18100006003 CASE PRESENTATIONS
LONG CASE:
A 44 year old man presented with a 3-day history of bilaterally symmetrical rapidly progressive generalized edema.
Present Illness
An agile stonemason, the patient reported that this symptom first started suddenly 3 days ago, at night, when he noticed he started feeling facial puffiness with pedal edema. The next morning, while brushing his teeth, the patient noticed he had facial puffiness, in the mirror. At the same time, he also noticed that he developed bilaterally symmetric, pitting type pedal edema, extending upto the middle of his legs. He immediately presented to the hospital with these complaints.
On interviewing the patient further, he denied having breathlessness, palpitations or chest pain. He reported frothing of urine but no haematuria. He also reported gradually decreasing urine output over the past 3 days. He did not have pain during micturition, no pus or any other abnormal discharge (casts) in urine. He did not have any history of vomiting or diarrhea, no history of acute retention of urine, no prior history of fever or rash, no history of antibiotic usage or any drugs in the past 1 week. The patient also denied any history of yellowish discoloration of skin or sclera.
Prior to this, the patient reported that since 2011, he had severe joint pains, which were initially asymmetric and gradually became bilaterally symmetrical and involving the small joints of his hands and wrist. The joint pains were associated with significant local edema, and painful limitation of movements, which made his job (stonemasonry) difficult.
[Other activities which were painful and difficult for the patient were -
Holding his cup of tea or glass of water,
Pain in his finger joints and wrist while brushing,
Pain while holding mug when taking bath and
Pain in toes and ankles on both sides when walking)]
He reported that he also had debilitating early morning pains and limitation of movements in his hands, wrists and feet, which usually lasts for about an hour, He reported that the pains and limitation of movements improved with activity, with gradual reduction in edema of joints.
From 2011 to 2019, these joint symptoms gradually progressed in severity, now also involving several large joints (shoulders, elbows, knees and hips) warranting several medical consults, where he was frequently prescribed pain killers. The patient did not have any documentation of the pain killers he took in these 8 years. In December 2020, he presented to our hospital with similar complaints of joint pains, when he was prescribed with Etoricoxib and Febuxostat (he had hyperuricemia). He reported that his symptoms alleviated with these drugs but he intermittently had worsening of same symptoms in the interim. The patient denied any history of skin rash, photosensitivity, nasal or oral ulcers, chest pain or abdominal pain, weakness in his limbs (such as difficulty in taking stairs or lifting heavy stones and nor any weakness in his distal aspects of limbs such as mixing food, buttoning his shirt or holding a glass or slipping of footwear), isolated single joint pain or edema, or a past history of kidney stones. He also does not have any history of difficulty in swallowing, altered bowel habits, pain in the pulp of his digits, or painful tearing, photophobia or visual loss. He also denied any history of gritty sensation in eyes or dryness of mouth.
Apart from these, the patient reported that, for the past 3 days, he has burning sensation in his eyes with increased tearing but no visual deficits. He also reported for the past 1 year, he developed subcutaneous swellings in the proximal joints of his fingers. He denies any history of early satiety or post prandial fullness or pain. He reported that his clothes have slightly loosened over the past 1 year with involuntary weight loss and loss of appetite. He denies having a history of wrist or foot drop, chest pain, palpitations or breathlessness. No history of loss of consciousness, falls or tingling or numbness in his feet or hands.
Past History
No significant past history.
Medical/Surgical History
Chronic intermittent use of analgesics (type and dose unknown). Has been using Etoricoxib 60 mg and Febuxostat 80 mg intermittently for the past 8 months. No relevant surgical history. No history of allergy or atopy.
Personal History
The patient had been working as a stonemason for the past 20 years. He is a devout Catholic Christian and a strict teetotal (has never smoked or consumed alcohol in his life). He stays with his wife and 2 children (elder son and younger daughter) in Miriyalguda. He is from a close-knit family and regularly socialises with his family (parents and his 2 elder brothers). Apart from his troubling joint pains, he used to a have a fairly balanced and good quality of life, with good sleep every night, good appetite and adequate access to nutritious food and clean drinking water. He also had a balanced social well-being with a tightly-knit community at home and his church.
However, since the last 1 year, his appetite started to decrease and he also involuntarily lost weight. His bowel and bladder habits have always been normal but these joints pains have forced him into early voluntary retirement from his job in 2019. His and his family's finances have been supported by his brothers and from generous donations from his church. He feels his mental health has remained intact, thanks to his supportive family and fellow churchgoers.
Family History
No significant family history reported.
Social & Educational History
Married for 18 years with 2 children. Primary education upto Class 7 in Telugu medium.
Immunization History
None taken since birth.
Problem Representation / History Analysis
A 44 year old stonemason from Miriyalguda, presented with a 3 day history of anasarca, frothy urine and gradually decreasing urine output, on a background of a 10 year history of chronic bilaterally symmetric polyarthritis (evidenced by severe pain, edema and limitation of joint movements).
