THIS IS AN ONLINE E LOG BOOK TO DISCUSS OUR PATIENT'S DE-IDENTIFIED HEALTH DATA SHARED AFTER TAKING HIS SIGNED INFORMED CONSENT. HERE WE DISCUSS OUR PATIENT'S PROBLEMS THROUGH SERIES OF INPUTS FROM AVAILABLE GLOBAL ONLINE COMMUNITY EXPERTS WITH AN AIM TO SOLVE THOSE PATIENT'S CLINICAL PROBLEMS WITH COLLECTIVE CURRENT BEST EVIDENCE BASED INPUTS.
EMR SUMMARY:
Age/Gender: 70 Years/Male
Address:
Discharge Type: Relieved
Admission Date: 29/08/2025 01:31 PM
Diagnosis
UREMIC ENCEPHALOPATHY
SEPSIS SECONDARY TO LEFT LOWER LIMB CELLULITIS
ACUTE ON CHRONIC CKD
K/C/O HTN SINCE 1 YEAR
Case History and Clinical Findings
CHIEF COMPLAINTS: FEVER SINCE 4 DAYS, NECK PAIN SINCE 4 DAYS, LEFT LOWER LIMB
SWELLING SINCE 3 DAYS
HISTORY OF PRESENTING ILLNESS: PATIENT WAS APPARENTLY ASYMPTOMATIC 3 DAYS
BACK THEN HE DEVELOPED LEFT LOWER LIMB SWELLING WHICH WAS INTERMITTENT AND RELIEVED ON MEDICATION. H/O FEVER SINCE 4 DAYS WHICH WAS HIGH GRADE,
INSIDIOUS IN ONSET, ASSOCIATED WITH CHILLS AND RIGOR. NECK PAIN INSIDIOUS IN
ONSET, AGGREVATED ON MOVEMENT, ASSOCIATED WITH RIGIDITY. LEFT LOWER LIMB
SWELLING EXTENDING ENTIRELY UPTO THE KNEE, RAISED LOCAL TEMPERATURE AND
PAIN. NO COMPLAINTS OF VOMITINGS, CHEST PAIN OR PALPITATIONS.
PAST HISTORY: K/C/O HTN SINCE 1 YEAR (ON AMLODIPINE 5 MG). N/K/C/O DM, CVA, CAD, TB, ASTHMA OR EPILEPSY.
PERSONAL HISTORY: MARRIED, MIXED DIET, NORMAL APPETITE, REGULAR BOWEL AND BLADDER HABITS, NO KNOWN ALLERGIES, REGULAR ALCOHOL CONSUMPTION PRESENT.
GENERAL EXAMINATION: PATIENT IS C/C/C. NO PALLOR, ICTERUS, CYANOSIS, CLUBBING, LYMPHADENOPATHY OR PEDAL EDEMA.
VITALS: TEMP-AFEBRILE, BP-90/60 MMHG, PR-115,
RR- 30CPM, SPO2- 96% AT RA, GRBS-222 MG/DL. GCS: E4V5M6
SYSTEMIC EXAMINATION: CVS, CNS, RS, P/A- NORMAL
** Tentative Date Page-2
KIMS HOSPITALS
Investigation
COMPLETE URINE EXAMINATION (CUE) 29-08-2025 02:52:PM COLOUR Pale
yellow APPEARANCE Clear REACTIONAcidicSP.GRAVITY1.010ALBUMIN Trace SUGAR Nil BILE SALTS Nil BILE PIGMENTS Nil PUS CELLS2-4EPITHELIAL CELLS2-3RED BLOOD
CELLS Nil CRYSTALS Nil CASTS Nil AMORPHOUS DEPOSITS Absent OTHERS Nil Arterial Blood Gas
Analysis (ABG) 30-08-2025 11:15:PM PH7.30PCO230.0PO259.0HCO314.6St.HCO316.1BEB-
10.3BEecf-10.5TCO231.2O2 Sat90.4O2 Count11.5RFT 30-08-2025 11:15:PM UREA71
mg/dlCREATININE2.8 mg/dl URIC ACID5.3 mmol/LCALCIUM8.3 mg/dlPHOSPHOROUS2.7
mg/dlSODIUM140 mmol/LPOTASSIUM3.3 mmol/L.CHLORIDE 98 mmol/L
RFT 02-09-2025 01:44:AM
UREA53 mg/dl CREATININE2.3 mg/dl URIC ACID4.6 mmol/LCALCIUM7.8 mg/dlPHOSPHOROUS2.0
mg/dlSODIUM139 mmol/LPOTASSIUM3.4 mmol/L.CHLORIDE98 mmol/L
HEMOGRAM: HB-10.9 G/DL, TLC-20500, RBC- 3.2 MILLIONS/MM3, PLT- 1.6 LAKH/MM3, APTT-32s, PT-16s, INR-1.11, LDH-283 IU/L, ESR-85MM/1ST HR, BLOOD LACTATE-3.4.
