Distal Parts Of Limbs Research Articles

Unusual presentations of cutaneous tuberculosis

Cutaneous tuberculosis occurs in 1–2% of world cases of tuberculosis and more common in tropical countries. It presents with different clinical forms. Unusual clinical presentations are not uncommon and awareness of these will help in suspecting and managing these patients successfully. Lupus pernio like lupus vulgaris, tuberculosis of glans penis and lichen scrofulosorum on the distal parts of limbs are presented here because of their unusual clinical presentation.

Distal limb weakness phenotype of Guillain-Barré syndrome

ObjectiveSeveral regional variants of Guillain-Barré syndrome (GBS) have been proposed in western countries, but other variants remain unclear, especially among mildly disabled cases. The aim of this study was to identify unvalidated GBS phenotypes among Japanese patients with mild GBS. MethodsRetrospective study of a cohort of patients at a University Hospital in Japan. ResultsAmong 107 GBS patients, 25 (23%) were classified as having mild GBS (GBS disability scale ≤ 2 at nadir). A review of mild cases identified 8 patients (7.5% of all GBS and 32% of mild GBS) with an unusual phenotype, namely a distal limb weakness form of GBS (DL-GBS), which showed limited distribution of limb weakness within hands and feet with preserved strength in proximal limb muscles. The patients with DL-GBS were characterized by mild-to-moderate weakness in distal parts of limbs especially fingers, lacking or mild sensory disturbance at distal limbs, sometimes hyperreflexia at legs, and having prior Campylobacter jejuni enteritis. Among the patients with GBS after C. jejuni enteritis, DL-GBS patients were characterized by frequent detection of anti-GM1 antibodies without anti-GD1a antibodies, whereas the others were often positive for the two antibodies. ConclusionsDL-GBS is a distinct regional phenotype of GBS, which should be differentiated from cervical myelopathy. It can be generally categorized as a mild type of GBS after C. jejuni enteritis, which has characteristic pattern of anti-ganglioside autoantibodies.

DHTKD1 Deficiency Causes Charcot-Marie-Tooth Disease in Mice.

DHTKD1, a part of 2-ketoadipic acid dehydrogenase complex, is involved in lysine and tryptophan catabolism. Mutations in DHTKD1 block the metabolic pathway and cause 2-aminoadipic and 2-oxoadipic aciduria (AMOXAD), an autosomal recessive inborn metabolic disorder. In addition, a nonsense mutation in DHTKD1 that we identified previously causes Charcot-Marie-Tooth disease (CMT) type 2Q, one of the most common inherited neurological disorders affecting the peripheral nerves in the musculature. However, the comprehensive molecular mechanism underlying CMT2Q remains elusive. Here, we show that Dhtkd1-/- mice mimic the major aspects of CMT2 phenotypes, characterized by progressive weakness and atrophy in the distal parts of limbs with motor and sensory dysfunctions, which are accompanied with decreased nerve conduction velocity. Moreover, DHTKD1 deficiency causes severe metabolic abnormalities and dramatically increased levels of 2-ketoadipic acid (2-KAA) and 2-aminoadipic acid (2-AAA) in urine. Further studies revealed that both 2-KAA and 2-AAA could stimulate insulin biosynthesis and secretion. Subsequently, elevated insulin regulates myelin protein zero (Mpz) transcription in Schwann cells via upregulating the expression of early growth response 2 (Egr2), leading to myelin structure damage and axonal degeneration. Finally, 2-AAA-fed mice do reproduce phenotypes similar to CMT2Q phenotypes. In conclusion, we have demonstrated that loss of DHTKD1 causes CMT2Q-like phenotypes through dysregulation of Mpz mRNA and protein zero (P0) which are closely associated with elevated DHTKD1 substrate and insulin levels. These findings further indicate an important role of metabolic disorders in addition to mitochondrial insufficiency in the pathogenesis of peripheral neuropathies.