Localisation of Acute Problem
Anasarca and frothy urine with decreasing urine output suggest a renal pathology. Proteinuria causing anasarca likely due to glomerular pathology. Other systemic causes like heart failure and liver dysfunction can be ruled out due to absence of dyspnea, palpitations, bendopnea or syncope. Liver dysfunction can be ruled out by lack of jaundice, melaena or hematemesis (from bleeding varices), and abdominal distention not occurring prior to pedal edema.
Within the kidney, pre-renal and post-renal causes can be effectively ruled out from the absence of volume loss (vomiting, diarrhea, diuretic abuse or burns) and no history of acute retention of urine or lower urinary tract symptoms (LUTS) like frequency, urgency, hesitancy or precipitancy. The presence of frothy urine and edema strongly supports a glomerular pathology due to significant loss of protein and also decreased urine output. Isolated defects in tubular/interstitium are unlikely as such patients have a deficit in maintaining urinary concentration, which causes polyuria. Such a high range of proteinuria causing anasarca is also not seen with tubular/interstitial pathologies alone.
Provisional Diagnosis - Acute Glomerulopathy (Glomerulonephritis / Nephrotic syndrome)
Features to look for -
- Hypertension (secondary hypertension in Glomerulonephritis)
- Haematuria on Urine Microscopy (particularly dysmorphic RBCs in urine)
- Quantification of Proteinuria
- Serum Albumin / Total Proteins
- Urine specific gravity / calculated urine osmolality to check for isosthenuria (to look for secondary tubular/interstitial damage)
- Renal biopsy, if diagnosis remains uncertain
Localisation of Chronic Problem
This 44 year old man has a 10 year history of bilaterally symmetrical progressive inflammatory polyarthritis. Features favouring an inflammatory pathology are -
- Features of inflammation such as severe pain associated with edema of the joints and limitation of range of active movements
- Early morning stiffness, lasting for more than 30 mins (for 1 hour in this patient)
- Pain and edema of joints improving with activity and worsening with rest
- Features of uncontrolled systemic inflammation such as fever, involuntary loss of weight associated with loss of appetite.
- Swellings at joints and deformation of normal joint posture
Provisional Diagnosis - Bilaterally Symmetric Chronic Progressive Inflammatory Peripheral Polyarthritis
Clinical Examination
Initial examination revealed, the patient was conscious, coherent and co-operative, lying in bed in supine position. He was in some visibly apparent distress with flexion at his elbows and wrists, bilaterally, which were mildly painful when resting on the bed and his abdomen, respectively. The patient was dressed in a round neck t-shirt and when asked to sit up and take his t-shirt off, he had significant pain and limitation of movements at multiple joints but no weakness.
Vitals were taken in supine and sitting position -
Supine Position
Pulse - 92 bpm, regular, normal volume, condition of vessel wall - normal, no radio-radial or radio-femoral delay. All peripheral pulses were normal.
Blood Pressure - 140/90 mmHg
Temperature - 99.3F
Respiratory Rate - 24 cycles per minute. Mildly acidotic + (with prolonged duration of expiration)
Sitting Position
Pulse - 96 bpm, regular, normal volume, condition of vessel wall - normal, no radio-radial or radio-femoral delay.
Blood Pressure - 150/90 mmHg
Head to Toe General Examination
General Condition - Fair built and appears well nourished.
Hair - Thin and slightly greyed. Not easily pluckable or no areas of scarring or non-scarring hair loss. No lesions noted on the scalp.
Eyes - No conjunctival chemosis or injection, No redness or corneal lesions. Bilateral, purplish reticular markings noted on the sclera of both eyes. Palpebral conjunctival pallor +. No icterus. No cyanosis. Bilateral Periorbital puffiness +
| Periorbital Edema +. Pallor was also + |
General Head, Neck & ENT - No abnormalities. No lymph node enlargement.
Axial - No apparent spinal deformities
Fingers and Nails - Leukonychia +. No clubbing or cyanosis. Capillary refill time - 2 seconds.
Bilateral pitting type pedal edema +, extending upto middle of legs.
Systemic Examination
Musculo-Skeletal System
Inspection - No visibly apparent spinal deformities;
Palpation - Inspectory findings confirmed. No spine tenderness.
