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The Neurohospitalist Jan 2021Pseudotumor cerebri syndrome (PTCS), also known as idiopathic intracranial hypertension (IIH), is defined by elevated intracranial pressure in the absence of a...
Pseudotumor cerebri syndrome (PTCS), also known as idiopathic intracranial hypertension (IIH), is defined by elevated intracranial pressure in the absence of a structural or cerebrospinal fluid (CSF) abnormality. Typical features include headache, visual symptoms, and pulsatile tinnitus. Cranial nerve deficits are seen in a minority of cases, most often sixth nerve palsies. We present a unique cause of PTCS complicated by diffuse ophthalmoparesis and polyradiculopathy. A 27-year-old healthy woman presented with 2 weeks of blurry vision, diplopia, and facial and arm weakness. On examination, she had decreased visual acuity, markedly constricted visual fields in both eyes, with severe bilateral optic disc edema on fundus examination. There was diffuse ophthalmoparesis, right upper and lower facial weakness, and bilateral arm weakness. Magnetic resonance imaging brain revealed optic disc protrusion and a partially empty sella but no other abnormalities. Electromyogram (EMG) was consistent with bilateral C5-6 radiculopathies. Lumbar puncture revealed an opening pressure of 56 cm H2O with otherwise normal CSF constituents. She was treated with high-dose acetazolamide and methylprednisolone followed by optic nerve sheath fenestration. Due to progressive vision loss, she ultimately required ventriculoperitoneal shunting, after which her papilledema, ophthalmoparesis, and facial and arm weakness rapidly improved. This is a unique case of PTCS associated with diffuse ophthalmoparesis and polyradiculopathy. This constellation of abnormalities usually suggests an underlying inflammatory process in the subarachnoid space. However, once this has been excluded, the possibility of PTCS should be considered as early treatment can result in rapid reversal of symptoms and preserve visual function.
PubMed: 33868562
DOI: 10.1177/1941874420945565 -
The American Journal of Case Reports Nov 2015Cytomegalovirus (CMV) infection has been well described as an opportunistic infection of patients with human immunodeficiency virus (HIV). To the best of our knowledge,... (Review)
Review
BACKGROUND
Cytomegalovirus (CMV) infection has been well described as an opportunistic infection of patients with human immunodeficiency virus (HIV). To the best of our knowledge, this is the first case report of a patient with AIDS and lumbosacral polyradiculopathy, associated with gastroparesis resulting from CMV infection.
CASE REPORT
A 46-year-old Hispanic woman with a history of HIV for 10 years was admitted to our hospital for nausea, vomiting, urinary retention, and generalized weakness. Bilateral lower extremity examination revealed flaccid paraplegia, decreased sensations from the groin downwards, bilateral lower extremity areflexia, and absent plantar reflexes, with enlarged urinary bladder. CMV was detected in CSF by PCR, and cervical and lumbar magnetic resonance imaging (MRI) revealed intense nodular leptomeningeal enhancement from the lower thoracic cord and extending along the conus medullaris/filum terminalis and nerve roots. Gastric emptying scintigraphy revealed severe delayed gastric emptying time. Ganciclovir was initiated and her neurological symptoms and gastrological symptoms gradually improved. Over 8 weeks, nausea and vomiting resolved and the patient was able to walk before being discharged from the hospital.
CONCLUSIONS
Polyradiculopathy and gastroparesis can result from CMV infection in AIDS patients. Whether the mechanism is secondary to viral infection or immune systems remains unclear. It is important for physicians to be aware of this uncommon presentation in the antiretroviral therapy (ART) era. CMV treatment should be initiated immediately once diagnosis is confirmed.
Topics: Acquired Immunodeficiency Syndrome; Cytomegalovirus; Cytomegalovirus Infections; DNA, Viral; Diagnosis, Differential; Female; Gastroparesis; Humans; Magnetic Resonance Imaging; Middle Aged; Polyradiculopathy; Tomography, X-Ray Computed
PubMed: 26552851
DOI: 10.12659/ajcr.894512 -
Neurology Apr 2021Sensory loss with normal nerve conduction studies (NCS) from focal sensory root inflammatory demyelination is characteristic of chronic immune sensory polyradiculopathy...
OBJECTIVE
Sensory loss with normal nerve conduction studies (NCS) from focal sensory root inflammatory demyelination is characteristic of chronic immune sensory polyradiculopathy (CISP). However, nonpure cases involving motor and distal sensory nerves exist (CISP-plus). We hypothesize that CISP-plus and CISP are fundamentally part of the same syndrome through comparison of clinical, neurophysiologic, and pathologic features.
METHODS
CISP-plus (primary dorsal root with lesser motor and sensory nerve involvement) and CISP cases were retrospectively analyzed (1986-2019).
