Recurrent Miller Fisher: A Case Report Along With a Literature and an EMG/NCS Review

2021 ◽  
pp. 194187442098705
Author(s):  
Jonathan Morena ◽  
Bakri Elsheikh ◽  
J. Chad Hoyle
Keyword(s):  
White Blood Cells ◽  
Sensory Nerve ◽  
Blink Reflex ◽  
Gad Antibodies ◽  
Lower Extremity Weakness ◽  
Elevated Protein ◽  
Sensory Nerve Action ◽  
History Of ◽  
Gad65 Antibody ◽  
Sensory Polyneuropathy

MFS has been reported to recur in 10-12% of patients. There may be a genetic component related to HLA-DR2. Anti-GAD antibodies can be present in MFS along with anti-GQ1b. Common EMG/NCS associations consist of a predominantly axonal, sensory polyneuropathy and absent H reflexes. A 32-year-old female with a history of hypothyroidism presented to our institution twice with symptoms of diplopia, lower extremity weakness and distal paresthesias occurring a year apart. She had ophthalmoplegia, reduced reflexes, and ataxia on exam. CSF showed a borderline elevated protein of 47 and white blood cells <3. She was positive for anti-GQ1b both times. Her anti-GAD65 antibody was elevated during both admissions. EMG/NCS on her first admission revealed comparatively reduced sensory nerve action potentials (SNAPs) and a normal blink reflex. Her SNAPs improved on the second admission, however, the EMG was performed only 2 days after the onset of her symptoms, limiting some early findings that may have not matured electrophysiologically. She was treated with IVIG on both occasions with rapid recovery within 5 days. This case highlights the fact that MFS can be recurrent. It also provides further evidence that anti-GAD antibodies may be associated with MFS. Reported findings of the blink reflex in MFS are diverse and further data is needed to determine if certain findings are more predominant than others. Treatment typically consists of IVIG, though steroids may also be considered for recurrence. Prognosis is generally favorable, regardless of treatment.

scholarly journals Electrophysiological diagnosis using sensory nerve action potential for the intraforaminal and extraforaminal L5 nerve root entrapment

2012 ◽  
Vol 22 (4) ◽  
pp. 833-839 ◽  
Author(s):  
Muneharu Ando ◽  
Tetsuya Tamaki ◽  
Mamoru Kawakami ◽  
Akihito Minamide ◽  
Yukihiro Nakagawa ◽  
...  
Keyword(s):  
Action Potential ◽  
Nerve Root ◽  
Sensory Nerve ◽  
Sensory Nerve Action Potential ◽  
Nerve Action ◽  
Sensory Nerve Action ◽  
Nerve Root Entrapment

scholarly journals Severe invasive Listeria monocytogenes rhombencephalitis mimicking facial neuritis in a healthy middle-aged man: a case report and literature review

2021 ◽  
Vol 49 (1) ◽  
pp. 030006052098265 ◽  
Author(s):  
Liming Cao ◽  
Yanwei Lin ◽  
Hongliang Jiang ◽  
Jiehong Wei
Keyword(s):  
Listeria Monocytogenes ◽  
White Blood Cells ◽  
Brain Magnetic Resonance Imaging ◽  
Antibiotic Administration ◽  
Stiff Neck ◽  
Atypical Symptoms ◽  
History Of ◽  
The Central Nervous System ◽  
Foodborne Infection ◽  
Immunocompetent Adults

Neurolisteriosis is a foodborne infection of the central nervous system that is easily misdiagnosed, especially in healthy adults with atypical symptoms. A 50-year-old man presented with a 3-day history of distortion of the oral commissure. Facial neuritis was diagnosed and treated with intravenous dexamethasone. His condition deteriorated rapidly, and he presented with a slow pharyngeal reflex, stiff neck, and signs of peripheral facial paralysis. Brain magnetic resonance imaging revealed multiple ring-enhanced foci in the brainstem. Routine and biochemical cerebrospinal fluid (CSF) analyses showed increased white blood cells and microproteins. Blood culture and high-throughput genome sequencing revealed Listeria monocytogenes DNA in the CSF. Ampicillin, amikacin, and meropenem were administered, and the patient was transferred from the intensive care unit to a standard medical ward after 2 months. The patient could walk and eat normally; however, he required intermittent mechanical ventilation at 11 months after discharge. Although L. monocytogenes meningitis is rare in healthy immunocompetent adults, it must be considered as a differential diagnosis, especially in adults whose conditions do not improve with cephalosporin antibiotic administration. L. monocytogenes rhombencephalitis mimics facial neuritis and develops quickly. Prompt diagnosis is essential for rapid initiation of antibiotic therapy to achieve the best outcome.