Movements - Atlanto-occipital - Flexion, extension and lateral flexion normal
Atlanto-axial - Rotation of head normal
Spinal Flexion, Spinal Extension, Lateral Flexion and Rotation are normal
Appendicular Skeleton - Upper Limbs (Positive Findings)
Shoulders (both sides) -
- Inspection - Attitude - Slightly flexed and internally rotated; Contour normal; No edema or erythema
- Palpation - Mild increase in temperature on both sides - Range of Movements - Mild Active and Passive limitation of all range of movements (flexion, extension, adduction, abduction, internal rotation and external rotation)
Elbows (both sides) -
- Inspection - Attitude - mid-flexion; alignment of elbow and forearm - normal; Edema + ; No scars or sinuses; no muscle wasting
- Palpation - All Inspectory findings are confirmed; Raised temperature +; Edema +; Wincing on touch + ; Fluctuation test + ; 3 point bony relationship intact
- Range of Movements - Severe pain on active movements of flexion, extension; Mild pain with supination and pronation;
Wrists (both sides) -
- Inspection - Attitude - Mild extension; Radial deviation of wrists +; Diffuse edema +; Redness +;
- Palpation - All Inspectory findings confirmed; Temperature raise +; Wincing on touch +;
- Range of Movements - Severely limited and extremely painful active movements of flexion, extension, radial deviation and ulnar deviation.
Hands (both sides) -
- Inspection - Attitude - Palmar subluxation and Ulnar deviation of the MCP joints; Swollen and Erythematous PIP joints; No swelling or redness of DIP joints; No apparent muscle wasting; Mild hyper-extension of PIP of thumbs; Pulp of fingers normal
- Palpation - All Inspectory findings are confirmed; Temperature raise +; Wincing on gentle palpation of MCP joints and PIP joints; Palpation of DIP joints is normal; Swellings also + on 3rd and 4th PIP joints on both sides. Z-deformity +.
- Range of Movements - Severe pain and severe limitation of active movements of flexion, extension and ulnar and radial deviation of MCP joints; severe pain and limitation of active and passive movements of flexion and extension at PIP joints. DIP joints normal.
Appendicular Skeleton - Lower Limbs (Positive Findings only)
Hip Joints (both sides)
- Limitation of passive movements of flexion and extension (towards the end of range of motion);
Knee Joints (both sides)
- Inspection - Swelling and erythema + ; Attitude - flexion;
- Palpation - All Inspectory findings are confirmed; Raised temperature + ;
- Range of movements - Severe pain and limitation of active and passive movements of flexion and extension and lateral and medial rotation; (Patient was unable to stand on Day 1 and was able to stand on Day 2 with analgesic use).
Ankles (both sides)
- Mild pain and limitation of active and passive movements of plantar flexion and dorsiflexion; Mild pain and limitation of movements of inversion and eversion.
- Palpation of Achilles tendon is normal.
Foot examination (both sides)
- Mild pain and limitation of passive movements of flexion and extension of MTP joints; great toe flexion and extension normal;
Other Systems Examination
Cardiovascular System - No abnormalities detected
Respiratory System - No abnormalities detected
Abdominal Exam - No abnormalities detected
Nervous System - No deficits detected
Investigations
 |
| X-ray AP view of the hands and wrists - Osteopenia and erosions of the MCP and PIP joints are noted. Scallop sign +. Significant soft tissue swelling is also noted. |
 |
| Chest X-ray PA view - Full inspiratory, underexposed film with no malrotation or angulation. Bones - Clavicle, Head of Humerus, Coracoid process and acromion of scapula appear normal. The ribs are normal. No mediastinal lymph nodes or enlargement. The right heart border shows mildly dilated right atrium. The left heart border shows a prominent aortic knuckle, the pulmonary bay area is normal, the left atrial appendage appears normal and the left ventricular free wall also appears normal. The bronchovascular markings are also prominent, likely due to underexposure. |
 |
| Standard 12 lead ECG with normal voltage and speed @ 25mm/s; P waves, QRS complexes and T waves have normal morphology and duration; P-P and R-R intervals are normal. PR and QTc intervals are normal. |
 |
| Current Admission - Blood tests |
 |
| Blood work from previous presentations to hospital. RA factor was negative 24hrs urinary protein: 1500 mg 24hrs urinary creatinine: 0.8 |
 |
| Urine Microscopy - Freshly voided urine sample was centrifuged at high speed (> 2700 RPM) and sediment collected and fixed on glass slide and examined under microscope at 400 (10x * 40x) showed DYSMORPHIC RBCs (black circles) and occasional pus cells (red circles). Dysmorphic RBCs were those that had altered shape, microcytic or with membrane defects. |
Diagnostic Approach
With a provisional diagnosis of Acute Glomerulopathy on the background of bilaterally symmetric chronic progressive erosive peripheral polyarthritis, features supporting the diagnosis of glomerulonephritis were -
- Secondary Hypertension
- Oliguria (360 ml urine in the last 24 hours)
- Hypoalbuminemia (Serum Albumin 2.5g/dl) and Anasarca
- Dysmorphic RBCs in Urine
(A review of literature was done to evaluate the sensitivity and specificity of dysmorphic RBCs for glomerular disease pathologies - One study conducted in Bangladesh showed that urinary dysmorphic RBCs were 92.7% sensitive and 100% specific for a biopsy confirmed diagnosis of glomerulonephritis. [1]
Similar values of sensitivity and specificity was also confirmed in another study jointly conducted in Australia and China, where glomerulonephritis was confirmed with renal biopsy. [2] )
Thus, with glomerular disease being most likely, an anatomical diagnosis is made. The etiological cause for glomerular injury needs to be ascertained.
