scholarly journals Waldenstrom-associated anti-MAG paraprotein polyneuropathy with neurogenic tremor

2019 ◽  
Vol 12 (3) ◽  
pp. e228376
Author(s):  
Carlo Canepa
Keyword(s):  
Nerve Conduction ◽  
Sensory Neuropathy ◽  
Ataxic Gait ◽  
Minimal Improvement ◽  
History Of ◽  
Immunoglobulin Treatment ◽  
Myelin Associated Glycoprotein ◽  
Kappa Light Chains ◽  
Lesion Treatment ◽  
Vibration Perception

A 71-year-old female patient presented with a 14-year history of slowly progressive distal limb numbness, paraesthesia and reduced vibration perception, ataxic gait and intentional tremor. Examination revealed with a length-dependent sensory neuropathy. Nerve conduction studies showed a chronic sensorimotor inflammatory demyelinating polyneuropathy. Intravenous immunoglobulin treatment (on two occasions) proved ineffective. Serum electrophoresis showed increased monoclonal IgM with kappa light chains. Anti-myelin-associated glycoprotein (MAG) levels were extremely elevated, >70 000 BTU. Bone marrow biopsy revealed 15%–20% small B cells and positive MYD88 mutation, indicative of Waldenstrom macroglobulinaemia. A diagnosis of Waldenstrom-associated anti-MAG paraprotein neuropathy with intentional (neurogenic) tremor was made. Repeat nerve conduction study showed a severe sensory demyelinating neuropathy with no axonal lesion. Treatment with rituximab was given for 1 month with minimal improvement. Repeat anti-MAG levels dropped to 53 670 BTU, with minimal clinical improvement.

Minocycline-Induced Agranulocytosis Presenting as Ecthyma Gangrenosum

2017 ◽  
Vol 1 (3) ◽  
pp. 161-164 ◽  
Author(s):  
Katherine Nolan ◽  
Reema Ishteiwy ◽  
John Alexis ◽  
Martin Zaiac ◽  
Anna Nichols
Keyword(s):  
Care Facility ◽  
Urgent Care ◽  
Severe Neutropenia ◽  
Dramatic Improvement ◽  
Therapy Discontinuation ◽  
Incision And Drainage ◽  
Ecthyma Gangrenosum ◽  
Minimal Improvement ◽  
History Of ◽  
Acute And Chronic Inflammation

A 51-year-old female with a history of rheumatoid arthritis was admitted for progressive fevers, chills and malaise. Five weeks prior, she started minocycline for an RA exacerbation. Two weeks after starting minocycline she developed an abscess on her right ankle that was treated at an urgent care facility with ceftriaxone and trimethoprim-sulfamethoxazole. She had minimal improvement so was switched to clindamycin. She developed additional abscesses on her right ankle and right axilla and spiking fevers so she was treated with incision and drainage under general anesthesia. Routine blood work obtained prior to surgery revealed severe neutropenia (0.74 103/ul) and the patient was urgently referred to the emergency department.  Skin biopsy was obtained on admission and revealed ulceration, necrosis, acute and chronic inflammation, vasculitis with vascular thrombosis and rod-shaped bacteria in blood vessel walls and lumina consistent with ecthyma gangrenosum. The following day tissue and blood cultures confirmed the growth of Pseudomonas aureginosa. Bone-marrow biopsy showed decreased granulopoiesis and hematopoiesis, and a diagnosis of minocycline-induced agranulocytosis presenting as ecthyma gangrenosum was made.  The patient had dramatic improvement with appropriate antibiotic therapy, discontinuation of minocycline and initiation of filgrastrim. She has remained healthy without recurrence for 17 months.    