scholarly journals Carpal tunnel syndrome: long-term nerve conduction studies in 261 hands

2009 ◽  
Vol 67 (1) ◽  
pp. 69-73 ◽  
Author(s):  
Dante Guilherme Velasco Hardoim ◽  
Guilherme Bueno de Oliveira ◽  
João Aris Kouyoumdjian
Keyword(s):  
Carpal Tunnel Syndrome ◽  
Carpal Tunnel ◽  
Nerve Conduction ◽  
Sensory Nerve ◽  
Nerve Conduction Studies ◽  
Sensory Nerve Action ◽  
Tunnel Syndrome ◽  
The Mean ◽  
Compound Action

OBJECTIVE: To compare a long-term carpal tunnel syndrome (CTS) on nerve conduction studies (NCS) in hands treated non-surgically. METHOD: We retrospectively selected 261 symptomatic CTS hands (166 patients), all of them confirmed by NCS. In all cases, at least 2 NCS were performed in an interval greater than 12 months. Cases with associated polyneuropathy were excluded. NCS parameters for CTS electrodiagnosis included a sensory conduction velocity (SCV) <46.6 m/s (wrist to index finger, 14 cm) and distal motor latency (DML) >4.25 ms (wrist to APB, 8 cm). RESULTS: 92.8% were women; mean age was 49 years (20-76); the mean interval between NCS was 47 months (12-150). In the first exam, the median sensory nerve action potential (SNAP) and the compound action muscular potential were absent in 9.8% and 1.9%, respectively. In the second/last exam, SCV worsened in 54.2%, remained unchanged in 11.6% and improved in 34.2%. SNAP amplitude worsened in 57.7%, remained unchanged in 13.1% and improved in 29.2%. DML worsened in 52.9%, remained unchanged in 7.6% and improved in 39.5%. Overall, NCS parameters worsened in 54.9%, improved in 34.3% and remained unchanged in 10.8%. CONCLUSION: Long-term changing in NCS of CTS hands apparently were not related to clinical symptomatology and could lead to some difficulty in clinical correlation and prognosis. Aging, male gender and absent SNAP were more related to NCS worsening, regardless the mean interval time between the NCS.

Close relationship between cardiovagal function and sural sensory nerve action potential in type 2 diabetes

2019 ◽  
Vol 130 (7) ◽  
pp. 1160-1165
Author(s):  
Yun-Ru Lai ◽  
Chih-Cheng Huang ◽  
Wen-Chan Chiu ◽  
Rue-Tsuan Liu ◽  
Nai-Wen Tsai ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Action Potential ◽  
Sensory Nerve ◽  
Sensory Nerve Action Potential ◽  
Nerve Action ◽  
Close Relationship ◽  
Sensory Nerve Action

scholarly journals Chondrosternal Arthritis in Infant: An Unusual Entity

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Athina Nikolarakou ◽  
Dana Dumitriu ◽  
Pierre-Louis Docquier
Keyword(s):  
Clinical Symptoms ◽  
Therapeutic Option ◽  
White Blood Cells ◽  
Laboratory Data ◽  
C Reactive Protein ◽  
Reactive Protein ◽  
History Of ◽  
Local Tenderness ◽  
Wait And See

Primary arthritis of chondrosternal joint is very rare and occurs in infants less than 18 months of age. Presentation is most often subacute but may be acute. Child presents with a parasternal mass with history of fever and/or local signs of infection. Clinical symptoms vary from a painless noninflammatory to a painful mass with local tenderness and swelling, while fever may be absent. Laboratory data show low or marginally raised levels of white blood cells and C-reactive protein, reflecting, respectively, the subacute or acute character of the infection. It is a self-limiting affection due to the adequate immune response of the patient. Evolution is generally good without antibiotherapy with a progressive spontaneous healing. A wait-and-see approach with close follow-up in the first weeks is the best therapeutic option.

