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Monoclonal gammopathy of renal significance encompasses renal disease in the nonlight chain disease manner in the presence of circulating monoclonal antibodies, likely without ongoing bone marrow proliferation. It has an acute course, variable presentation and needs to followed up on.

AMA Research Challenge 2024 

Monoclonal gammopathy of renal significance

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Background

Amoxicillin-clavulanate can cause both liver injury and acute kidney injury. The
majority of the existing literature has described liver-related injury, with fewer
reports noting kidney injury. In this report, we describe a rare case of
concomitant drug-induced liver injury and acute interstitial nephritis as a result
of amoxicillin-clavulanate exposure.

Case Presentation

A 62-year-old male presented to the emergency department with weight loss,
nausea, and diarrhea. Two weeks prior to presentation, he was treated for
sinusitis with a ten-day course of amoxicillin-clavulanate (875 mg-125 mg)
twice daily. The patient was jaundiced, and his abdominal examination
revealed mild distension. Labs included a blood urea nitrogen of 98, creatinine
of 6.6 mg/dL, total bilirubin of 20.5, alkaline phosphatase of 397, aspartate
transaminase of 138, alanine transaminase of 88, and International Normalized
Ratio of 11.4 with pro-time of 127.9. A complete blood count was notable for
9.5% eosinophils, with a total level of 700 eosinophils. A computerized
tomography scan of the abdomen was performed, which revealed gallbladder
wall thickening; subsequent right upper quadrant ultrasonography showed a
minimally distended gallbladder and focally dilated intrahepatic ducts in the
left lobe of the liver. Given concern for renal failure, the patient was admitted
to the ICU. He was started on high-dose methylprednisolone for empiric
treatment of suspected autoimmune hepatitis. Given that drug-induced liver
injury was on the differential, tissue biopsies were obtained. A percutaneous
core kidney biopsy revealed acute tubular injury with bile cast nephropathy,
focal acute tubulointerstitial nephritis, moderate arteriolar hyalinosis, and mild
arteriosclerosis. The liver biopsy showed bile pigment within hepatocytes and
bile canaliculi in peri-central vein areas. These biopsy findings were suggestive
of amoxicillin-induced renal and hepatic injury. The patient was continued on
high-dose IV steroids (which he received for a total of five days) that were
transitioned to an eighteen-day oral prednisone taper. His creatinine gradually
normalized, and the patient’s LFTs remained mildly elevated on the day of
discharge.

Discussion 

This case study demonstrates a rare occurrence in which a patient developed drug-induced liver injury and renal failure. Though antibiotics are widely used to treat infection, their use is not without consequences. In our case, the pathology revealed findings consistent with the immuno-allergic response in amoxicillin-clavulanate drug-induced injury. As this relays the etiology behind the multiorgan failure, tissue biopsy is an essential component in guiding the treatment of patients afflicted with concomitant hepatic failure and renal failure caused by amoxicillin-clavulanate.

Utility of Tissue Biopsy in Amoxicillin-Clavulanate Induced Hepatic Failure and Renal Failure

Single-energy computed tomography (SECT) head is a common diagnostic tool to evaluate for intracranial hemorrhage in emergency settings due to its widespread accessibility and non-invasive nature. However, SECT has densitometric evaluation limitations. For example, hyperdensities on SECT such as blood product and iodine contrast appear similarly. Dual-energy CT (DECT) is a relatively under-utilized imaging modality that has the capability to differentiate between multiple materials. This imaging technique can be extremely useful in identifying materials that are otherwise indistinguishable from standard SECT.

The authors present a case of a patient with findings suspicious of intraventricular and subarachnoid hemorrhage on conventional SECT. The suspected hemorrhage was subsequently ruled out utilizing DECT, as iodinated contrast can be subtracted out, yielding an image that can differentiate iodine contrast from blood or other hyperdense material. The authors discuss the underlying physics, potential advantages, and limitations of the DECT.


Utility of Dual Energy CT Head to Differentiate Myelography Contrast from Intraventricular and Subarachnoid Hemorrhage

Background
Guillain-Barré Syndrome (GBS) is a rare autoimmune disorder of the peripheral nervous system, classically characterized by symmetric, ascending muscle weakness that can progress to life-threatening respiratory depression. Diminished sensation, paresthesia, and autonomic dysfunction may also occur. GBS is a dysregulated immune response triggered by infections.  