USG IMPRESSION: GRADE-III RPD CHANGES OF RIGHT KIDNEY, GRADE-I RPD CHANGES OF LEFT KIDNEY, B/L RENAL CORTICAL CYSTS.
Treatment Given (Enter only Generic Name)
IV FLUIDS NS@ 75ML/HR
INJ. PIPTAZ 2.25 GM IV/TID
INJ. CLINDAMYCIN 600MG IV/BD
INJ. PAN 40MG IV/OD
INJ. NEOMAL 1 GM IV/SOS IF TEMPERATURE >101F
INJ. THIAMINE 200MG IV/TID
TAB. DOLO 650MG IV/TID
NEB. IPRAVENT 6TH HRLY AND BUDECORT 8TH HRLY
LEFT LOWER LIMB ELEVATION AND REGULAR DRESSINGS
SYP. POTKLOR 15 ML PO/TID
TAB. CHYMORAL FORTE PO/TID
Advice at Discharge
SALT RESTRICTION <2GM/DAY
FLUID RESTRICTION <1.5LIT/DAY
TAB.CLINDAMYCIN 600MG PO/TID 7 DAYS
TAB.AGUMENTIN 625MG PO/TID 7 DAYS
TAB. PAN 40MG PO/OD FOR 7 DAYS
TAB. DOLO 650MG PO/SOS
TAB. CHYMORAL FORTE PO/TID FOR 5 DAYS
** Tentative Date Page-3
KIMS HOSPITALS
TAB.NODOSIS 500MG PO/BD FOR 1MONTH
TAB.OROFER XT PO/OD FOR 1 MONTH
TAB. SHELCAL PO/OD FOR 1MONTH
REGULAR DRESSINGS FOR LEFT LOWER LIMB
Follow Up
REVIEW TO NEPHROLOGY OPD AFTER 1 WEEKS/SOS WITH RFT AND HEMOGRAM REPORTS
REVIEW TO GENERAL SURGERY OPD FOR REGULAR DRESSINGS
When to Obtain Urgent Care
IN CASE OF ANY EMERGENCY IMMEDIATELY CONTACT YOUR CONSULTANT DOCTOR OR
ATTEND EMERGENCY DEPARTMENT.
Preventive Care
AVOID SELF MEDICATION WITHOUT DOCTORS ADVICE, DONOT MISS MEDICATIONS. In case of Emergency or to speak to your treating FACULTY or For Appointments, Please Contact:
08682279999 For Treatment Enquiries Patient/Attendant Declaration : - The medicines prescribed
and the advice regarding preventive aspects of care, when and how to obtain urgent care have been
explained to me in my own language
SIGNATURE OF PATIENT /ATTENDER
SIGNATURE OF PG/INTERNEE
SIGNATURE OF ADMINISTRATOR
SIGNATURE OF FACULTY
Discharge Date
Date:3/9/2025
Ward: AMC
NEPHROLOGY
[05-09-2025 21:02] PPM 1: The main point of interest in this patient was the diagnostic uncertainty where OPLL due to Narketpally fluorosis was confounding a diagnostic uncertainty between neck rigidity and meningitis vs uremia sepsis encephalopathy similar to the illustrative case published in @PPM7 @PPM6@PPM5 @PPM2 @CR @PPM3's report here: https://pubmed.ncbi.nlm.nih.gov/40674544/
While that case was mentioned in passing in the above report mainly to illustrate the Narketpally imaginary pillow sign, it was archived in detail and can be accessed from the reference 29 of the paper here: https://onlinelibrary.wiley.com/doi/10.1111/jep.70186
[06-09-2025 01:06] PPM 3: Certainly! I will analyze the diagnostic challenges involving OPLL from Narketpally fluorosis confounded with meningitis vs. uremic sepsis encephalopathy. The main contents of the report are as follows:
- *Introduction and clinical background*: Overview of the complex diagnostic scenario and key clinical features.