Extradural Thoracic Arteriovenous Malformation in a Patient with Klippel-Trenaunay-Weber Syndrome: Case Report

Neurosurgery ◽  
2002 ◽  
Vol 51 (5) ◽  
pp. 1275-1279 ◽  
Author(s):  
Michael J. Alexander ◽  
Peter M. Grossi ◽  
Robert F. Spetzler ◽  
Cameron G. McDougall
Keyword(s):  
Spinal Cord ◽  
Cord Compression ◽  
Magnetic Resonance Imaging Scan ◽  
Lower Extremity Weakness ◽  
Thyrocervical Trunk ◽  
Weber Syndrome ◽  
History Of ◽  
Cutaneous Hemangioma ◽  
The Right ◽  
Upper Thoracic

Abstract OBJECTIVE AND IMPORTANCE Spinal cord involvement in Klippel-Trenaunay-Weber (KTW) syndrome is rare. Cases of intradural spinal cord arteriovenous malformations (AVMs) have been associated with this syndrome. Likewise, cases of epidural hemangioma and angiomyolipoma have been reported to occur at the same segmental level as cutaneous hemangioma in KTW syndrome. This report details a rare case of an extradural thoracic AVM in a patient with KTW syndrome. CLINICAL PRESENTATION A 30-year-old man presented with a 10-month history of progressive myelopathy, bilateral lower-extremity weakness, and numbness, with the right side affected more than the left. His symptoms had progressed to the point that he was unable to walk. The patient had the characteristic manifestations of KTW syndrome, including numerous cutaneous angiomas and cavernomas, limb hypertrophy and syndactyly, and limb venous malformations. A magnetic resonance imaging scan and subsequent angiogram demonstrated a large extradural AVM causing cord compression at the T3–T4 levels. INTERVENTION The patient underwent two separate endovascular procedures, including embolization of upper thoracic and thyrocervical trunk feeders. Subsequently, he underwent T1–T4 laminectomy and microsurgical excision of the AVM. Clinically, the patient improved such that he could walk without assistance. CONCLUSION KTW syndrome represents a spectrum of clinical presentations. Although involvement of the spinal cord is uncommon, the manifestations of this syndrome may include both intradural and extradural AVMs in addition to various tumors.

scholarly journals Susac's Syndrome

2011 ◽  
Vol 38 (2) ◽  
pp. 335-337 ◽  
Author(s):  
Kevin Lian ◽  
Rekha Siripurapu ◽  
Robert Yeung ◽  
Julia Hopyan ◽  
Kenneth T. Eng ◽  
...  
Keyword(s):  
Corpus Callosum ◽  
White Matter Hyperintensities ◽  
Brain Biopsy ◽  
High Dose ◽  
Normal Brain ◽  
Left Hand ◽  
Elevated Protein ◽  
Fluid Attenuated Inversion Recovery ◽  
History Of ◽  
Previous Medical History

A 40-year-old woman with no significant previous medical history presented with a three month history of ataxia, confusion, memory difficulties, and headaches. Physical examination revealed numbness in the left hand, but was otherwise unremarkable. Magnetic resonance imaging fluid-attenuated inversion recovery (MRI FLAIR) images demonstrated multiple small white matter hyperintensities, including lesions involving the corpus callosum. There were also deep gray nuclei lesions (Figure 1). The corpus callosum lesions involved the central fibers (Figure 2). Post gadolinium T1 images demonstrated enhancement of some of the lesions as well as extensive perivascular and leptomeningeal enhancement (Figure 3). Extensive infectious serology, autoimmune panel, and paraneoplastic antibodies were negative. Lumbar puncture revealed elevated protein (1116 mg/L), but was otherwise normal. Brain biopsy indicated no apparent pathology. The patient was tentatively diagnosed with acute encephalopathy and treated with high dose steroids seven days after presentation. She was subsequently discharged and was sent for rehabilitation.

scholarly journals Reversible Nerve Damage and Corneal Pathology in Murine Herpes Simplex Stromal Keratitis