A careful construction of the problem representation for this patient and insight into the sequence of his life events can provide clues that the current acute problem could be a sequelae of his long term, poorly treated chronic problem.
Thus, a good clinical diagnosis of his musculo-skeletal problems is required to get a better picture of his current illness.
The patient has Bilaterally Symmetrical Chronic Progressive Erosive Peripheral Polyarthritis. Differential diagnosis for such conditions include -
- Rheumatoid Arthritis (most likely)
- Rheumatoid Arthritis with coexistent Gout
- Psoriatic Arthritis
- Enteropathic Arthritis
- Reactive Arthritis
- SLE
- Polymyositis / MCTD (Mixed Connective Tissue Disorder) (least likely)
With Rheumatoid Arthritis being most likely, ACR/EULAR classification criteria can be applied for diagnosis -
 |
| This patient has >10 joints involved with multiple small joints involvement - 5 points; Symptom duration 10 years - 1 point; RA Factor - NEGATIVE; CRP elevated & ESR - 120 mm/hr - 1 point; Total Score - 7/10 [3] Thus, a diagnosis of Rheumatoid Arthritis is likely. The clinical utility of RA factor came into question. A review of literature showed that sensitivity of RA factor for Rheumatoid Arthritis was 28% and specificity was 87%. For non RA rheumatological disorders, the sensitivity was 29% and the specificity was 88% [4]. Thus, the authors concluded that (due to high specificity and NPV), the test is best ordered when the suspicion for a rheumatological is low but just high enough, that a negative result would increase the post-test probability of a rheumatological disorder being unlikely. This patient had a chronic history of symmetric small joint and then large joint inflammatory peripheral polyarthritis, With minor erosions notable in the PIP and MCP joints of both hands, classification criteria are diagnostic for Rheumatoid Arthritis. No history of skin rash (psoriatic arthritis) or chronically altered bowl habits (enteropathic arthritis); No history of dysuria or burning pain during micturition or a history of severe burning pain in eyes with photophobia and excessive tearing or discharge (reactive arthritis) makes the other diagnoses unlikely. Epidemiologically, SLE occurs more commonly in females at a ratio of 9:1, coupled with this, the absence of other features of SLE, such as alopecia, photosensitivity rash, nasal or oral ulcers, serositis, hemolytic anemia etc. makes this diagnosis very unlikely. The absence of muscle weakness, muscle pain and the presence of destructive arthritis makes Polymyositis / MCTD extremely unlikely (Polymyositis usually causes nonerosive arthritis). Thus the current acute glomerulonephritis can be framed in the background of Chronic Poorly Treated Rheumatoid Arthritis. 4 scenarios are possible - 1. Poorly treated RA causing Secondary Amyloidosis (most likely) Secondary amyloidosis is most commonly seen in chronic poorly treated systemic inflammatory syndromes. This study shows that secondary amyloidosis is the most common cause of rapidly progressive glomerulonephritis in patients who were untreated for more than 10 years. [4] Coupled with features of amyloidosis of the heart, this is the most likely cause of his renal dysfunction. 2. Vasculitic Glomerulonephritis (IgA Mediated) The incidence of IgA nephropathy in patients with RA is similar to that in the general population. [5] 3. Primary Glomerulonephritis (Idiopathic) These include Mesangial / Mesangio-proliferative glomerulonephritis; Membranous Nephropathy; FSGS [6] 4. Renal Dysfunction secondary to drug use (less likely) Most commonly implicated drugs causing nephritic/nephrotic syndrome are Gold and Pencillamine, neither of which the patient used. The patient had chronic intermittent use of Etoricoxib but NSAIDS usually cause Tubulo-interstitial nephritis and not nephrotic syndrome. 5. Crystal Nephropathy (less likely) Gout crystals precipitate at a pH of 7.0 and often precipitated in the collecting ducts in the medulla, causing Acute Tubular Necrosis with little interstitial or glomerular involvement. Final Diagnosis - A 44 year old, who presented with a 3 day history of anasarca and decreased urine output is diagnosed with -Acute Glomerulonephritis, likely due to Secondary Amyloidosis due to Chronic Poorly Treated Seronegative Erosive Rheumatoid Arthritis. Dilutional Hyponatremia secondary to Anasarca due to Glomerulonephritis Hyperuricemia likely due to decreased Uric Acid Excretion Precipitating Gouty Arthritis Anemia of Chronic Disease secondary to Poorly Treated Rheumatoid Arthritis. Further PlanAbdominal Fat Pad Biopsy for Amyloidosis Needle Aspiration of Synovial Fluid for Crystal Induced Arthritis (Gout) Treatment