- *Fluorosis and OPLL*: Explains the relationship between fluorosis and OPLL, including diagnostic methods.
- *Meningitis vs. meningism*: Differentiates between true meningitis and OPLL-induced meningism.
- *Uremic sepsis encephalopathy*: Details pathophysiology and diagnostic features of USAE.
- *Diagnostic approach*: Provides a systematic method for differential diagnosis.
- *Management strategies*: Outlines targeted treatment approaches for each condition.
- *Conclusion*: Summarizes key points for clinical differentiation.
-------
# Diagnostic Complexity in Ossification of Posterior Longitudinal Ligament (OPLL) with Fluorosis: Differentiating Neck Rigidity of Meningitis versus Uremic Sepsis Encephalopathy
## 1 Introduction and Clinical Background
The *diagnostic challenge* presented in this case represents a fascinating convergence of musculoskeletal, infectious, metabolic, and neurological pathologies. This complex clinical scenario involves a patient with *ossification of the posterior longitudinal ligament (OPLL)* in the context of *endemic fluorosis* from the Narketpally region, which creates diagnostic uncertainty between meningeal signs suggestive of meningitis and altered mental status potentially attributable to *uremic sepsis encephalopathy*. The mention of the "Narketpally imaginary pillow sign" references a specific clinical observation documented in previous case reports from this region known for its high fluoride content in groundwater. This sign reportedly manifests as a characteristic posture adopted by patients to alleviate discomfort from cervical spine pathology related to fluorosis.
The complexity of this case lies in the fact that *neck rigidity—a classic sign of meningeal irritation—can also present in advanced cervical OPLL due to mechanical constraints on spinal movement. Simultaneously, **systemic illness* with sepsis and uremia can produce encephalopathy that further clouds the clinical picture. This intersection of pathologies creates a diagnostic dilemma requiring careful consideration of epidemiology, clinical presentation, diagnostic findings, and targeted investigations to arrive at an accurate diagnosis and appropriate treatment plan. The following sections will systematically analyze each component of this complex presentation and provide a framework for differential diagnosis.
## 2 Understanding Fluorosis and OPLL in the Narketpally Context
### 2.1 Epidemiological and Pathophysiological Considerations
- *Endemic fluorosis* in the Narketpally region of India results from *excessive fluoride content* in groundwater, which leads to chronic fluoride accumulation in the body. This condition affects multiple systems, with *skeletal fluorosis* being a prominent manifestation characterized by increased bone density, ligament calcification, and osteophyte formation.
- The *pathophysiological relationship* between fluorosis and OPLL is well established, with studies demonstrating that fluoride stimulates osteoblastic activity and promotes bone formation in ligamentous tissues. Research has shown that fluoride levels are significantly higher in patients with OPLL compared to controls, with urinary fluoride levels >1.6 mg/L serving as a diagnostic indicator of fluorosis.
- *Radiological characteristics* of fluorosis-associated OPLL include continuous, segmental, mixed, or focal patterns of ossification along the posterior longitudinal ligament. The continuous and mixed variants are associated with higher urinary fluoride levels and tend to produce more severe compression of the spinal canal . Advanced imaging with *CT scanning* is superior to plain radiography in characterizing the extent and pattern of OPLL, with occupying ratios >60% and hill-shaped ossification associated with poorer neurological outcomes.
### 2.2 Clinical Manifestations of OPLL in Fluorosis
- The *primary clinical presentation* of OPLL ranges from asymptomatic incidental findings to progressive neurological deficits. Myelopathic features including gait disturbance, bladder dysfunction, and sensory changes are common in advanced cases, while radicular symptoms may predominate in earlier stages .
- *Cervical rigidity* and limited range of motion are hallmark features of advanced OPLL, which can mimic meningismus. This mechanical restriction results from the ossification process itself rather than true muscular spasm associated with meningeal irritation. The aforementioned "Narketpally imaginary pillow sign" represents a compensatory posture that patients adopt to minimize discomfort and neurological symptoms through strategic head positioning.