845 Progressive external ophthalmoplegia in sleep apnea presenting as floppy eyelid syndrome

SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A329-A329
Author(s):  
Pratibha Anne ◽  
Rupa Koothirezhi ◽  
Ugorji Okorie ◽  
Minh Tam Ho ◽  
Brittany Monceaux ◽  
...  
Keyword(s):  
Obstructive Sleep Apnea ◽  
Surgical Treatment ◽  
Sleep Apnea ◽  
Genetic Disorder ◽  
Deltoid Muscle ◽  
Progressive External Ophthalmoplegia ◽  
Ataxic Gait ◽  
Pressure Setting ◽  
Obstructive Sleep ◽  
History Of

Abstract Introduction Floppy eye lid syndrome (FES) is known to be associated with Obstructive sleep apnea (OSA) and chronic progressive external ophthalmoplegia (CPEO) is a rare genetic disorder with mitochondrial myopathy that may present with isolated eye lid ptosis in the initial stages. In a patient with loud snoring and obesity, treating obstructive sleep apnea may improve Floppy eyelid syndrome. Report of case(s) 52-year-old African – American male with past medical history of Hypertension, obesity, glaucoma, CPEO status bilateral blepharoplasty with failed surgical treatment. Patient was referred to Sleep medicine team to rule out Obstructive Sleep Apnea aa a cause of possible underlying FES and residual ptosis. On exam, patient was noted to have bilateral brow and eyelid ptosis and mild ataxic gait. MRI brain with and without contrast was unremarkable. Deltoid muscle biopsy was suggestive of possible congenital myopathy and mild denervation atrophy. Polysomnogram showed severe OSA with AHI of 74.1 per hour and patient was initiated on Auto CPAP at a pressure setting of 7–20 cm H2O. CPAP treatment improved snoring, OSA and subjective symptoms of excessive day time sleepiness but did not improve the residual ptosis. Conclusion Treatment of severe OSA in a patient previously diagnosed with CPEO and failed surgical treatment with bilateral blepharoplasty, did not alter the course of residual ptosis/ floppy eyelids even though his other sleep apnea symptoms have improved. Support (if any) 1. McNab AA. Floppy eyelid syndrome and obstructive sleep apnea. Ophthalmic Plast Reconstr Surg. 1997 Jun;13(2):98–114. doi: 10.1097/00002341-199706000-00005. PMID: 9185193.

Sciatic Nerve Conductivity is Impaired by Hamstring Strain Injuries

2017 ◽  
Vol 38 (11) ◽  
pp. 803-808 ◽  
Author(s):  
Karina Kouzaki ◽  
Koichi Nakazato ◽  
Masuhiko Mizuno ◽  
Tooru Yonechi ◽  
Yusuke Higo ◽  
...  
Keyword(s):  
Sciatic Nerve ◽  
Conduction Velocity ◽  
Nerve Conduction ◽  
Nerve Conduction Velocity ◽  
Control Group ◽  
Injured Limb ◽  
Hamstring Strain ◽  
History Of

AbstractThe aim of this study was to assess sciatic nerve conductivity in athletes with a history of hamstring strain injuries. Twenty-seven athletes with a history of hamstring strain injuries were included in the injured group. The control group consisted of 16 uninjured participants. We measured the proximal and distal latencies and calculated the sciatic nerve conduction velocity to evaluate neuronal conductivity. The results were expressed as median values and interquartile ranges. Both proximal latency and distal latency of the injured limb in the injured group were significantly longer than those of the uninjured limb (p<0.05). The nerve conduction velocity of the injured limb in the injured group was significantly lower than that of the uninjured limb (p<0.05). There were no significant side-to-side differences in the control group. Sciatic nerve conductivity impairments may exist in athletes with a history of hamstring strain injuries.