Pedagogic Questions
The clinical data and biopsy results of 194 SA patients who were treated in Peking Union Medical College Hospital from January 2009 to June 2015 were retrospectively analyzed. Results The highest sensitivity was achieved by biopsy of affected organs,with renal biopsy 97.4%,heart biopsy 95.0% and liver biopsy 87.5%. Among non-invasive biopsy methods,tongue biopsy was found to be 75% sensitive,followed by gingiva biopsy at 57%,abdominal fat pad aspiration at 57%,rectum biopsy at 16%,and bone marrow examination at 8%. Combination of tongue and abdominal fat pad biopsy yielded a detection rate of 93.1%. Conclusions Biopsy of the involved organ has the highest sensitivity. However,combination of multiple non-invasive biopsy methods may has sensitivity comparable to organ biopsy and is safer and more convenient. [7] 2. Single DMARD vs Combination therapy ? A Cochrane review, published in The BMJ [8] looked at the clinical efficacy of Methotrexate monotherapy vs Combination therapy (MTX + Non-biological or MTX + Biologicals). Data of Methotrexate -naïve patients was gleaned from this meta-analysis - Outcomes - The major outcomes of the review were American College of Rheumatology (ACR) 50 response, a composite measure of improvement in disease activity (dichotomous outcome); radiographic progression, measured by Larsen, Sharp, or modified Larsen/Sharp scores (continuous outcome); and withdrawals due to adverse events, including death (dichotomous outcome).    3. When to initiate dialysis ? How long can we wait ? Ex tempore interpretation of the AKIKI-2 trial. [9]
4. Can Rheumatoid Arthritis and Gout co-exist together ? The study population included 813 patients, 537 (66%) were rheumatoid factor positive; 33% had rheumatoid nodules, and 53% had erosive joint disease. During 9771 total person-years of follow-up (mean 12.0 years per RA patient), 22 patients developed gout by clinical criteria. The great toe was the most common site of gout (12 of 22 patients). The 25 year cumulative incidence of gout diagnosed by clinical criteria was 5.3%. Typical intracellular monosodium urate crystals were present in 9 of 22 patients with acute gout; all had developed gout after the RA incidence date. The 25 year cumulative incidence of gout diagnosed by clinical criteria including presence of urate crystals is 1.3%. The prevalence of gout in RA on Jan 1, 2008 was 1.9% (11 of 582 patients) as opposed to expected prevalence of 5.2% (or 30 patients) based on National Health and Nutrition Examination Survey (NHANES) data using age and sex specific prevalence rates. [10] 5. Efficacy of Febuxostat vs Allopurinol for Gout ?  [11] |
References
- Sultana T, Sultana T, Rahman MQ, Rahman F, Islam MS, Ahmed AN. Value of dysmorphic red cells and G1 cells by phase contrast microscopy in the diagnosis of glomerular diseases. Mymensingh Med J. 2011 Jan;20(1):71-7. PMID: 21240166.
- Pollock C, Liu PL, Györy AZ, Grigg R, Gallery ED, Caterson R, Ibels L, Mahony J, Waugh D. Dysmorphism of urinary red blood cells--value in diagnosis. Kidney Int. 1989 Dec;36(6):1045-9. doi: 10.1038/ki.1989.299. PMID: 2689749.
- https://www.eular.org/myUploadData/files/RA%20Class%20Slides%20ACR_Web.pdf.
- Helin H, Korpela M, Mustonen J, et al. Renal biopsy findings and clinicopathologic correlations in rheumatoid arthritis. Arthritis Rheum 1995;38(2):242–7.
- Korpela M, Mustonen J, Helin H, et al. Immunological comparison of patients with rheumatoid arthritis with and without nephropathy. Ann Rheum Dis 1990;49(4): 214–8.
- Horak P, Smrzova A, Krejci K, et al. Renal manifestations of rheumatic diseases. A review. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2013;157(2):98–104.
- Zhang CL, Feng J, Cao XX, Zhang CL, Shen KN, Huang XF, Zhang L, Zhou DB, Li J. Selection of Biopsy Site for Patients with Systematic Amyloidosis. Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2016 Dec 20;38(6):706-709. doi: 10.3881/j.issn.1000-503X.2016.06.013. PMID: 28065238.
- Hazlewood GS, Barnabe C, Tomlinson G, Marshall D, Devoe DJ, Bombardier C. Methotrexate monotherapy and methotrexate combination therapy with traditional and biologic disease modifying anti‐rheumatic drugs for rheumatoid arthritis: A network meta‐analysis. Cochrane Database of Systematic Reviews. 2016(8).
- Gaudry S, Hajage D, Martin-Lefevre L, Louis G, Moschietto S, Titeca-Beauport D, La Combe B, Pons B, De Prost N, Besset S, Combes A. The Artificial Kidney Initiation in Kidney Injury 2 (AKIKI2): study protocol for a randomized controlled trial. Trials. 2019 Dec;20(1):1-0.
