilaterally approximately 6 months prior to presentation. Her right hand wounds eventually healed and fibrosed, while the left hand continued to worsen. She stated that she was treated on several occasions with a combination of oral and intravenous antibiotics but never underwent formal irrigation and debridement. She was minimally compliant with treatment modalities, however, and continued self-administration of the drug. After progressing to the point where she had lost meaningful function of her hand, her family brought her to the USA for treatment.

Job Analysis Techniques for Distal Upper Extremity Disorders

Distal upper extremity (DUE) work-related musculoskeletal disorders (WMSDs) are among the most costly injuries suffered in industry today. These WMSDs are reported in both office (computer use) and manufacturing environments. Job physical exposure analysis techniques for DUE WMSDs range from simple checklists to quantitative models. A summary of literature review of biomechanical, physiological, psychophysical and epidemiological bases for job physical exposure risk factors for DUE WMSDs is provided. Several job analysis methods suitable for manufacturing environments are reviewed and discussed. A comparative analysis of Rapid Upper Limb Assessment (RULA), Threshold Limit Value for Hand Activity Level (TLV for HAL), and the Strain Index is provided along with results from validation studies and advantages and disadvantages of each method. Three examples from industries are provided to demonstrate applications of RULA, TLV for HAL, and the Strain Index. Last, issues with current job analysis methods when a worker rotates to different jobs and/or when a job consists of several tasks are discussed as well as the need for more robust models to account for these variations in physical exposure in real-world environments.

Hirayama disease: An uncommon cause of distal upper extremity deficit

Hirayama disease is a myelopathy related to flexion movements of the neck that produce ischemic damage in the anterior horn of the cervical cord. The disease affects young people and is characterized by a distal upper extremity deficit that develops gradually. The diagnosis is confirmed by cervical magnetic resonance imaging (MRI) in flexion demonstrating a forward shift of the posterior dural sac and spinal cord compression. On radiology, there should be suggestive signs in neutral position such as cord atrophy, intramedullary bilateral high signal intensity on T2-weighted images, straightening of the cervical spine, and loss of attachment between the posterior dural sac and subjacent lamina. Exploration should be completed by an MR study in neck flexion. We report here on two typical cases of the disease and also include a review of the literature.

Ultraviolet Light-induced Köbner Phenomenon Contributes to the Development of Skin Eruptions in Multicentric Reticulohistiocytosis

Multicentric reticulohistiocytosis is a rare systemic disease of unknown aetiology characterized by erosive arthritis and cutaneous lesions consisting of multiple reddish-brown papules and nodules, mainly involving the face and distal upper extremities. It has been suggested that skin eruptions in multicentric reticulohistiocytosis are associated with Köbner phenomenon due to their characteristic distribution, such as on the dorsal aspects of the hands and fingers. We report here a case of a Japanese woman with multicentric reticulohistiocytosis, in whom erythematous macules and papules were widely distributed over the face, ears, neck and the V-area of the chest. Notably, repeated irradiation of ultraviolet (UV) B on the uninvolved back skin resulted in the induction of erythematous macules with infiltration of reticulohistiocytes, indicating the association of UVB-induced Köbner phenomenon with the development of skin lesions, especially on the sun-exposed area. This is the first known report demonstrating the contribution of UV-light-induced Köbner phenomenon for the development of skin eruptions in patients with multicentric reticulo-histiocytosis.

Prevalence of musculoskeletal disorders among plastics industry workers

The prevalence of musculoskeletal disorders among workers in the plastics industry in Salvador, Bahia State, Brazil was estimated. Cases were defined by reported symptoms of pain in the previous 12 months, lasting more than a week or having monthly minimum frequency, which had given rise to restrictions at work or to seeking medical attention, or where respondents had a severity score greater than or equal to 3 (on a numerical scale of 0 to 5). A stratified proportional random sample of 577 workers was studied. The prevalence of musculoskeletal disorders, considering of all body segments, was 50.1%. The prevalence of musculoskeletal disorders was higher among women than among men at distal upper extremities (34.6% and 11.6% respectively) and also in the region of the neck, shoulder or upper part of the back (27.4% and 17.6% respectively). There was no difference between genders for the prevalence of lower back pain (21.2% and 21.4% respectively); 65% of cases in this region had reports of pain in the previous seven days. Due to the importance and prevalence of musculoskeletal disorders, it is necessary that their measurement in epidemiological studies be done properly.