- The *mass occupying effect* of OPLL can lead to significant spinal canal compromise, with dural penetration occurring in severe cases. The "Rule of Nine" classification system helps surgeons assess the feasibility and safety of anterior resection based on the distribution of ossification within the spinal canal.
## 3 Differentiating Meningitis from OPLL-Induced Meningism
### 3.1 Clinical Assessment of Neck Rigidity
- *True meningismus* typically presents with nuchal rigidity that is painful in all directions of movement and is often accompanied by other signs of meningeal irritation, such as Kernig's and Brudzinski's signs. In contrast, *OPLL-related restricted movement* may demonstrate directional preference, with typically greater limitation in extension than flexion, and lacks the associated meningeal signs .
- The *temporal pattern* of symptom development offers important diagnostic clues: meningeal signs in infectious meningitis typically develop acutely over hours to days, while OPLL-related restrictions develop insidiously over months to years. However, acute exacerbation of OPLL symptoms can occur following minor trauma or whiplash injuries, potentially mimicking acute meningitis presentation .
- *Systemic symptoms* such as fever, photophobia, and severe headache are characteristic of meningitis but absent in isolated OPLL. The presence of these symptoms should prompt immediate investigation for infectious or inflammatory meningeal processes.
### 3.2 Diagnostic Evaluation
- *Cerebrospinal fluid (CSF) analysis* remains the gold standard for diagnosing meningitis. In bacterial meningitis, CSF typically shows elevated opening pressure, neutrophilic pleocytosis, elevated protein, and decreased glucose levels. Viral meningitis demonstrates lymphocytic predominance with normal glucose levels. These CSF findings would be normal in cases of isolated OPLL.
- *Neuroimaging* plays a crucial role in differentiation. While both conditions might show enhancement on post-contrast imaging, meningitis typically demonstrates leptomeningeal enhancement, whereas OPLL shows characteristic ossification along the posterior longitudinal ligament on CT scans. MRI in meningitis may also show complications such as hydrocephalus, cerebral edema, or venous thrombosis, which are absent in OPLL .
- *Advanced imaging techniques* including the "K-line" assessment on neutral lateral radiographs help determine whether OPLL exceeds the line connecting the midpoints of the spinal canal at C2 and C7. K-line negative status suggests poor posterior shift of the spinal cord after laminoplasty, helping guide surgical decision-making .
Table: Differentiating Features Between Meningitis and OPLL-Induced Meningism
| *Feature* | *Meningitis* | *OPLL-Induced Meningism* |
|---------------------------|--------------------------------|-----------------------------------|
| *Onset* | Acute (hours to days) | Chronic (months to years) |
| *Fever* | Present | Absent |
| *Headache* | Severe, diffuse | Variable, often neck-centered |
| *Kernig's/Brudzinski's* | Positive | Negative |
| *CSF Findings* | Abnormal inflammatory markers | Normal |
| *Imaging* | Leptomeningeal enhancement | Ossification along PLL |
| *Systemic Symptoms* | Prominent | Absent or related to fluorosis |
## 4 Uremic Sepsis Encephalopathy (USA) – Pathophysiology and Clinical Features
### 4.1 Sepsis-Associated Encephalopathy (SAE)
- *Sepsis-associated encephalopathy* represents diffuse brain dysfunction resulting from systemic inflammatory response to infection without direct CNS infection. The pathophysiology involves *complex mechanisms* including vascular damage, endothelial activation, blood-brain barrier breakdown, altered brain signaling, brain inflammation, and apoptosis .
- The *clinical spectrum* of SAE ranges from mild symptoms such as malaise and concentration deficits to deep coma. Delirium is a common presentation, characterized by acute fluctuation in mental status, inattention, and disorganized thinking. However, SAE is not synonymous with delirium, as symptoms may bypass the delirium stage entirely .
- *Diagnostic considerations* for SAE include the presence of systemic infection meeting sepsis criteria, exclusion of other encephalopathy causes, and supportive findings on EEG (generalized theta or delta slowing) or elevated biomarkers of brain injury (neuron-specific enolase, S-100 β protein). Importantly, SAE may present in early sepsis stages before full diagnostic criteria are met .