Ultrasound elastography as a method of emergency diagnosis of the first signs of transient ischemia of the lower limbs in diabetic foot syndrome

Aim. Evaluate the data of elastography characteristic for the first signs of transient ischemia of lower limbs in diabetes foot syndrome.Methods. In Kazan city clinical hospital №7 measurement of Young’s modulus of elasticity was performed in 12 patients with transient ischemia in diabetic foot syndrome. The methods and assessment of parameters typical for soft tissues of lower limbs we have developed earlier (patent «Method of elastography diagnosis of tissue changes in diabetic foot syndrome»). Color duplex scan of the arteries was performed according to standard method with the use of 5 to 10 MHz linear array probe starting from distal parts of limbs. Measurement of ultrasound density was performed with the use of shear wave elastography with 4 to 15 MHz wide-band linear probe. All parameters were measured in real time mode.Results. Increase of elastomeric index (Young’s modulus of elasticity) to 23.74±2.34 kPa (93,49±1,26%) in the distal segment of the lower extremity (on the foot) is associated with development of transient disorders in the lower limbs in diabetic foot syndrome, which is not always detected with invasive methods of diagnosis.Conclusion. Shear wave elastography is recommended for emergency medical care in diabetic foot syndrome; patients after being diagnosed with type 2 diabetes should dynamically monitor microcirculation of lower limbs that will decrease the risk of need of surgical treatment.

Addition of Dexmedetomidine to Low Dose Solution of Lidocaine for Intravenous Regional Anesthesia A Randomized Study

Background and Aims: Intravenous Regional Anesthesia (IVRA) provides analgesia of distal part of limb by intravenous injection of local anesthesia in to the vein of the same limb, while the circulation to the limb is occluded by application of tourniquet. However, IVRAis limited by local anesthesia toxicity, tourniquet pain and absence of postoperative analgesia. Various additives to local anesthetics such as ketamine, clonidine, opiods, NSAIDs are used to address these issues. The aim of our study was to evaluate the effectiveness of Dexmedetomidine to low dose Lidocaine solution for IVRA.Patients and Methods: A prospective, randomized double blind study was conducted on 60 patients divided in to two groups of thirty each; Group I patients received 40 ml of Lidocaine 0.25% while Group II patients received 40 ml of 0.25% of lidocaine mixed with I mcg/kg of dexmedetomidine. The motor and sensory block onset and recovery times were assessed. Tourniquet pain and sedation score were assessed intraoperatively and postoperatively.Results: Sensory and motor block onset times were shorter and recovery times were prolonged in Dexmedetomidine group. The quality of anesthesia was better in the Dexmedetomidine group and the fentanyl dose required was also lower in the dexmedetomidine group. There was a significant difference in the postoperative analgesia time which was demonstrated by less use of intramuscular diclofenac in dexmedetomidine group.Conclusions: A safe intravenous dose of lidocaine can be used for IVRA for superficial hand surgery, and the addition of I mcg /kg dexmedetomidine shortened the onset times for both sensory and motor blockade and improved the quality of anesthesia, with prolonged postoperative analgesia time. By adding dexmedetomidine we were able to reduce the dose of lidocaine to 0.25% concentration without compromising analgesia. JMS 2016; 19(1):21-25

Clinical and electrophysiological characteristics and therapeutic analysis of 9 patients with Lewis-Sumner syndrome

To investigate the clinical characteristics, electrophysiological findings and treatment response of Lewis-Sumner syndrome (LSS). Data of nine patients with LSS, who were diagnosed and treated from May 2008 to August 2014 in Department of Neurology, Peking University Third Hospital, were analyzed retrospectively, including clinical features, electrophysiological studies, pathological characteristics, therapy and follow up. The nine cases included seven males and two females, with the average age being 29 years old (18-64 years old). The follow-ups after treatment were between 6 months to 48 months. All 9 cases were asymmetrical onset, and the distal part of limbs was initially affected in 8 cases. Five cases presented with the initial symptom of sensorimotor, 3 cases with purely motor and only 1 case with sensory. The disease developed two forms: multifocal distribution and symmetrical distribution. Electrophysiological studies demonstrated conduction blocks on non-entrapment sites, involving mainly median nerve and ulnar nerve. Sural nerve biopsy presented mild demyelination without inflammatory infiltration. The symptoms of patients had some degrees of improvement after immunotherapy, and the prognosis was favorable. Lewis-Sumner syndrome presents the initial symptoms of asymmetrical sensorimotor neuropathy mostly affecting the upper extremities. Median nerve and ulnar nerve are the most common involvements. Electrophysiological studies demonstrate motor nerve conduction block. The major finding of pathology is mild demyelinated. The patients have a positive response to immunotherapy.

