088 Cryoglobulinemic neuropathy associated with lymphoplasmacytic lymphoma

2018 ◽  
Vol 89 (6) ◽  
pp. A35.3-A36
Author(s):  
Yew Li Dang ◽  
Emma Foster ◽  
Moira Finlay ◽  
Andrew Evans
Keyword(s):  
Peripheral Neuropathy ◽  
Upper Limb ◽  
Early Recognition ◽  
Oral Prednisolone ◽  
Livedo Reticularis ◽  
Lower Limbs ◽  
Lymphoplasmacytic Lymphoma ◽  
Hepatitis C Infection ◽  
Limb Weakness

IntroductionA 64 year old woman presented with livedo reticularis and peripheral neuropathy secondary to Type 1 (IgM paraproteinemia) cryoglobulinemia, associated with lymphoplasmacytic lymphoma. Type 1 cryoglobulinemia is rare and remains poorly studied. However, given the common association of Type 1 cryoglobulinemia with lymphoproliferative diseases, it is important to consider Type 1 cryoglobulinemia as a differential diagnosis in presentations of a rash and peripheral neuropathy, and search for underlying malignancy.CaseOur patient presented with a leukocytoclastic vasculitic rash over bilateral lower limbs, which improved with topical betamethasone and oral prednisolone. Two months later, she had rapidly progressive right upper limb and bilateral lower limb weakness, absent reflexes in these limbs, and right arm reduced sensation. Nerve conduction studies revealed generalised axonal sensorimotor peripheral neuropathy in all four limbs, most prominent in the right upper limb. She then developed a livedo reticularis rash over her right forearm and punch biopsy revealed luminal pseudo-thrombi in small vessels consistent with Type 1 cryoglobulinemia. Her cryoglobulin (1558 mg/L, N 0–50 mg/L) and IgM paraprotein levels (4 g/L, N 0.4–2.3) were elevated. Vasculitic and other infective screens were unremarkable. Computer tomography imaging of chest, abdomen and pelvis found widespread lymphadenopathy. Subsequent lymph node core biopsy and bone marrow aspirate revealed lymphoplasmacytic lymphoma—the likely underlying cause of the Type 1 (IgM paraproteinemia) cryoglobulinemia.ConclusionPeripheral neuropathy is commonly associated with Type 2 and 3 cryoglobulinemia, especially in the presence of hepatitis C infection. However, reports of vasculitic peripheral neuropathy due to Type 1 cryoglobulinemia are limited. Early recognition is essential to allow the identification and treatment of the underlying haematological malignancy, commonly associated with Type 1 cryoglobulinemia. Treatment of the underlying cause indirectly treats the cryoglobulinemia, and avoids or reduces the associated; sometimes severe; cutaneous, neurological, and renal manifestations of this condition.

Traumatic upper limb weakness in a man with type 1 neurofibromatosis

2011 ◽  
Vol 82 (8) ◽  
pp. 883-884
Author(s):  
E. S.-T. Ng ◽  
C. K. Ong ◽  
E. Wilder-Smith
Keyword(s):  
Upper Limb ◽  
Limb Weakness ◽  
Type 1 Neurofibromatosis

scholarly journals An Unusual Case of Myonecrosis

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
P. Mukhopadhyay ◽  
R. Barai ◽  
C. A. Philips ◽  
J. Ghosh ◽  
S. Saha
Keyword(s):  
Diabetes Mellitus ◽  
Upper Limb ◽  
Unusual Case ◽  
Case Reports ◽  
Rare Complication ◽  
Quadriceps Muscle ◽  
Diabetes Mellitus Type 1 ◽  
Lower Limbs ◽  
Muscle Infarction

Diabetic Myonecrosis is a rare complication of long-standing Diabetes Mellitus Type 1 and 2. The most likely affected areas are of proximal lower limbs, mostly the quadriceps muscle. The presenting features are myriad and a diagnostic conundrum for the physician. There has been previously mentioned, through few case reports, the classical presentation of diabetes-related muscle infarction. Here we present a patient of diabetic myonecrosis, in whom the initial presentation of diabetes mellitus was that of bilateral symmetric proximal upper limb predominant muscle infarction, which has never been reported before.

scholarly journals Cervical myeloradiculopathy with Hypoglossal Schwannoma mimicking Amyotrophic lateral sclerosis: a Case report

2019 ◽  
Author(s):  
Sathyajith Buddhika Ambawatte ◽  
Dulmini Weerathunga ◽  
Athula Dissanayake ◽  
Surangi Somarathne
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Upper Limb ◽  
Clinical Signs ◽  
Lower Limbs ◽  
Common Disease ◽  
Limb Weakness ◽  
Dual Pathology ◽  
Hypoglossal Schwannoma ◽  
Lateral Sclerosis ◽  
Magnetic Resonant Imaging