Factors Related to Musculoskeletal Disorders in Nursing Workers

This cross-sectional study identified factors associated to musculoskeletal disorders in nursing auxiliaries and technicians in Salvador - Bahia. Three hundred and eight randomly selected female workers answered a questionnaire administered by an interviewer, with questions related to physical and psychosocial demands at work, individual characteristics and activities outside work. Multivariate analysis revealed that musculoskeletal disorders in neck, shoulder or upper back and musculoskeletal disorders in low back are associated to physical demand (material handling, poor back posture and work repetitiveness), psychosocial demand and precarious physical fitness. Musculoskeletal disorders in distal upper extremities are associated to physical demands (repetitiveness and force) and years of work (> 19). The results appoint the need for intervention strategies, incorporating both the organizational aspects of work and adaptations in the physical environment and in the characteristics of tasks.

Electrophysiological characteristics of juvenile muscular atrophy of distal upper extremity ( Hirayama's disease)

Objective The purpose of this study was to retrospectively evaluate the electrophysiological characteristics of Hirayama disease. Methods Electrophysiological records of 77 patients with a diagnosis of Hirayama disease were reviewed. We analyzed the amplitude of compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) as well as the F wave latency and frequency of median and ulnar nerves. We also evaluated the changes of needle electromyography. Results Of the 77 patients, 74 were male and 3 female. Mean age of onset was ( 17.7 ± 2.6 ) years and mean disease duration (28.2 ± 25.7 ) months. 57 of the 77 patients showed clinical presentation of unilateral involvement. Reduced ulnar CMAP amplitude was found prodominantly. Abnormal F wave frequency was found in the majority of the patients in both median and ulnar nerves and there was also correlation between F wave frequency and amplitude of CMAP. Sensory conduction studies were normal. In the needle EMG, neurogenic changes were found in both upper limbs in 67 cases and the remaining 10 patients were unilateral involved. Denervation was found in the C7-T1 myotomes in all of the 77 patients. Among them, 15 also showed C5, C6 involvement with different extent. Conclusion In this study, the most obvious electrophysiological abnormality was reduced F wave frequency in both median and ulnar nerves followed by reduced ulnar CMAP amplitude. Although most patients showed unilateral involvement clinically,needle EMG found 87% had bilateral neurogenic changes. Although C7, C8, T1 myotomes were mainly damaged in Hirayama disease, C5,C6 was also involved in around 1/5 of the patients. Key words: Muscular atrophy; Upper extremity; Electromyography; F wave

Tremor and Deep Brain Nuclei Hyperintensities in Kabuki Syndrome

Kabuki syndrome is a rare congenital disorder first described in 1981. Case reports indicate multiple congenital abnormalities: skeletal anomalies, cognitive impairment, characteristic facial appearance, and peculiar dermatoglyphic patterns. We describe a patient with Kabuki syndrome who presented with physiologic tremor in her distal upper extremities. Cranial magnetic resonance imaging revealed symmetric T(2)-hyperintense lesions with mildly restricted diffusion in the lentiform nuclei, red nuclei, and dentate nuclei bilaterally. Although multiple abnormalities of the central nervous system were reported previously in Kabuki syndrome, this patient is the first, to the best of our knowledge, with the unique findings that we observed.

Guillain-Barre Syndrome Following Azacitidine for Treatment of Myelodysplastic Syndrome.