RESULTS
We identified 44 CISP-plus and 28 CISP cases (n = 72) with 86% (38/44) of patients with CISP-plus and 79% (22/28) of patients with CISP experiencing imbalance. On examination, large fiber sensory loss was present in 98% (43/44) of patients with CISP-plus and 96% (27/28) of patients with CISP. Gait ataxia was evident in 93% (41/44) of patients with CISP-plus and 79% (22/28) of patients with CISP. Mild distal weakness was common in CISP-plus (75%, 33/44). NCS showed mild abnormalities in all patients with CISP-plus and were normal (by definition) in all patients with CISP. Elevated CSF protein, slowing of somatosensory evoked potentials, and MRI root enhancement occurred in most CISP-plus and CISP cases. Eleven CISP-plus nerve biopsies showed loss of large myelinated fibers and onion-bulb formations, most prominent in rootlet biopsies. Immunotherapy resulted in marked improvement of gait ataxia in 84% (27/32) of patients with CISP-plus and 93% (13/14) of patients with CISP with return to normal neurologic examination in half (25/46).
CONCLUSION
The recognition of CISP-plus expands the spectrum of CIDP by combining CISP-plus (predominant sensory polyradiculopathy with mild motor and sensory nerve involvement) with pure CISP (focal sensory polyradiculopathy) together as proximal sensory CIDP.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Motor Neurons; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Retrospective Studies; Sensory Receptor Cells; Young Adult
PubMed: 33653905
DOI: 10.1212/WNL.0000000000011792 -
Cureus Sep 2021Idiopathic nutritional deficiencies are often overlooked in patients with no history of malabsorption. However, it may lead to severe neurologic dysfunction that can...
Idiopathic nutritional deficiencies are often overlooked in patients with no history of malabsorption. However, it may lead to severe neurologic dysfunction that can sometimes be irreversible. We present a case in which early recognition of copper deficiency has led to a better outcome for the patient, who presented with acute myeloneuropathy. A 45-year-old male with no significant history of malnutrition or malabsorption presented with complaints of acute encephalopathy, bilateral wrist drop, bilateral tingling and weakness in his hands as well as urinary incontinence. Workup upon arrival was nonrevealing, the patient was treated initially as presumed AIDP (acute inflammatory demyelinating polyradiculopathy), and he underwent plasmapheresis with no response. Since the patient did not respond to plasmapheresis and he had a significantly low folate levels with initial labs. Further nutritional workup was done, which revealed low copper (levels of 0.45), vitamins A, E, and B1. The patient was also tested for celiac which was negative, underwent upper endoscopy and colonoscopy which were both not significant. Decision was made to treat patient early with IV copper infusion as symptoms were deemed most likely due to copper deficiency. The patient received a total of 4 IV doses, after which the patient had a significant clinical response after infusion therapy and repeat copper levels revealed an increase as well (levels of 0.71). Prior to discharge, the patient had significant improvement in wrist drop as well as symptoms of tingling and numbness. Despite being a trace element, copper deficiency can cause significant neurologic impairment. Furthermore, early recognition has proved to be imperative in neurologic recovery and supplementation has proven to be successful in improving patient's quality of life.
PubMed: 34692339
DOI: 10.7759/cureus.18130 -
Ochsner Journal 2020Meningitis caused by is associated with devastating clinical outcomes. A considerable number of patients will develop long-term neurologic complications. Hearing loss,...
Meningitis caused by is associated with devastating clinical outcomes. A considerable number of patients will develop long-term neurologic complications. Hearing loss, diffuse brain edema, and hydrocephalus are frequently encountered. Acute spinal cord dysfunction and polyradiculopathy can develop in some patients. A 63-year-old female was admitted to our hospital with sudden-onset bilateral lower extremity weakness. On admission, the patient had evidence of spinal cord dysfunction given the abnormal motor and sensory physical examination findings and the absent sensation with a sensory level at dermatome T4 on neurologic examination. Computed tomography myelography did not show evidence of spinal cord compression or transverse myelitis. Cerebrospinal fluid examination was positive for pneumococcal meningitis. The patient was treated with antibiotics and steroids. Nerve conduction studies demonstrated the absence of response, suggesting damage to the peripheral nerves and polyradiculopathy. The patient was treated with plasmapheresis for possible Guillain-Barré syndrome; however, she did not improve despite appropriate antibiotics, steroids, and plasmapheresis. She developed persistent quadriparesis, sensory impairments in upper and lower extremities, and bowel and bladder sphincter dysfunction. Our case demonstrates the development of spinal cord dysfunction (supported by the sudden onset of paraplegia and the presence of a sensory level) and polyradiculopathy (flaccid paralysis, ascending weakness, and absence of response in neurophysiologic studies suggesting severe damage to the peripheral nerves). The appearance of either complication is unusual, and the simultaneous occurrence of both complications is even more uncommon.