Abstract Background: A presentation of upper motor and lower motor clinical signs without sensory impairment may suggest Amyotrophic lateral sclerosis, especially when it involves limbs and bulbar regions. However, co-existence of two or more unrelated pathologies can give rise to a clinical picture similar to Amyotrophic lateral sclerosis. Case presentation: A 45 year old lady presented with slowly progressive right upper limb weakness for 6 months and mild left upper limb weakness for 2 months associated with muscle twitching. Her family members have noticed a change in character of her speech for few weeks. She couldn’t recall a radicular pain at any given time. On examination, her right deltoid was wasted with numerous fasciculations over right proximal muscles. Right shoulder movements were weak. Her left deltoid was slightly wasted and fasciculations were appreciated. Her both biceps and supinator jerks were normal with exaggerated triceps jerks and positive Hoffman sign. Sensory and proprioception examinations were unremarkable. Both lower limbs were hypertonic with exaggerated knee and ankle jerks. Ankle clonus was absent and plantar responses were equivocal. Her tongue was deviated to right side with fascicuations and wasting of right side. Palatal movements and Jaw jerk were normal. She was clinically diagnosed as possible amyotrophic lateral sclerosis. Electromyography showed denervation changes in deltoids and biceps with right predominance and right genioglossus muscle too showed denervation changes. Magnetic resonant imaging of brain stem was undertaken especially because her tongue wasting and fasciculations were unilateral and it showed a right sided hypoglossal schwannoma. Magnetic resonant imaging of cervical spine revealed degenerative disk disease with mild cord compression and exit foramina narrowing at multiple levels. Conclusions: Pure motor symptoms with a mixture of upper motor and lower motor signs may suggest a clinical diagnosis of Amyotrophic lateral sclerosis. However, when there are rare manifestations of a relatively common disease, such as unilateral tongue involvement as in the above scenario, a higher degree of clinical suspicion is needed to think of a dual pathology. Keywords: Hypoglossal schwannoma, cervical myeloradiculopathy, Amyotrophic lateral sclerosis, dual pathology.

Impaired awareness of hypoglycemia in adults with type 1 diabetes is not associated with peripheral neuropathy

2017 ◽  
Vol 12 (S 01) ◽  
pp. S1-S84
Author(s):  
D Tsitlakidis ◽  
M Djordjevic ◽  
N Müller ◽  
UA Müller ◽  
C Kloos
Keyword(s):  
Type 1 Diabetes ◽  
Peripheral Neuropathy ◽  
Impaired Awareness

Computed tomography angiography and microsurgical flaps for traumatic wounds: What is the added value?

2021 ◽  
pp. 1-9
Author(s):  
Lucas Sousa Macedo ◽  
Renato Polese Rusig ◽  
Gustavo Bersani Silva ◽  
Alvaro Baik Cho ◽  
Teng Hsiang Wei ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Upper Limb ◽  
Computed Tomography Angiography ◽  
Lower Limb ◽  
High Energy ◽  
Lower Limbs ◽  
Added Value ◽  
Vascular Lesions ◽  
Limb Injuries ◽  
Significant Difference

BACKGROUND: Microsurgical flaps are widely used to treat complex traumatic wounds of upper and lower limbs. Few studies have evaluated whether the vascular changes in preoperative computed tomography angiography (CTA) influence the selection of recipient vessel and type of anastomosis and the microsurgical flaps outcomes including complications. OBJECTIVE: The aim of this study was to evaluate if preoperative CTA reduces the occurrence of major complications (revision of the anastomosis, partial or total flap failure, and amputation) of the flaps in upper and lower limb trauma, and to describe and analyze the vascular lesions of the group with CTA and its relationship with complications. METHODS: A retrospective cohort study was undertaken with all 121 consecutive patients submitted to microsurgical flaps for traumatic lower and upper limb, from 2014 to 2020. Patients were divided into two groups: patients with preoperative CTA (CTA+) and patients not submitted to CTA (CTA–). The presence of postoperative complications was assessed and, within CTA+, we also analyzed the number of patent arteries on CTA and described the arterial lesions. RESULTS: Of the 121 flaps evaluated (84 in the lower limb and 37 in the upper limb), 64 patients underwent preoperative CTA. In the CTA+ group, 56% of patients with free flaps for lower limb had complete occlusion of one artery. CTA+ patients had a higher rate of complications (p = 0.031), which may represent a selection bias as the most complex limb injuries and may have CTA indicated more frequently. The highest rate of complications was observed in chronic cases (p = 0.034). There was no statistically significant difference in complications in patients with preoperative vascular injury or the number of patent arteries. CONCLUSIONS: CTA should not be performed routinely, however, CTA may help in surgical planning, especially in complex cases of high-energy and chronic cases, since it provides information on the best recipient artery and the adequate level to perform the microanastomosis, outside the lesion area.