Abstract 4861 BackgroundIn this case report we describe a 65-year-old woman with myelodysplastic syndrome who was started on azacitidine and subsequently developed Guillain-Barre Syndrome (GBS). To our knowledge, there are no other reported cases of azacitidine-associated GBS. Case ReportThis patient was diagnosed in 2004 when a bone marrow biopsy done for anemia showed erythroid, myeloid and megakaryocytic dysplasia. Cytogenetics were normal. Her baseline hemoglobin was 7g/dL. Despite combined growth factor support and thalidomide, this patient remained transfusion-dependent, requiring packed red blood cells (PRBCs) every 3 weeks. Her only other medical problem was well-controlled diabetes without renal impairment or neuropathy. Liver function was normal. Her medications included deferasirox, glyburide, metformin and pioglitazone. At baseline she had an Eastern Cooperative Oncology Group performance status (ECOG PS) of 0. This patient received a 7-day course of subcutaneous azacitidine, after which she required a total of 7 units PRBCs for grade 3 anemia. She did not experience any other complications such as viral illness or diarrheal infection. There were no changes in medications. Thirty-four days after starting azacitidine she experienced acute onset of bilateral lower extremity weakness starting distally and moving proximally. The next day she developed bilateral weakness in her distal upper extremities. On initial evaluation she was found to have symmetric bilateral upper and lower extremity weakness. Strength in the distal and proximal extremities (both upper and lower) was 1/5 and 3/5, respectively. Lower extremity deep tendon reflexes were absent. Sensation remained intact, and cranial nerve exam was normal with preserved respiratory muscle strength. A non-contrast head CT and cervical spine MRI without contrast were normal. CSF studies revealed elevated protein in the absence of cells. Lumbosacral MRI with contrast showed a non-specific, faint enhancement of the anterior lumbosacral nerve roots. An EMG indicated an acute demyelinating process. She was diagnosed with GBS and received a 5-day course of intravenous immunoglobulin with notable improvement in upper extremity strength within 1 week. At baseline after recovery from acute presentation, she was wheelchair-bound with an ECOG PS of 3. DiscussionThis is the first reported case of azacitidine-associated GBS. Azacitidine is a DNA hypomethylating agent that was FDA-approved in 2004 for the treatment of myelodysplastic syndrome. A common side effect is grade 3-4 myelosuppression. The common non-hematologic side effects are nausea and vomiting. Severe adverse non-hematologic reactions to azacitidine are uncommon (Sullivan, et al, AJHP 2005;62:1567-73). In 1975 neuromuscular toxic syndrome was described in 17 leukemic patients after they received combination therapy containing azacitidine (Levi JA, Wiernik PH Cancer Chemother Rep 1975;59:1043-45). A case report in 1985 documented a child with acute rapidly reversible CNS toxicity (coma preceded by somnolence/myalgias) days after receiving an erroneously high dose of 5-azacytidine for acute leukemia (Weisman, et al, J Pediatr Hematol Oncol 1985;7:86-88). No other azacitidine-associated adverse neurologic events, and specifically no reports of a demyelinating process, have been published. While the association between GBS and certain infections and immunizations has been well documented (Hughes RA, Cornblath DR Lancet 2005;366:1653-66), there is data to suggest that GBS can be medication-induced. Zimeldine has been described as the probable link to the development of GBS in 10 patients (Fagius, et al, JNNP 1985;48:65-69). In our patient, the temporal relationship between starting treatment and the development of neurologic symptoms, along with the lack of other known GBS-inciting factors, form a strong basis for an associational relationship between azacitidine and the development of GBS. It is therefore important that clinicians are aware of this potential severe adverse effect. DisclosuresNo relevant conflicts of interest to declare.

Dengue Virus Associated Hemophagocytic Syndrome with Neurogical Manifestations in Children: A Case Report.

Abstract 4516Dengue fever, caused by dengue virus, can cause increased vascular permeability, which leads to a bleeding diathesis or disseminated intravascular coagulation known as Dengue Hemorrhagic Fever (DHF). Hemophagocytocytic Syndrome (HPS) with neurological manifestations is an uncommon presentation of DHF. There are no reports of virus associated with HPS and neurological manifestations in the pediatric literature. Hemophagocytic Syndrome (HPS) is a clinico-pathologic entity characterized by proliferation of T lymphocytes and macrophages leading to cytokine overproduction. HPS may be diagnosed in association with malignant, genetic, or autoimmune diseases. Dengue virus is considered non-neurotropic, however neuroinvansion has been reported. We present the case of a 10 month old female patient who developed DHF, manifested with upper GI bleeding, hypotension and pancytopenia. The infant subsequently developed dengue shock syndrome, with thrombocytopenia, intravascular hemolysis, coagulopathy, elevated transaminase, hyperbilirubinemia, and creatine kinase (CK) of 2,876U/L. She required artificial hemodynamic and blood components support. During her course of illness, she developed ecchymosed, purpuric bullae lesions of the skin in the distal upper extremities. Her IgM ELISA for Dengue virus was positive. Bone marrow aspiration and biopsy was diagnostic for HPS. The patient was initiated on IV steroids and antibiotics therapy. An improvement was noticed on day 8 of illness and was weaned off completely of all artificial support by the day 9. A complete recovery of her hematological, transaminase, billirubin, and coagulation parameters was noticed on day 12. A repeated bone marrow aspirate and biopsy examination was normal. Patient's recovery was significant by muscle weakness (MCG grade ≤3), hypotonia, reduced tendon reflex and increased CK suggestive of possible myositis. The cerebrospinal fluid and magnetic resonance imaging studies of the brain were normal. She was given IV immunoglobulin (500mg/kg) for 2 consecutive days with remarkable neurological improvement including a normal CK (56U/L).She was weaned of steroids completely on day 20.Dengue virus infection was confirmed by a positive serology result at the convalescent stage. On day 23 she had a complete clinical recovery. To our knowledge, this is the first case reported of dengue virus-associated HPS and neurological manifestations in a pediatric patient with DHF. Clinicians should consider that the occurrence of HPS and neurological manifestations in children could de due to dengue virus infection. Disclosures:No relevant conflicts of interest to declare.