Case Presentation
A 15-year-old male with history of depression and substance use presented to the emergency room with one week of bilateral leg weakness, dyspnea, and migratory joint pains. One month prior, he had an upper respiratory infection which resolved spontaneously. Patient was travelling when he developed gait instability, dysarthria, photophobia, and shortness of breath. CT Head was normal. His respiratory function worsened, requiring escalating oxygen requirements, followed by acute deterioration of mental status. He was sedated, put on ventilatory support, and transferred to our critical care unit.  

Neurological exam revealed hypotonia, present reflexes, and poor muscle coordination out of proportion to muscle weakness, which initially suggested functional etiology. MRI and MRA brain and spine were unremarkable. Cerebral spinal fluid studies revealed albuminocytologic dissociation and elevated inflammatory markers (IL-6, IL-8). Potential autoimmune, paraneoplastic, rheumatologic, demyelinating, infectious, and toxic causes were ruled out. After unsuccessful extubation attempts, serial examination revealed involvement of cranial nerves VII, IX, and X. 

He received a three-day course of intravenous immunoglobulin (2g daily) and gabapentin. Major complications included hospital acquired pneumonia and decompensated psychiatric state (worsening mood, panic attacks). Over three weeks, his mental status normalized, and he regained significant muscle strength and coordination. After confirming regained diaphragmatic function on ultrasound, he was extubated and weaned to room air. The patient was then transferred in stable condition to an acute rehabilitation center. 

Discussion
In pediatric patients, clinicians should conceptualize GBS as a spectrum of neurological symptoms that can differ from the typical ascending pattern but remain responsive to immunoglobulin treatment. This case proved unique because our patient’s history aligned with classic GBS, but he did not have expected radiological findings and patchy, rather than length-dependent, motor and sensory symptoms. His presentation had mixed features of multiple GBS subtypes, including Acute Motor Axonal Neuropathy and Multifocal Acquired Demyelinating Sensory and Motor Neuropathy. In addition to diaphragmatic weakness, patients can develop cranial neuropathies that prolong need for mechanical ventilation and increase risk of pneumonia. Lastly, in adolescents with prior psychiatric history, GBS can acutely exacerbate mood and anxiety symptoms which may be falsely attributed to functional neurological disorder.

An Eccentric Case of Guillain-Barré Syndrome in an Adolescent Male

An acute dilated pupil signals dysfunction of the third cranial nerve or sympathetic/parasympathetic pupillary pathways. Life-threatening etiologies of third nerve cranial impairment include uncal herniation, cerebral aneurysm, or another space-occupying lesion. Consequently, the finding of an acute, unilateral, dilated pupil warrants immediate clinical evaluation and neuroimaging.
However, anisocoria without findings of oculomotor deficits is rare in compression-induced third cranial nerve palsies but is rather commonly associated with pharmacological etiologies or Adie’s pupil. Specifically, anticholinergics allow unopposed sympathetic stimulation of the dilator pupillae muscle, resulting in a dilated pupil. Administration of ipratropium through a face mask inhaler is common in intensive care respiratory management. However, masks are susceptible to leakage and displacement. Pupillary dilatation secondary to ipratropium has been described but is not widely known. We presented a case report where an unilateral anisocoria is secondary to contamination of ipratropium to the right eye.

Ipratropium-Induced Anisocoria due to an Ill-Fitting Face Mask Inhaler

Abstract: 

Background: 

Myelitis is a debilitating neurological condition characterized by inflammation and demyelination of the spinal cord. It leaves two-thirds of patients with moderate to severe residual disability. Generally occurring independently, often as a complication of infection, it can also be a component of neuro-inflammatory spectrum disorders like multiple sclerosis, neuromyelitis Optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis, and MOG antibody disease. NMOSD is distinct by the presence of aquaporin-4 IgG antibodies (AQP4-IgG) and has an estimated global prevalence of 1 in a million, predominantly affecting females. 