PubMed: 32612480
DOI: 10.31486/toj.18.0153 -
Muscle & Nerve Dec 2009Neuromuscular disorders are common in human immunodeficiency virus (HIV); they occur at all stages of disease and affect all parts of the peripheral nervous system.... (Review)
Review
Neuromuscular disorders are common in human immunodeficiency virus (HIV); they occur at all stages of disease and affect all parts of the peripheral nervous system. These disorders have diverse etiologies including HIV itself, immune suppression and dysregulation, comorbid illnesses and infections, and side effects of medications. In this article, we review the following HIV-associated conditions: distal symmetric polyneuropathy; inflammatory demyelinating polyneuropathy; mononeuropathy; mononeuropathy multiplex; autonomic neuropathy; progressive polyradiculopathy due to cytomegalovirus; herpes zoster; myopathy; and other, rarer disorders.
Topics: HIV Infections; HIV-1; Humans; Polyneuropathies; Quality of Life
PubMed: 19771594
DOI: 10.1002/mus.21465 -
International Journal of Molecular... Nov 2022The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is based on a combination of clinical, electrodiagnostic and laboratory features. The... (Review)
Review
The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is based on a combination of clinical, electrodiagnostic and laboratory features. The different entities of the disease include chronic immune sensory polyradiculopathy (CISP) and autoimmune nodopathies. It is debatable whether CIDP occurring in the course of other conditions, i.e., monoclonal IgG or IgA gammopathy, should be treated as a separate disease entity from idiopathic CIDP. This study aims to evaluate the molecular differences of the nodes of Ranvier and the initial axon segment (AIS) and juxtaparanode region (JXP) as the potential cause of phenotypic variation of CIDP while also seeking new pathomechanisms since JXP is sequestered behind the paranode and autoantibodies may not access the site easily. The authors initially present the structure of the different parts of the neuron and its functional significance, then discuss the problem of whether damage to the juxtaparanodal region, Schwann cells and axons could cause CIDP or if these damages should be separated as separate disease entities. In particular, AIS's importance for modulating neural excitability and carrying out transport along the axon is highlighted. The disclosure of specific pathomechanisms, including novel target antigens, in the heterogeneous CIDP syndrome is important for diagnosing and treating these patients.
Topics: Humans; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Axons; Autoantibodies; Syndrome
PubMed: 36362407
DOI: 10.3390/ijms232113621 -
The American Journal of Tropical... Jan 2021Spinal sparganosis of the cauda equina has been rarely reported. A 54-year-old man presented at the hospital after having experienced lower back pain for 10 months,... (Review)
Review
Spinal sparganosis of the cauda equina has been rarely reported. A 54-year-old man presented at the hospital after having experienced lower back pain for 10 months, progressive weakness and numbness of the left leg for 4 months, and urinary incontinence for 3 weeks. Magnetic resonance imaging of the lumbosacral spine revealed a heterogeneous enhancing mass at the T12-S1 level. Spinal sparganosis was diagnosed by histological examination and molecular identification of the parasite in the tissue section. The patient was treated with a high dose of praziquantel because the parasitic mass was only partially removed and symptoms worsened following surgery.
Topics: Anthelmintics; Anti-Ulcer Agents; Cauda Equina; Cimetidine; Humans; Male; Middle Aged; Polyradiculopathy; Praziquantel; Sparganosis
PubMed: 33124542
DOI: 10.4269/ajtmh.20-0712 -
Postgraduate Medical Journal Oct 1963
Review
Topics: Bacterial Infections; Bronchitis; Bronchopneumonia; Encephalitis; Heart Diseases; Humans; Influenza, Human; Klebsiella; Neurologic Manifestations; Pneumonia; Pneumonia, Viral; Polyradiculopathy; Staphylococcus; Streptococcus pneumoniae; Streptococcus pyogenes; Tracheitis
PubMed: 14053697
DOI: 10.1136/pgmj.39.456.578 -
The Korean Journal of Pain Oct 2016Muscular hypertrophy is caused mainly due to myopathic disorder. But, it is also rarely produced by neurogenic disorder. A 74-year-old woman complained of right calf...
Muscular hypertrophy is caused mainly due to myopathic disorder. But, it is also rarely produced by neurogenic disorder. A 74-year-old woman complained of right calf pain with hypertrophy for several years. Recent lumbar spine magnetic resonance imaging (MRI) showed central and lateral canal narrowing at the L4-L5 intervertebral space. Lower extremity MRI revealed fatty change of right medial head of the gastrocnemius and soleus, causing right calf hypertrophy. Electrodiagnostic examinations including electromyography and nerve conduction velocity testing demonstrated 5 lumbar and 1 sacral polyradiculopathy. Integrating all the results, the diagnosis was neurogenic muscle hypertrophy. Neurogenic muscle hypertrophy is very rare, but we recommend that clinicians consider this problem when a patient complains of lower limb hypertrophy and pain.
PubMed: 27738507
DOI: 10.3344/kjp.2016.29.4.270