Segmental muscular atrophy of the distal upper extremity (Hirayama disease): An atypical case with anterior dural detachment

Segmental non-progressive spinal muscular atrophy of the upper extremity, also known as Hirayama disease (HD), is a disorder seen mainly in young male patients of Asian ancestry. On MRI loss-of-attachment between the posterior dural sac and adjacent lamina has been described as the only significantly important predictor of HD. We herein present a patient with HD who showed an atypical anterior detachment of the dura mater from the adjacent vertebral body.

Vancomycin‐Induced Leukocytoclastic Vasculitis

Vancomycin is well recognized as causing the nonallergic skin reaction known as red man syndrome; however, it is rarely suspected as causative in the setting of an immune-mediated skin reaction. We describe a 76-year-old Caucasian woman with a history of penicillin and sulfa allergies who was transferred to our medical center while receiving vancomycin for treatment of persistent methicillin-sensitive Staphylococcus aureus (MSSA) bacteremia. After admission, the patient's pacemaker was explanted; cultures from the pacemaker grew MSSA. Based on the culture data and her allergy to penicillin, vancomycin was continued. On day 4 of therapy, the patient developed a papular rash with small blisters on her distal upper extremities. Furosemide, which she was receiving intermittently to maintain fluid balance, was initially suspected as the likely cause. Furosemide was withheld; however, the rash worsened and spread to her neck and torso. Results of skin biopsy confirmed a severe leukocytoclastic, necrotizing small-cell vasculitis that met the criteria for a hypersensitivity vasculitis associated with drug therapy. Five days after discontinuation of vancomycin, the vasculitis was resolving and continued to resolve throughout the remainder of her hospitalization. Furosemide was readministered without worsening of the vasculitis. Use of the Naranjo adverse drug reaction probability scale indicated that the likelihood of vancomycin being the cause of the vasculitis was probable (score of 5). Clinicians should be aware of vancomycin as a potential cause of small-vessel vasculitis.

Bilaterally symmetric form of Hirayama disease

Hirayama disease (brachial monomelic amyotrophy) is a unilateral or grossly asymmetric bilateral disease. Bilaterally symmetric involvement has never been described. Based on cardinal clinical and MRI criteria, a total of 106 patients with Hirayama disease from two tertiary care hospitals of North India seen between 1992 and 2008 formed the basis of this study. All those found to have bilaterally symmetric involvement on clinical and electrophysiologic basis were evaluated for clinical, electrophysiologic, and MRI correlates other than those required for the diagnosis. Eleven patients, who constituted around 10% of all the patients with Hirayama disease, were found to have bilaterally symmetric involvement. Nine of them had a history of unilateral onset. The important characteristics of this type of presentation included severe weakness and wasting in C7, C8, and T1 myotomes that frequently spilled over to C6 segment, predominant autonomic dysfunction in distal upper extremities in the form of cold paresis, cold skin, excessive sweating, and hair loss over the dorsum of the hands, and a very prominent bilateral minipolymyoclonus. MRI during complete flexion of neck showed severe flattening of lower cervical spinal cord against C5-C6 vertebral bodies and development of a crescent-shaped enhancing epidural space extending from C4 to T2 spine. Bilaterally symmetric Hirayama disease is a severe form of a classic disease which remains undiagnosed due to a common notion that it is a unilateral or grossly asymmetric disease. This description calls for review of the term "brachial monomelic amyotrophy" described to denote this disease.