### 4.2 Uremic Encephalopathy in the Context of Sepsis
- *Uremic encephalopathy* represents a metabolic encephalopathy resulting from accumulated toxins in renal failure. When superimposed on sepsis, the combined effect produces more profound neurological dysfunction than either condition alone. The *pathophysiological mechanisms* include blood-brain barrier disruption, neuroinflammation, neurotransmitter imbalances, and increased brain oxidative stress.
- *Clinical features* of uremic encephalopathy include cognitive impairment, memory deficits, agitation, psychosis, seizures, and various movement disorders including asterixis and myoclonus. These features help distinguish uremic encephalopathy from other encephalopathy types, though overlap exists .
- The *diagnostic approach* to uremic sepsis encephalopathy requires comprehensive assessment including renal function tests, inflammatory markers, microbiological studies, and exclusion of other metabolic derangements. Neuroimaging may show characteristic findings such as lentiform fork sign or basal ganglia changes in uremic encephalopathy, while SAE may demonstrate leukoencephalopathy or normal imaging .
## 5 Comprehensive Diagnostic Approach to the Complex Presentation
### 5.1 Systematic Evaluation Strategy
- *Initial assessment* should prioritize identifying immediately life-threatening conditions. The *ABCs (Airway, Breathing, Circulation)* take precedence, especially given the potential for sepsis-induced hemodynamic instability and neurological deterioration that might require intensive care management .
- *History gathering* should focus on establishing the temporal sequence of symptoms, particularly which developed first: neck rigidity/stiffness or altered mental status. A careful history might reveal chronic symptoms suggestive of fluorosis (dental mottling, joint stiffness, radicular symptoms) preceding acute deterioration. Epidemiological factors including residence in endemic fluorosis areas like Narketpally should be specifically queried .
- *Physical examination* must include comprehensive neurological assessment with attention to meningeal signs, focal neurological deficits, and manifestations of systemic illness. The "Narketpally imaginary pillow sign" – a characteristic posture alleviating cervical discomfort – should be specifically sought .
### 5.2 Diagnostic Investigations
- *Laboratory evaluation* should include complete blood count, comprehensive metabolic panel (assessing renal function and electrolytes), inflammatory markers (CRP, procalcitonin), urinary fluoride levels, and blood cultures. Arterial blood gas analysis helps assess metabolic derangements and acid-base status in sepsis .
- *CSF analysis* via lumbar puncture is essential when meningitis is suspected. Measurement of opening pressure, cell count with differential, glucose, protein, gram stain, culture, and appropriate PCR studies (e.g., for HSV) should be performed. CT head is recommended before LP if concern exists for
- *Neuroimaging* with *cervical spine CT* is the gold standard for characterizing OPLL extent, increased ICP or mass effect . pattern, and spinal canal compromise. MRI brain and spine helps evaluate meningeal enhancement, spinal cord compression, and alternative structural causes. The "double-layer sign" on CT suggests dural ossification, which has surgical implications .
- *Neurophysiological studies* including EEG are valuable in detecting subclinical seizures and encephalopathic patterns. Somatosensory-evoked potentials may quantify the severity of spinal cord dysfunction in OPLL.
Table: Diagnostic Findings in Key Differential Considerations
| *Test* | *OPLL with Fluorosis* | *Bacterial Meningitis* | *Uremic Sepsis Encephalopathy* |
|-------------------|--------------------------------|----------------------------------|----------------------------------|
| *Urinary Fluoride* | >1.6 mg/L | Normal | Normal or elevated |
| *CSF Analysis* | Normal | Neutrophilic pleocytosis, ↑ protein, ↓ glucose | Typically normal |
| *Cervical CT* | Ossification along PLL | Normal or mild degenerative changes | Normal or unrelated changes |
| *Renal Function*| Normal | May be abnormal in severe sepsis | Significantly abnormal |
| *Inflammatory Markers* | Normal or mildly elevated | Markedly elevated | Elevated |
| *EEG* | Normal or mild slowing | Diffuse slowing | Moderate to severe slowing |
## 6 Management Strategies for Complex Diagnostic Scenarios
### 6.1 Targeted Treatment Approaches
- *OPLL management* depends on neurological severity and radiological characteristics. *Surgical intervention* is indicated for moderate to severe myelopathy or progressive neurological deficits. Surgical options include anterior resection with or without corpectomy and fusion, posterior decompression with or without fusion, or combined approaches. The K-line status, occupying ratio, and presence of dural ossification guide surgical approach selection .