Case presentation: 

A 59-year-old female with a questionable history of Brown-Séquard syndrome in 2004, for which she avoided surgery due to concerns about losing mobility, presented with acute neck pain following a bug bite on the back of her neck, with pain and tingling sensation in both the hands. An MRI of the neck revealed T2-weighted hyperintense signals from the cervico medullary junction to the upper border of T1, indicating spinal cord edema(Figure 1). Blood work for paraneoplastic syndrome(s) was negative but the presence of AQP-4 antibodies confirmed the diagnosis of NMOSD. She was treated with a 5-day course of high-dose intravenous methylprednisolone and plasma-exchange, leading to significant symptom improvement. She was placed on rituximab monotherapy (1000 mg once every 6 months) for chronic treatment. 
case.png

Discussion: 

NMOSD is definitively diagnosed based on AQP-4 antibodies in serum and CSF with signs of area postrema syndrome and acute myelitis (2015 diagnostic criteria). Moreover, this patient's symptoms improved following immunotherapy, but not anti-infective medication. Intravenous methylprednisolone is typically used to treat acute NMOSD episodes. If unresponsive, plasma exchange can be added. Acute attack outcomes have improved in single case series by experimental therapies like IVIG, anti-CD20, and anti-complement therapy. 

Relapses have reduced with immunosuppressive maintenance therapy using rituximab, azathioprine, mycophenolate mofetil, and oral prednisone. Shi Z et al., with 281 Chinese NMOSD patients, showed azathioprine and mycophenolate mofetil lowered disability and relapse rates. Yaguchi H et al. found cyclophosphamide (monotherapy or in combination with methylprednisolone) effective in both acute and chronic phases. Approved immunotherapies, inebilizumab, eculizumab, and Satralizumab, reduce the relapses and the cumulative impairment. Newer biologics (theravulizumab, ublituximab, aquaporumab, and sedelestat) are undergoing review for use in NMOSD. 

Conclusion: 
For atypical presentation of NMOSD, a high index of suspicion is needed for early treatment and better patient outcomes. Although novel treatment modalities provide a hope for more effective treatment, more trials are required to estimate their safety and efficacy.

Neuromyelitis Optica Spectrum Disorder: A case report and a review of treatment modalities

Background 
Immediate reperfusion is vital to treating an acute ischemic stroke (AIS). Tenecteplase can be used for thrombolysis. However, there is a chance of early recurrence of ischemia which can affect the high cervical spinal cord (HCSC) which often gives a bad prognosis but with appropriate management, a good clinical outcome can still be expected. 

Case Presentation 
A 69-year-old female with no known comorbidities suffered an HCSC infarct after treatment with tenecteplase for an acute ischemic stroke. 

She presented with sudden pain in the right neck with nausea, diaphoresis, and dizziness. Neurologic examination was significant only for a left facial droop. CT angiogram showed an occlusion of the right vertebral artery at V3-V4 segment. She was given IV Tenecteplase within 4.5 hours of onset. 

Few hours later, she developed difficulty breathing. She had bilateral diaphragmatic paralysis resulting in respiratory acidosis requiring mechanical ventilation. Additionally, she developed quadriplegia. MRI brain showed an HCSC infarction. She was on ventilatory support for 2 months due to diaphragmatic compromise. She underwent a tracheostomy and percutaneous endoscopic gastrostomy. Multiple bronchoscopies were done to manage mucous plugs and retained secretions, and she received antibiotics for ventilator-associated pneumonia. 

Despite all complications, she was weaned to a speaking valve and eventually was able to communicate. Modified barium swallow showed tolerance to oral intake. Tracheostomy was successfully decannulated thereafter. 

She was eventually discharged with a motor exam of 2-3/5 in all extremities with no other focal neurologic deficits. 

Discussion 
The patient suffered from a mild ischemic stroke. Alteplase is the only approved thrombolytic drug by the Food and Drug Administration (FDA) for the treatment of AIS. If given within 3-4.5 hours of onset, it can prevent further disability. 

Coming in during the therapeutic window for thrombolysis, she was given tenecteplase, a modified variant of alteplase. Tenecteplase is FDA-approved for thrombolysis in acute myocardial infarction but has found off-label use for AIS. 