scholarly journals Audio-visual stimulation in conjunction with functional electrical stimulation to address upper limb and lower limb movement disorder

2016 ◽  
Vol 26 (2) ◽  
Author(s):  
Deepesh Kumar ◽  
Sunny Verma ◽  
Sutapa Bhattacharya ◽  
Uttama Lahiri
Keyword(s):  
Electrical Stimulation ◽  
Movement Disorder ◽  
Upper Limb ◽  
Functional Electrical Stimulation ◽  
Lower Limb ◽  
Neurological Disorders ◽  
Visual Stimulation ◽  
Lower Limbs ◽  
Lower Limb Movement ◽  
Rehabilitation Exercises

Neurological disorders often manifest themselves in the form of movement deficit on the part of the patient. Conventional rehabilitation often used to address these deficits, though powerful are often monotonous in nature. Adequate audio-visual stimulation can prove to be motivational. In the research presented here we indicate the applicability of audio-visual stimulation to rehabilitation exercises to address at least some of the movement deficits for upper and lower limbs. Added to the audio-visual stimulation, we also use Functional Electrical Stimulation (FES). In our presented research we also show the applicability of FES in conjunction with audio-visual stimulation delivered through VR-based platform for grasping skills of patients with movement disorder.

A variant of the gene HARS detected in the clinical exome: etiology of a peripheral neuropathy undiagnosed for 20 years

2020 ◽  
Vol 0 (0) ◽  
Author(s):  
Raquel Lahoz Alonso ◽  
Paula Sienes Bailo ◽  
Jose Luis Capablo Liesa ◽  
Sara Álvarez de Andrés ◽  
Jose Luis Bancalero Flores ◽  
...  
Keyword(s):  
Peripheral Neuropathy ◽  
Neurological Disease ◽  
Motor Impairment ◽  
Lower Limbs ◽  
Case Presentation ◽  
Genetic Sequencing ◽  
Charcot Marie Tooth ◽  
Sensory Motor ◽  
Exacerbation Of Symptoms ◽  
Over Time

AbstractObjectivesDescribe a case with axonal Charcot-Marie-Tooth (CMT) type 2W, a neurological disease characterized by peripheral neuropathy typically involving the lower limbs and causing gait alterations and distal sensory-motor impairment.Case presentationWe report this case, where the application of massive genetic sequencing (NGS) with clinical exome in a molecular genetics laboratory enabled to detect the presence of candidate variants of the clinic of the patient.ConclusionsThe variant detected in HARS gene suggests that this variant could be causative of the symptoms of the patient, who went undiagnosed for 20 years and experienced an exacerbation of symptoms over time.

Case Report: Atypical Cutaneous Presentation of Human T-cell Lymphotropic Virus Type 1-Related Adult T-cell Lymphoma

2021 ◽  
Author(s):  
Gianluca Avallone ◽  
Mattia Trunfio ◽  
Luca Mastorino ◽  
Andrea Agostini ◽  
Martina Merli ◽  
...  
Keyword(s):  
T Cell ◽  
Virus Type ◽  
Significant Risk ◽  
Clinical Work ◽  
Lower Limbs ◽  
Interferon Α ◽  
Adult T Cell Leukemia ◽  
Human T Cell ◽  
Diagnostic Delays

Human T-cell lymphotropic virus type 1 (HTLV-1) is a retrovirus endemic in many parts of the world. Because of migration, cases of HTLV-1 in non-HTLV-1 endemic countries have been increasingly reported. Clinical presentation of HTLV-1 infection is highly variable, with a significant risk of diagnostic delays. Skin can be the first site affected by HTLV-1-related manifestations such as cutaneous involvement of adult T-cell leukemia/lymphoma (ATLL) and infective dermatitis associated with HTLV-1. A 32-year-old Nigerian man was admitted to the infectious disease department for high fever, asthenia, lymphocytosis, and vesicular bullous lesions on both hand palms and lower limbs. After clinical work-up was performed, bacterial superinfected herpes simplex viurs-2 ulcers were the presenting sign of HTLV-1-related chronic type ATLL. Standard treatment based on interferon-α plus zidovudine was started, but it was poorly tolerated; therefore, switching to an off-label dual antiretroviral regimen was attempted. The increasing prevalence of HTLV-1 in nonendemic areas may enhance the development of alternative treatments with better efficacy and tolerability profiles.

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