2014 ◽  
Vol 88 (14) ◽  
pp. 7870-7880 ◽  
Author(s):  
Hongmin Yun ◽  
Alexander M. Rowe ◽  
Kira L. Lathrop ◽  
Stephen A. K. Harvey ◽  
Robert L. Hendricks
Keyword(s):  
T Cells ◽  
Herpes Simplex ◽  
Mouse Model ◽  
Human Disease ◽  
Sensory Nerve ◽  
Blink Reflex ◽  
Nerve Damage ◽  
Corneal Scarring ◽  
Corneal Nerves ◽  
Stromal Keratitis

ABSTRACTHerpes simplex virus type 1 (HSV-1) shedding from sensory neurons can trigger recurrent bouts of herpes stromal keratitis (HSK), an inflammatory response that leads to progressive corneal scarring and blindness. A mouse model of HSK is often used to delineate immunopathogenic mechanisms and bears many of the characteristics of human disease, but it tends to be more chronic and severe than human HSK. Loss of blink reflex (BR) in human HSK is common and due to a dramatic retraction of corneal sensory nerve termini in the epithelium and the nerve plexus at the epithelial/stromal interface. However, the relationship between loss of BR due to nerve damage and corneal pathology associated with HSK remains largely unexplored. Here, we show a similar retraction of corneal nerves in mice with HSK. Indeed, we show that much of the HSK-associated corneal inflammation in mice is actually attributable to damage to the corneal nerves and accompanying loss of BR and can be prevented or ameliorated by tarsorrhaphy (suturing eyelids closed), a clinical procedure commonly used to prevent corneal exposure and desiccation. In addition, we show that HSK-associated nerve retraction, loss of BR, and severe pathology all are reversible and regulated by CD4+T cells. Thus, defining immunopathogenic mechanisms of HSK in the mouse model will necessitate distinguishing mechanisms associated with the immunopathologic response to the virus from those associated with loss of corneal sensation. Based on our findings, investigation of a possible contribution of nerve damage and BR loss to human HSK also appears warranted.IMPORTANCEHSK in humans is a potentially blinding disease characterized by recurrent inflammation and progressive scarring triggered by viral release from corneal nerves. Corneal nerve damage is a known component of HSK, but the causes and consequences of HSK-associated nerve damage remain obscure. We show that desiccation of the corneal surface due to nerve damage and associated loss of BR severely exacerbates and prolongs inflammation-induced pathology in mice. Preventing corneal desiccation results in a milder and more transient HSK with variable scarring that mirrors HSK seen in most humans. We further show that nerve damage is reversible and regulated by CD4+T cells. Thus, we provide a mouse model that more closely resembles typical human HSK and suggest nerve damage is an important but largely overlooked factor in human disease.

scholarly journals Congenital Toxoplasmosis presenting as multiple ring enhancing lesions in brain on neuroimaging

2018 ◽  
Vol 5 (5) ◽  
pp. 2020
Author(s):  
Pareshkumar A. Thakkar ◽  
Aakashi Shah ◽  
Bakul B. Javadekar ◽  
Saurabh Modasia
Keyword(s):  
Ct Scan ◽  
Past History ◽  
Stable Condition ◽  
Congenital Toxoplasmosis ◽  
Cellular Reaction ◽  
Protein Levels ◽  
Elevated Protein ◽  
Intravenous Antibiotics ◽  
History Of ◽  
Multiple Ring

We report a case of congenital toxoplasmosis in a male child who presented at 1 month 21 days of life. He had a past history of meningitis on third day of life which resolved with intravenous antibiotics. This time, the child presented with progressive enlargement of head and CSF (Cerebrospinal fluid) showed elevated protein levels with cellular reaction and CT scan showed dilated ventricular system with aqueductal obstruction and multiple ring-enhancing lesions in bifrontal and bioccipital regions with a right porencephalic cyst. Repeat CSF after 14 days of intravenous antibiotics showed elevated protein with no cellular reaction. Fundus examination was normal. Toxoplasma blood serology (IgG and IgM) in baby and mother were positive. The child received treatment with Pyrimethamine, Clindamycin, Folinic acid and Prednisolone. CSF parameters got normalized and multiple ring-enhancing lesions disappeared on repeat CT scan. He underwent a Ventriculo-Peritoneal shunt surgery for the hydrocephalus and was discharged in a stable condition.

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