Early recurrence of ischemic stroke has been reported after alteplase. For tenecteplase, there are instances of expansion of the original infarct after thrombolysis but no documented report of an HCSC infarct as of this writing. Nevertheless, due to the similar pharmacokinetic properties of tenecteplase to alteplase, such a phenomenon of ischemic stroke recurrence may occur. 

Acute spinal cord infarct is a rare occurrence and usually involves non-cervical territory. It may be associated with episodes of hypotension and may present with quadriplegia and diaphragmatic paralysis which were all found in this patient. 


Risks of Thrombolytic Therapy: Uncommon Complications with A Good Outcome

Miller Fisher Syndrome (MFS) is an uncommon neurological disorder with an estimated yearly incidence of 1 in 1,000,000 in the United States. MFS is a variant of Guillain-Barre syndrome (GBS) characterized by ophthalmoplegia, ataxia, and areflexia, likely preceded by either a recent infection of the upper respiratory or gastrointestinal tracts, or vaccination. This syndrome most commonly affects adult males with a median age of 44 years. Here we want to draw attention to a rare case of MFS with atypical presentation in a 60-year-old female patient who presented to the Emergency Department (ED) with a medical history of hypertension and diabetes mellitus with complaints of blurred vision and walking difficulty for 2 days. She developed respiratory weakness requiring Intensive Care Unit (ICU) admission, and hyporeflexia, bilateral ptosis, and ophthalmoplegia subsequently. Provisional diagnosis of MFS was made upon cerebrospinal fluid (CSF) analysis which was later confirmed by the presence of Anti-GQ1b antibody. A single dose of Intravenous Immunoglobulin (IVIG) was sufficient to stabilize the patient’s condition, allowing her to be moved out of the ICU. Given the favorable prognosis of this disorder, early identification and effective drug treatment would minimize unnecessary medical interventions, curtail expenditures, and ease psychological distress.

Unusual Manifestation of Miller Fisher Syndrome as a Stroke-Mimic: A Case Report and Literature Review

Abstract Title
Unveiling the Unusual: Adrenoleukodystrophy and Paraphilia - A Rare Case Study
Background 
X-linked adrenoleukodystrophy (ALD) is a rare genetic disorder affecting approximately 1 in 21,000 males, primarily impacting the white matter of the nervous system and the adrenal cortex. The cerebral forms of ALD, including childhood, adolescent, and adult variants, typically present initially with symptoms such as hyperactivity or inattention, followed by a progressive decline in cognitive, motor, visual, and auditory functions. Here, we present the case of a 22-
year-old male with ALD, who exhibits paraphilia along with neuropsychiatric manifestations of ALD, a unique presentation that has been scarcely documented.
Case Presentation 
A 22-year-old male with a medical history significant for adrenoleukodystrophy, seizure disorder, Asperger syndrome, Attention-Deficit/Hyperactivity Disorder, progressive hearing and visual loss was admitted due to altered mental status. His adoptive mother reported developmental delays and social cue difficulties since childhood, with a decline in functionality beginning in October 2023. Neuro-ophthalmological evaluation for progressive vision loss, led to a subsequent MRI which showed diffuse periventricular and subcortical white matter hyperintensities involving parietal, occipital, temporal, and frontal regions. Elevated very long chain fatty acids (VLCFA) confirmed ALD diagnosis. A month after admission, he developed compulsive rectal picking and digital manipulation leading to rectal bleeding. Psychiatry suggested that it could be due to either OCD-type skin picking or a gratifying sexual compulsion. The nursing staff reported frequent episodes of uncontrollable impulses for aggressive rectal digital manipulation ending in ejaculation, over the past three months, necessitating the use of restraints and mittens for his safety. Despite treatments including Paroxetine, risperidone, and 
anti-androgens, no improvement was noted. Guanfacine was later initiated to enhance frontal inhibition, yet efficacy remained limited. Upon discharge, transitioning to a group home was planned.
Discussion
ALD is a disorder in which defective beta-oxidation leads to the accumulation of very long-chain fatty acids in peroxisomes. It typically presents with neurobehavioral disturbances starting in childhood. The patient's behavioral symptoms began in childhood but were never formally evaluated. We believe an undiagnosed ALD might have contributed to his symptoms. ALD usually leads to hyposexuality due to adrenal insufficiency. However, our patient exhibited recurrent rectal digital stimulation suggesting impulsivity and disinhibition, which is a distinctive presentation. We hypothesize that the patient's paraphilia may be linked to his frontal lobe 
involvement. No evidence of adrenal insufficiency was observed in his labs. We emphasize the importance of considering ALD as a differential diagnosis when evaluating patients with neuropsychiatric disturbances, as it is a progressive and debilitating condition