- Jebakumar A, Crowson C, Udayakumar D, Matteson E. Co-Existence of Gout in Rheumatoid Arthritis: It Does Happen! A Population Based Study.: 134. Arthritis & Rheumatism. 2012 Oct;64.
- Huang X, Du H, Gu J, Zhao D, Jiang L, Li X, Zuo X, Liu Y, Li Z, Li X, Zhu P. An allopurinol‐controlled, multicenter, randomized, double‐blind, parallel between‐group, comparative study of febuxostat in C hinese patients with gout and hyperuricemia. International journal of rheumatic diseases. 2014 Jul;17(6):679-86.
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SHORT CASE-1:
Case Presentation
History taken from Patient and Attendant (elder brother); Reliability 9/10.
A 49 year old English and Telugu language lecturer presented with a 2 month history of progressive asymmetric involuntary movements of his right index and middle fingers.
Present Illness
The patient reports that he first noticed them happening nearly 6 months ago, which was very small in amplitude, affecting these two fingers only. He says that these movements often worsened with rest and abated with activity. They were not troublesome initially but for the past 2 months he has been unable to correct answer sheets because of the involvement of his thumb and maintaining stability of his hand was proving difficult. He describes these movements as involuntary, rhythmic to and fro oscillations.
He also adds that his handwriting has become ugly with very small letters. On interviewing further, the patient reports that he feels stiffness in his wrists (Right>Left), which has now ascended to his elbows. He says the stiffness is present throughout the range of motion. He also says that since the last 1 month, the same involuntary movements also started appearing in his left hand.
At this point, he also says that his walking has become difficult with small, short steps and a forward stoop, and he feels that although he weighs 60 kgs. he feels like it weighs 100 kgs.
He does not report any difficulty in reading the newspaper, holding the paper, turning pages or folding it back. He does not have any difficulty in brushing his teeth or combing his hair. He also denies having difficulty in holding objects, such as holding a water bottle to drink nor any difficulty in mixing food and eating it. He does not have any difficulty in wearing a vest or in buttoning or unbuttoning his shirt. No difficulty in lifting his lower limbs and wearing a trouser.
The interview continues and we question for any difficulty in taking the stairs - he reports that he has been having difficulty in taking stairs up, in that he feels he sometimes might lose balance. He has no difficulty in descending stairs. The patient also denies having swaying of his trunk while walking or overshooting his hand while picking up objects.
On pressing further - he reports that he hasn't been having morning erections since 2 months and also reports a loss of sexual desire. He also says that since 2 months his bowel habits have been incredibly erratic, in that he sometimes has an immediate urge to defecate when he has tea and sometimes goes 2 to 3 days with constipation.
He, however, denies feeling dizzy or lightheaded when waking up in the morning. He denies having stiffness in his lower limbs, denies cotton wool sensation of floor, denies burning pain or inability to feel hot or cold stimuli. He also denies buckling of knees but, however, he reports that he has been having a great difficulty to walk in the dark since 2 months and says that he feels like he would definitely fall without support.
His brother gives a positive affirmation for all his symptoms and also says that he previously used to be a fairly jovial and hardworking man with good oratory skills, however, since the last 2 months he says his brother's speech lacks that 'edge' which he previously had. On asking further, the brother says that he has been speaking in a monotonous drab since 2 months.
The patient and his brother deny noting any fluctuations in his alertness, apathy or emotional instability to family or personal issues, no history of visual hallucinations, no history of incoherence of speech or irrelevant talk and no history of difficulty in managing finances or poor self care.
The patient denies ever having urinary incontinence, memory deficits, the brother vehemently denies the patient ever being anti-social, he does not have any difficulty in forming new memories or any visual deficits.
Past History
No similar complaints in the past. No history of Diabetes, Hypertension, Coronary Artery Disease, Seizures, Asthma or Tuberculosis.
Medical/Surgical History
The patient is not on any medications. He also did not report usage of any herbal or alternative medicine. He has no past surgical history.
Personal History
The patient is a teacher; he teaches English and Telugu for B.com and B.sc students in a college in Miriyalguda. He has been in this profession for the last 9 years. He has a triple M.A. in English, Sanskrit and Telugu.
The patient neither smokes nor drinks, he had a fairly routine lifestyle with waking up early in the morning and getting ready for his job. However since the lockdown, he had to stay at home with his college shut. He spends most of his time reading the newspaper and other literature. He lives with his wife and one son, who is studying Inter 2nd year. His sleep habits are well maintained. However, his bowel habits have changed in the last 2 months as detailed above. His appetite is good, he consumes vegetarian food on most days with a good access to nutritious food and clean drinking water. He occasionally enjoys non vegetarian food. He socialises well with his extended family and his local community. He is well respected among his peers and students alike.
Family History
No significant family history reported.
Social & Educational History
Married for 24 years with 1 child. Triple degree in MA in English, Sanskrit and Telugu.