Ganglionopathies Associated with MERRF Syndrome: An Original Report

2020 ◽  
Vol 7 (4) ◽  
pp. 419-423
Author(s):  
Maud Michaud ◽  
Tanya Stojkovic ◽  
Thierry Maisonobe ◽  
Anthony Behin ◽  
Benoit Rucheton ◽  
...  
Keyword(s):  
Nerve Conduction ◽  
Mitochondrial Diseases ◽  
Sensory Neuropathy ◽  
Nerve Conduction Studies ◽  
Myoclonic Epilepsy ◽  
Sensory Abnormalities ◽  
Ragged Red Fibers ◽  
Original Report ◽  
Merrf Syndrome

Neuropathies in Myoclonic Epilepsy with Ragged Red Fibers (MERRF) syndrome are frequent but ganglionopathies have never been reported. We retrospectively identified 24 patients with MERRF mutations in the neuromuscular center Nord/Est/Ile de France (Pitié-Salpêtrière, Paris, France). Seventeen nerve conduction studies (NCS) were available. Five patients had MERRF syndrome and ganglionopathy, a pure sensory neuropathy. All of them displayed ataxia and mild clinical sensory abnormalities. Ganglionopathies have been reported in mitochondrial diseases but never in MERRF syndrome. We suggest that patients presenting with ganglionopathy, especially if associated with myopathy, lipomatosis or epilepsy, should be screened for MERRF mutations.

Surgical treatment of a cerebral brain abscess in a cat

2011 ◽  
Vol 24 (01) ◽  
pp. 72-75 ◽  
Author(s):  
M. Beukers ◽  
L. F. H. Theyse ◽  
E. G. H. Wouters
Keyword(s):  
Magnetic Resonance Imaging ◽  
Histopathological Examination ◽  
Resonance Imaging ◽  
Ataxic Gait ◽  
Pupillary Light ◽  
Small Defect ◽  
History Of ◽  
The Right ◽  
Extensive Necrosis ◽  
Temporal Lobe Lesion

SummaryA nine-year-old male castrated European Shorthair cat was presented with a six-day history of progressive depression and ataxic gait. Neurological examination revealed depression, absent menace in the left eye, absent pupillary light reflex in the right eye, anisocoria, circling to the right, and delayed proprioception in all limbs. Magnetic resonance imaging showed a space-occupying right temporal lobe lesion adjacent to a small defect in the temporal bone suggestive of a meningo-encephalitis with concurrent abscess formation. The site was surgically approached by a rostrotentorial craniectomy. A cerebral abscess was found and debrided. Histopathological examination of the removed tissue demonstrated a subacute to chronic purulent encephalitis with extensive necrosis of brain tissue. Neurological symptoms resolved completely within two weeks and full recovery was observed four weeks after surgery.

Brain tumour? – Cerebellar histoplasmosis as a solitary mass lesion

2019 ◽  
Vol 30 (12) ◽  
pp. 1218-1220 ◽  
Author(s):  
Michael Riste ◽  
Neena Bodasing ◽  
Anthony Cadwgan
Keyword(s):  
Histoplasma Capsulatum ◽  
Brain Magnetic Resonance Imaging ◽  
Brain Biopsy ◽  
Ataxic Gait ◽  
Stereotactic Brain Biopsy ◽  
Cd4 Cell Count ◽  
Immunodeficiency Virus ◽  
History Of ◽  
Cd4 Cell ◽  
Inflammatory Syndrome

A 50-year-old man who had been living in Thailand presented with a history of falls, deteriorating vision and weight loss over several months. He had been admitted to a hospital in Thailand where he was given a diagnosis of multiple sclerosis. Neurological examination revealed a mild ataxic gait and lateral nystagmus, but no other abnormalities. He tested positive for human immunodeficiency virus with a CD4 cell count of 16 cells/µL. Brain magnetic resonance imaging was suggestive of an intrinsic neoplasm and he underwent stereotactic brain biopsy which showed numerous yeast-like organisms. Panfungal polymerase chain reaction was positive for Histoplasma capsulatum. He received liposomal amphotericin B for six weeks, followed by itraconazole, and started antiretroviral therapy four weeks into treatment. He developed an immune reconstitution inflammatory syndrome which responded well to steroids. Six months after diagnosis, he has no neurological symptoms or signs and remains on itraconazole. Isolated bulky central nervous system histoplasmomas are exceedingly rare. A clinical suspicion of immunosuppression was key in making this diagnosis.

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