Unveiling the Unusual: Adrenoleukodystrophy and Paraphilia - A Rare Case Study

Background: Cases of incarcerated gravid uterus (IGU) are quite rare at about 1 in 3000 pregnancies. IGU is defined as the entrapment of the gravid uterus between the pubic symphysis and the sacral promontory. Due to compression of adjacent structures, symptoms of IGU present non-specifically as urinary retention, dysuria, abdominal pain, and constipation, often causing this condition to be misdiagnosed. Reduction of an IGU proves to be crucial as serious complications such as urinary tract obstructions, urinary tract infections, renal failure, fetal growth restriction, fetal demise, and uterine wall rupture may occur. 

Case Presentation: A 34-year-old G2P1 woman at 12 3/7 weeks presented with difficulty urinating, difficulty stooling, and a feeling of incomplete emptying. At the patient’s first visit a week prior, it was thought that she had a urinary tract infection as it proved to be the most likely cause of her symptoms; however, cultures were negative and completion of the antibiotic treatment provided no relief. The patient was reevaluated the following week when an anterior displacement of the cervix on speculum exam was noted. This led to the diagnosis of an IGU, pivoting the management to passive and active reduction techniques. A catheter was placed to relieve the patient’s bladder. 2200 cc of urine were drained after which the patient reported significant relief of pelvic pressure. The patient was placed in the knee/chest position for two hours before a manual replacement was attempted. The uterus was lifted from the cul-de-sac, and the patient was sent home with the foley in case the incarceration recurred. She returned to the clinic 10 days later for a voiding trial after removal of the foley which proved to be successful. The patient is continuing with her pregnancy without any issues.

Discussion: IGU often presents with urinary symptoms and can be conservatively managed by draining of the bladder, non-invasive methods such as knee-chest, and manual reduction if none of these efforts are successful. Early identification and management of an IGU is crucial for a safe pregnancy. Due to its rarity, it is often not considered when patients present with non-specific or primarily urinary symptoms. Cases are often misdiagnosed, leading to a delay in treatment and resolution of symptoms for patients. In this case, we note that the presentation of an IGU can easily be attributed to other causes and that if identified properly, can be reduced earlier in the pregnancy to avoid serious complications.

A Case of an Initially Misdiagnosed Incarcerated Gravid Uterus (IGU)

Current research emphasizes that ectopic pregnancies (EPs) are quite rare, with roughly 1-2% incidence in typical pregnancies and 2-5% in pregnancies resulting from Assisted Reproductive Technology. Non-tubal EPs (NTEPs) are significantly less common with an incidence of <10% out of all ectopic pregnancies. This study includes seven patients evaluated for NTEP treatment from January 2017 to August 2023. The team assessed risk factors while conducting an in-depth analysis of lab values, ultrasounds, complications, demographics, and outcomes. Analysis shows that of the 7 NTEP patients, 1 patient had a low-implantation intrauterine pregnancy (IUP), 5 patients had cesarean ectopic pregnancies, and 1 patient had an upper cervical canal ectopic pregnancy. 5 of the NTEP patients required in-patient treatment due to complications. Treatment options included: Transvaginal US-guided MTX injection, dilatation and curettage (D&C), systemic methotrexate (MTX), uterine artery embolization (UAE), and manual vacuum aspiration (MVA). The limited data pool for this condition and lack of consensus on best treatment for NTEP indicates the need for further research, especially less invasive options for patients who desire fertility preservation. By demonstrating that this approach is applicable in a variety of treatment settings and NTEP patient scenarios, our study verifies consistency with prior research studies on transvaginal MTX injection for NTEP management.

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Utility of Tissue Biopsy in Amoxicillin-Clavulanate Induced Hepatic Failure and Renal Failure

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