Immunization History
Complete upto age 5.
General Examination
Patient is conscious, coherent & cooperative.
Vitals at the time of history taking -
PR - 88 bpm
BP - 190/110 mm Hg
After standing for 3 mins - BP - 160/110 mm Hg
Temp - Afebrile
RR - 16 cpm
No signs of Pallor, Icterus, Cyanosis, Clubbing, Generalized lymphadenopathy or edema.
Nervous System Examination
Higher Mental Functions
1. Level of consciousness - Normal (GCS 15/15)
2. Attention - Intact
3. Orientation - to time, place and person - Intact
4. Language - fluency & latency, comprehension, repetition, naming, reading and writing - Intact. Prosody - impaired
5. Memory - immediate recall, recent and remote - Intact
6. Other higher mental functions - general knowledge, abstraction, judgement, insight and reasoning - Intact.
MMSE
29/30 (No cognitive impairment)
CN Examination
1st
Normal (smell of soap).
2nd
Counting fingers at 6mts both eyes normal.
3rd,4th,6th
Pupil size. N N
DLR/CLR. N. N
No pstosis, nystagmus.
5th
Both sensory & motor normal.
Corneal & Conjunctival reflex +.
7th
Nasolabial fold normal.
No deviation of mouth.
Salivation & Lacrimation unaffected.
8th
Rinne's AC>BC.
Weber's - No lateralization.
9 th, 10th &
Palatal movements normal.
No difficulty in swallowing.
Gag reflex present.
11th
Movements of neck in all directions+.
Lifting of shoulders +.
12th
Tone of tongue - Normal.
No wasting, no fibrillations & deviation of tongue.
Tongue tremor+.
MOTOR SYSTEM
Right. Left
✓Bulk Normal Normal
✓Tone
Upper limb R. L
Shoulder Normal Normal
Elbow Normal Normal
Wrist Hypertonia Normal
(Cog wheel rigidity)
Lower limb Normal Normal
✓Power
UL
Proximal 5/5 5/5
Distal 5/5 5/5
LL
Proximal 4/5 4/5
Distal 5/5 5/5
✓Reflexes
Superficial reflexes
Right Left
Corneal + +
Conjunctival + -
Abdominal + -
Plantar - -
Deep tendon reflexes
Right Left
Biceps 2+ 2+
Triceps 2+ 1+
Supinator 1+ Absent
Knee 3+ 3+
Ankle 1+ 1+
Clonus Absent. Absent
Involuntary movements - Resting tremors of Right upper limb , 3-4Hz, high amplitude.
Gait - Reduced arm swing.
Finger tap and toe tap - Normal.
No decrease in speed on repeating the movement continuously.
✓SENSORY SYSTEM
Right Left
Pain + +
Fine touch + +
Temperature + +
Vibration
Medial malleolus 5.7s 4.6s
Patella 9s 4.3s
Elbow 4.8s 6.4s
Wrist 5s 7s
Proprioception Normal Normal
Stereognosis Normal Normal
✓CEREBELLUM
Titubation - absent
HINTS
Head Impulse - negative
Nystagmus - negative
Test for skew - negative
Gait Ataxia absent.
Dysarthria absent.
Rebound phenomenon absent.
Intentional tremors - absent.
Pendular knee jerk - absent.
Tandem walking normal.
Coordination tests
Dysdiadochokinesia absent
Finger nose test normal
Heel knee test normal
Rombergs Test - Negative
Micrographia +
✓Findings - The movements in the right lower limb is slower than the movements in the left lower limb.
✓The first test was only toe tapping, the second test is entire foot tapping.
Rapid supination and pronation - Diadochokinesia
✓MENINGEAL SIGNS
Neck stiffness - Absent
Kernig's sign - Negative
Brudzinski's sign - Negative
✓AUTONOMIC
Postural hypotension+(Supine - 180/110, Standing 160/90).
Resting tachycardia absent(PR 80/min,regular)
Abnormal sweating absent.
H/O erectile dysfunction+.
Cardiovascular System
S1, S2 +.
Apex beat in 5th ICS on the MCL.
JVP normal.
✓ Shows Sinus Tachycardia with pseudo infarct pattern in leads I and aVL with dagger q waves in the same leads.
✓ No late intrinsicoid deflection of R wave with modified Cornell criteria showing LVH.
2 D Echo
✓ Grade II diastolic dysfunction.
Respiratory System
Shape & symmetry of chest - Normal
Respiratory movements - Equal on both sides
BAE+
NVBS
Abdominal Examination
Soft, Non-tender
No organomegaly
Bowel sounds+
Problem Representation -
A middle aged man presenting with a 6 months history of gradually progressive, asymmetric rest tremor with autonomic features is provisionally diagnosed with
1. Idiopathic Parkinson's Disease Stage 1 with denovo HTN.
2. Multiple System Atrophy - Parkinsonian Type (MSA-P).
Treatment
1. Tab. Syndopa Plus 125 mg QID
2. Tab. Syndopa 125 mg CR OD
3. Tab. Telma 40 mg OD
Sourced From - Resting Tremor (sravyakandala.blogspot.com)
-------------------------------------------------------------------------------------------------
SHORT CASE-2:
19 year old male resident of Nalgonda and currently studying intermediate ,came to opd with complaints of :
-Itchy Ring leisons over arms ,abdomen ,thigh and groin since 1 and half year .