КЛИНИЧЕСКИЕ ЛАТЕРАЛИЗАЦИОННЫЕ ПРИЗНАКИ ПРИ СИМПТОМАТИЧЕСКОЙ ВИСОЧНОЙ ЭПИЛЕПСИИ

Abstract: the article presents a review of world literature on significance of various clinical lateralizing signs in temporal lobe epilepsy, such as dystonic posturing, limb automatisms, ictal and postictal aphasia, ictal nystagmus, head turning, periiktal headache, etc. The results of current research are compared with previously published reports. The ictal dystonic unilateral limb posturing (possibly isolated in a hand, foot, predominantly in distal parts of limbs) is still one of the most informative lateralizing signs and it is lateralized in contralateral hemisphere in 100% of cases. It is shown that the “early” and “late” head turning, and unilateral limb automatisms are the most important and convincing lateralizing signs, which must be considered in connection with the EEG data to determine the epileptogenic focus in patients with temporal lobe epilepsy.

Management of general anesthesia for a patient with Maroteaux type acromesomelic dysplasia complicated with obstructive sleep apnea syndrome and hereditary myopathy

To the Editor: Oropharynx abnormality may lead to difficult intubation in patients with osteochondrodysplasia [1]. For the very rare disease, acromesomelic dysplasia Maroteaux-type (AMDM), which is one such abnormality, however, there is no report whether special consideration is needed in airway management. AMDM is seldom combined with other significant disease [2]. We report the first case of a patient with AMDM complicated by severe obstructive sleep apnea syndrome (OSAS) and hereditary myopathy undergoing smooth management of general anesthesia for elective L4–S1 laminectomy and discectomy. AMDM, which was first described by Maroteaux et al. in 1971 [3], is a very rare autosomal recessive disorder of progressive osteochondrodysplasia with a prevalence of about 1 in 2,000,000 [2]. It is characterized by severe dwarfism and acromesomelia, which presents with disproportionately shortened middle and distal segments of the four limbs. The 33-year-old female patient suffered from AMDM, which was diagnosed 13 years ago based on gene mapping (Affymetrix Genechip Mapping 100K set), in which the linkage data showed abnormality in chromosome 9 [4], and on typical clinical features, such as short stature with disproportionately shortened distal part of limbs (Fig. 1). She also had severe OSAS with apnea-hypopnea index up to 48, and had received treatment of bi-level positive airway pressure for 3 years [5]. Muscle biopsy performed 2 years ago in response to progressive muscle weakness had suggested hereditary muscle atrophy, which was not considered AMDM related. Preoperative evaluation revealed no significant head deformity except prognathia. Neck range of motion was normal. Mallampati classification was grade 2. Muscle power of the four limbs was four, worse in the distal parts. Preoperative laboratory data, chest X-ray, and pulmonary function test were unremarkable. Under train-of-four (TOF) monitoring over the adductor pollicis, induction agents of fentanyl 100 lg, 2 % lidocaine 40 mg, propofol 100 mg, and cisatracurium 7 mg were given intravenously after sufficient preoxygenation. When the patient was apneic, an oropharyngeal airway was necessary for mask ventilation. When the second twitch of TOF disappeared, endotracheal intubation using direct laryngoscope was performed uneventfully under the view of Cormack and Lehane classification grade one. After a 2-h surgical procedure, tracheal extubation was performed under fully awake conditions and recovery of TOF in the operating room. The patient was then discharged smoothly on the fourth postoperative day without any sequelae of general anesthesia. As this is a very rare case report, we do not know whether patients with AMDM may have a difficult airway. In such a situation, succinylcholine may be the first choice P.-C. Huang J.-H. Chang K.-B. Chen (&) Department of Anesthesiology, China Medical University Hospital, No. 2, Yuh-Der Road, North District, Taichung 40447, Taiwan, ROC e-mail: pppat21@yahoo.com.tw