- *Fluorosis management* primarily involves *reducing fluoride exposure* by providing alternative water sources and nutritional interventions to mitigate fluoride absorption. Symptomatic management includes analgesics for bone and joint pain and physical therapy to maintain mobility .
- *Meningitis treatment* requires prompt empiric antibiotics covering likely pathogens based on patient age and risk factors, with refinement based on culture results and CSF analysis. Adjunctive corticosteroids may be beneficial in certain bacterial meningitides to reduce inflammatory complications .
- *Uremic sepsis encephalopathy management* demands a dual approach: *infection control* with appropriate antibiotics and source control, and *renal support* with volume resuscitation, correction of metabolic derangements, and renal replacement therapy when indicated. Neurological symptoms typically improve with treatment of the underlying conditions .
### 6.2 Considerations for Multidisciplinary Care
- The complexity of this presentation necessitates a *collaborative multidisciplinary approach* involving internists, neurologists, neurosurgeons, nephrologists, infectious disease specialists, and rehabilitation physicians. This team should coordinate to develop a comprehensive management plan addressing all pathological components .
- *Long-term follow-up* is essential for monitoring OPLL progression, renal function recovery, and cognitive sequelae from encephalopathy. Serial imaging may be required to assess OPLL progression, particularly if neurological symptoms worsen .
- *Rehabilitation services* including physical therapy, occupational therapy, and cognitive rehabilitation play crucial roles in functional recovery, particularly given the potential for residual neurological deficits from OPLL myelopathy and encephalopathy .
## 7 Conclusion and Key Clinical Takeaways
The diagnostic dilemma presented by this case highlights the challenges in distinguishing between OPLL-related neck rigidity, meningeal irritation, and metabolic encephalopathy in the context of endemic fluorosis and sepsis. Several key principles emerge from this analysis:
First, a *thorough history* and *physical examination* remain fundamental, with attention to epidemiological factors (e.g., residence in fluorosis-endemic areas), temporal symptom progression, and characteristic signs (e.g., the "Narketpally imaginary pillow sign").
Second, *targeted diagnostic investigations* are essential for differentiation: urinary fluoride levels and cervical spine CT for fluorosis-related OPLL; CSF analysis for meningitis; and renal function tests, inflammatory markers, and EEG for uremic sepsis encephalopathy.
Third, *simultaneous pathologies* may coexist, particularly in complex presentations. The presence of fluorosis-related OPLL does not exclude concurrent meningitis or sepsis-related encephalopathy, necessitating comprehensive evaluation.
Finally, *multidisciplinary management* is crucial for addressing the diverse pathological elements, including surgical intervention for symptomatic OPLL, infection control and renal support for uremic sepsis encephalopathy, and long-term rehabilitation for optimal functional outcomes.
This case underscores the importance of maintaining broad differential diagnosis and systematically evaluating each potential contributing condition to develop appropriate, targeted management strategies for patients with complex, multifactorial presentations.
Table: Priority Interventions Based on Diagnostic Considerations
| *Diagnostic Possibility* | *First-Line Interventions* | *Specialist Involvement Needed* |
|----------------------------|-------------------------------------------|-------------------------------------|
| *OPLL with Myelopathy* | Surgical consultation, cervical spine MRI/CT | Neurosurgery, Neurology |
| *Bacterial Meningitis* | Immediate empiric antibiotics, CSF analysis | Infectious Disease, Neurology |
| *Uremic Encephalopathy* | Renal function support, metabolic correction | Nephrology, Critical Care |
| *Sepsis-Associated Encephalopathy* | Infection source control, hemodynamic support | Infectious Disease, Critical Care |
| *Skeletal Fluorosis* | Fluoride reduction, pain management | Endocrinology, Rheumatology |
[06-09-2025 06:42] PPM 1: Thanks for these very useful insights
[06-09-2025 10:25] PPM 4: Yes, absolute feast.
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