Differentiation of myoclonic seizures in epileptic syndromes: A video‐polygraphic study of 26 patients

We conducted a video-polygraphic study of myoclonic seizures (MS) in different epileptic syndromes to clarify semiologic and electroencephalography (EEG) differences among them. The subjects were 26 children with MS, including benign myoclonic epilepsy in infants (BME) in 10, severe myoclonic epilepsy in infants (SME) in 6, idiopathic epilepsy with myoclonic-astatic seizures (IEMAS) in 4, and juvenile myoclonic epilepsy (JME) in 6. We reviewed the video-polygraphs of MS, including the predominant area of muscle involvement (neck, trunk, and proximal or distal upper extremities), postural changes including astatic falling, and mode of appearance. We also analyzed the frequency of a corresponding generalized spike-and-wave complex (GSW) and the duration of myoclonic electromyography (EMG) activity. A total of 550 MS were documented in the 26 cases. MS manifested with proximal predominance/forward flexion/single occurrence in BME, proximal predominance/forward astatic flexion/single occurrence in IEMAS, proximal predominance/extension/succession in SME, and distal predominance/extension/succession in JME. The median frequency of GSW was 1.5, 1.3, 3.2, and 3.1 Hz, respectively, and the median duration of the myoclonic EMG activity was 387, 587, 81, and 65 ms, respectively. MS in the four different epileptic syndromes show significant semiologic and EEG differences, as well as similarities. Although our study has the limitations of the small number of patients and retrospective methodology, these results should be considered in the classification and differential diagnosis of myoclonic epileptic syndromes.

A Case Report of Juvenile Asymmetric Muscular Atrophy of Bilateral Distal Upper Extremity: Hirayama Disease

A Case Report of Juvenile Asymmetric Muscular Atrophy of Bilateral Distal Upper Extremity: Hirayama Disease

38. The ionic mechanisms of cold paresis in juvenile muscular atrophy of distal upper extremity (Hirayama disease): A multiple excitability measurement study

38. The ionic mechanisms of cold paresis in juvenile muscular atrophy of distal upper extremity (Hirayama disease): A multiple excitability measurement study

25. Amyotrophy of the distal upper extremities associated with Devic’s disease

25. Amyotrophy of the distal upper extremities associated with Devic’s disease

Comparison of single, transarterial and multiple injection techniques, for axillary brachial plexus block for distal upper extremity surgery

Comparison of single, transarterial and multiple injection techniques, for axillary brachial plexus block for distal upper extremity surgery

104: Comparison of single, transarterial and multiple injection techniques, for axillary brachial plexus block for distal upper extremity surgery

104: Comparison of single, transarterial and multiple injection techniques, for axillary brachial plexus block for distal upper extremity surgery

Teaching Neuro Image : Tremor in aqueductal stenosis and response to endoscopic third ventriculostomy

Idiopathic aqueductal stenosis (AS) may account for up to 59% of cases presenting with triventricular noncommunicating hydrocephalus.1 The clinical presentations associated with triventricular hydrocephalus differ depending on age at onset and the acuity of obstruction. Acute syndromes include Parinaud's syndrome (vertical gaze restriction, lid retraction, and pupillary abnormalities), the rostral midbrain syndrome (upward gaze palsy, retraction nystagmus, pyramidal and extrapyramidal signs), and deficits in arousal. In the very young, chronic diencephalic compression can produce the bobble-head doll syndrome (high frequency head movements, limb ataxia, tremor, and cognitive deficits). Resolution of transtentorial pressure gradients by CSF diversion typically produces rapid improvement.2 We describe an adult patient with upper extremity tremor due to decompensated hydrocephalus from AS, who demonstrated improvement following endoscopic third ventriculostomy. An 18-year-old right-handed African American man presented with chronic, progressive tremor of the right hand. He was the product of an uncomplicated pregnancy and satisfied normative criteria for both growth and developmental milestones including head circumference. There was no history of head injury or encephalitis. At age 9 he developed action tremor in his dominant hand, which began in the distal upper extremity and progressed over 6 months to include the proximal arm and right leg. These symptoms were aggravated by stress, and interfered with activities of daily living. While he was considered less agile than his peers, he had no functional impairment or problems with falls. Rest tremor was not appreciated. By age 13, …