Hamartoma angiomatoso ecrino: presentación de dos casos

Eccrine angiomatous hamartoma is a rare, benign tumor characterized by a proliferation of eccrine and vascular structures. We present 2 cases and review the characteristics of this disorder. The first patient was a 33-year-old woman who consulted for a brownish lesion on her back associated with local hyperhidrosis. The second patient was a 25-year-old man with an asymptomatic erythematous lesion on his left palm. In both patients a diagnosis of eccrine angiomatous hamartoma was made based on the histological findings. Eccrine angiomatous hamartoma is usually present at birth or develops during childhood. It is typically a solitary lesion and signs and symptoms can vary; diagnosis is therefore based on histological study. The most common site is on the distal parts of limbs. The lesions tend to be asymptomatic, but there may be associated pain and hyperhidrosis. Treatment is not usually necessary except in cases with persistent symptoms, excessive sweating, or cosmetic concerns.

Manifestation of morphofunctional specificity of muscles in spectral evaluations of the surface electromyogram

It is shown that, when the global recording method is used, the muscles of upper limbs (in comparison with lower ones) and of distal parts of limbs (in comparison with proximal ones) generate wider surface electromyograms (EMGs) with a higher dominant frequency and lower saturation of power spectra owing to the specific features of the morphofunctional organization of both the muscles proper and corticospinal systems controlling their voluntary contractive activity. There is an interrelation among different spectral and amplitude-frequency response (EMG analysis according to Willison) characteristics of the surface EMG. Limb lengthening may be considered, on the basis of criteria of changes in spectral characteristics of the EMG of lengthened segment muscles and results of its analysis according to Willison, as a model of a decrease in reliability of corticospinal connections.

Australian wolf spider bites (Lycosidae): clinical effects and influence of species on bite circumstances.

Necrotic arachnidism continues to be attributed to wolf spider bites. This study investigates the clinical effects of bites by wolf spiders in Australia (family Lycosidae). Subjects were recruited prospectively from February 1999 to April 2001 from participating emergency departments or state poison information centers. Subjects were included if they had a definite bite by a wolf spider and had collected the spider, which was later identified by an arachnologist. Spiders were identified to the lowest taxonomic level possible and cephalothorax width was measured to correlate bite effects and spider size. There were 45 definite wolf spider bites (23 male and 22 female patients; age range 1 to 69 years, median age 28 years). Species level identifications (14 species) were possible for 31 of 43 spiders belonging to seven different generic groupings. Most bites were by spiders from four generic groupings, Tasmanicosa (including 'Lycosa') (15), Venatrix (8), Venator (10), and Hogna (7). Bites occurred more commonly in south-eastern Australia and occurred throughout the year, with 7 bites (16%) in late autumn or winter. In 7 cases (16%) the person was swimming in or cleaning a pool. Seventy-two percent of bites occurred on distal parts of limbs. Pain occurred in all bites and was severe in 11 cases (24%), with a median duration of 10 min (IQR: 2-60 min). Other effects included puncture marks/bleeding (33%), swelling (20%), redness (67%), and itchiness (13%). Minor systemic effects occurred in three patients (7%): nausea (two), headache (one) and malaise (one). There were no cases of necrotic ulcers [0%; 97.5% CI 0-8%]. Tasmanicosa spider bites caused significantly more itchiness and redness, and large spiders (>5 mm) more often caused severe pain and left fang marks. Wolf spider bites cause minor effects, no more severe than most other spiders, and do not appear to cause necrotic ulcers. The effects are likely to be due to mechanical injury, although minor local envenomation occurs with Tasmanicosa bites.

Treatment of Vitiligo with Oral 5‐Methoxypsoralen

Thirty-six patients with vitiligo were treated with oral 5-methoxypsoralen (5-MOP) and subsequently exposed to UVA irradiation. The patients were treated once or twice weekly over a period of 2-10 months, taking 40-60 mg of 5-MOP 2 hours before exposure to UVA light. The amount of exposure to UVA light was slowly increased according to the patient's tolerance. Eleven (31%) patients showed remarkable repigmentation of the areas of vitiligo within six months. Overall, 78% of the patients showed effective repigmentation. Areas of vitiligo on the face and trunk were more responsive to treatment than those on the distal part of limbs. Adverse effects due to the drug included 2 patients with nausea and 1 patient with headache. It is suggested that treatment with systemic 5-MOP is effective, safe, and useful in selected cases of vitiligo.

Heat exchanges in wet suits.

Flow of water under foam neoprene wet suits could halve insulation that the suits provided, even at rest in cold water. On the trunk conductance of this flow was approximately 6.6 at rest and 11.4 W . m-2 . C-1 exercising; on the limbs, it was only 3.4 at rest and 5.8 W . m-2 . degrees C-1 exercising; but during vasoconstriction in the cold, skin temperatures on distal parts of limbs were lower than were those of the trunk, allowing adequate metabolic responses. In warm water, minor postural changes and movement made flow under suits much higher, approximately 60 on trunk and 30 W . m-2 . degrees C-1 on limbs, both at rest and at work. These changes in flow allowed for a wide range of water temperatures at which people could stabilize body temperature in any given suit, neither overheating when exercising nor cooling below 35 degrees C when still. Even thin people with 4- or 7- mm suits covering the whole body could stabilize their body temperatures in water near 10 degrees C in spite of cold vasodilatation. Equations to predict limits of water temperature for stability with various suits and fat thicknesses are given.

Anatomical and joint dissection studies of tissue weight distribution in buffalo bulls and cows: Part 1—Distribution of muscle and bone

Carcasses from six buffalo bulls (slaughtered between 430 and 560 kg liveweight) and six buffalo cows (slaughtered between 405 and 583 kg liveweight) were dissected, the left sides by the anatomical approach, the right sides being jointed and the joints being dissected into fat, muscle, bone and other tissues. Weights of the anatomically dissected muscles and bones, and weights of muscle and bone dissected from each cut, were regressed on weight of the respective tissue in the side. The influence of the animals' sex on tissue distribution was investigated using a one-way analysis of covariance. Results were consistent with those reported on cattle in that (i) sex had no influence on the amount of total muscle occurring in the distal parts of limbs or surrounding the spinal column and connecting the fore limb to the thorax and neck, (ii) females had more of their total muscle in the flank or abdominal wall and less in the loin than males, and (iii) females tended to have less of their total side bone in the appendicular skeleton and more in the axial skeleton than males. However, the influence of the animal's sex on the amount of total muscle in the proximal parts of its limbs was significant in buffaloes but not in cattle; the reverse was true for the muscle group of the thorax and neck. The overall sex differences in bone-weight distribution for buffaloes were as small as previously reported for cattle, sheep and pigs. Features of better muscle-weight distribution in buffalo males as compared with females and in Egyptian buffaloes as compared with Australian buffaloes and British cattle breed types are discussed.

Foraging Relationships of Brown‐Headed Nuthatches and Pine Warblers

Foraging behavior and interactions between Brown—headed Nuthatches (Sitta pusilla) and Pine Warblers (Dendroica pinus) were studied in longleaf pine lands of Louisiana during the fall and winter of 1963—64 and 1964—65. Both species frequently were members of flocks that formed around Carolina Chickadees (Parus carolinensis) and Tufted Titmice (P. bicolor). Most contact between the former two species occurred in these flocks. While in flocks, Brown—headed Nuthatches foraged most heavily on the distal parts of limbs and on twigs, Pine Warblers on proximal parts of limbs. When alone, Brown—headed Nuthatches foraged more heavily on trunks and proximal parts of limbs than they did when Pine Warblers. When pine seeds were abundant, Brown—headed Nuthatches fed upon them heavily and regularly ventured to large limbs and trunks to crack seeds, causing increased hostilities with Pine Warblers. Attempts to crack seeds where bark was not sufficiently rough to permit adequate anchoring met with little success. Pine Warblers fed upon pine seeds much less frequently than did Brown—headed Nuthatches. During the height of seed abundance, Brown—headed Nuthatches lingered behind mixed flocks and often became separated from them. More competition for food apparently exists between Brown—headed Nuthatches and Pine Warblers than between either of these species and others. The noticeably different foraging distribution of the two species when together and apart is probably largely the result of the presence or absence of the other. Dividing the habitat in each other's presence avoids fighting over a similar foraging station. Abundant food sources may cause this system to break down.