-Purple stretch marks all over abdomen ,lower back ,upper limbs ,thighs since 1 year .
-Abdominal distension and facial puffiness since 6 months.
- Pedal edema since 3 months.
- Low back ache since 3 months .
- Feeling low , not feeling to talk to anyone.
- Weight gain and decreased libido since 3months.
- Loss of libido and erectile dysfunction since 2 months .
Pt was apparently alright one and half year ago , when he slowly developed erythematous round leisons which are annular shaped and itchy all over abdomen , upper limb ,groin and inner thigh region .
No history of fever back then. No other complaints apart from skin lesions.
Pt visited local RMP where he prescribed auyurvedic medications and other creams ( unknown composition as pt don't have them currently ). He also prescribed tablets (unknown composition) . Patient started using all these medications for 1-2 months .
Leisons reduced a bit after using medications .
Later after 2 months he developed multiple hyper pigmented plaques over lower limbs ,abdomen , for which he again visited same place and used ayurvedic oils over the leisons.
He also used clobetasol ointment over the leisons.(for approximately 1 year all over the body)
He started noticing pink striae over his abdomen first and later on back and over arms,which were gradually increasing in size .
Later he visited a hospital and used miconazole and luliconazole ointments also.
He used clobetasol ointment all over the leisons for long time .
He started noticing abdominal distension and facial puffiness ,weight gain, but never visited any hospital.
Later he developed pedal edema and low back ache since 3 months .
His consulted a dermatologist at this point of time who advised to consult physician and prescribed monteleukast , itraconazole tablets ,luliconazole ointment for tenia corporis.
He stopped all medications one month ago and visited our opd with complaints of pink striae and easy fatigue ,weakness and low back ache .
His brother also gave history of pt being moody and feeling of low self esteem due to multiple leisons.
He even complaints pt wouldn't step out of house and always stays indoor and wouldn't interact with others .
No complaints of chest pain ,sob , palpitations .
No complaints of decreased or frothy urine.
No other negative history.
No h/o DM,HTN,TB,ASTHMA,CAD.
ALLERGIC HISTORY - pt gives h/o allergy to eggs ,brinjal .
O/E : Pt was c/c/c
BP - 160/100 mmHg
Pr - 96 BPM ,regular ,normovolemic .
Rr - 18/min
Spo2- 98% on ra.
Weight - 63 kg.
Height - 175 cm.
GENERAL EXAMINATION :
NO pallor ,icterus ,cyanosis , clubbing, lymphadenopathy.
Pedal edema present - pitting type extending upto knee.
Abdominal distension present.
Moon face present
Pink striae noted over anterior abdominal wall and on low back and on upper arms and thighs.
Thin skin present .
Poor healing noticed over leg ulcers and easy bruising noted .
Acne present over face .
Acanthosis nigrans noted over neck.
GYNECOMASTIA PRESENT .
Buffalo hump present .
Sparse scalp hair .
.
Skin examination - Multiple itchy erythematous annular leisons noted all over abdomen , upper limb ,groin and inner thigh region .
Multiple hyperpigmented plaques noted over bilateral lower limbs .
SYSTEMIC EXAMINATION :
CVS - S1S2 heard .No murmurs
RS - BAE present .
No adventitious sounds .
P/A - Soft , distended .
No organomegaly .
Bowel sounds present .
CNS - HMF - INTACT R. L
MOTOR SYSTEM - POWER - UL 5/5 5/5
LL 5/5 5/5
Proximal muscles lower limb - power is 4/5 .
TONE - NORMAL.
REFLEXES - B. T. S. K. A. P
R. +2 +2. +1. +2. +1. FELXOR
L. +2. +2. +2. +2. +1. FLEXOR.
CRANIAL NERVES - NORMAL .
Difficulty in getting up from chair was noticed.
PROVISIONAL DIAGNOSIS -
? IATROGENIC CUSHINGS SYNDROME .
TINEA CORPORIS .
DENOVO HTN .
INVESTIGATIONS :
CBP - HB - 13.4 g/dl
TLC - 6,800
PLT - 1.5 lakhs.
RBS - 139 mg/dl
CUE - ALBUMIN - +1
SUGARS - NIL .
PUS CELLS - 3-4
RBC - NIL .
LFT - TB -1.03
DB-0.21
ALBUMIN - 3.9
RFT - UREA - 22
SERUM CREATININE -0.6
ELECTROLYTES - NA - 136
K- 4
CL-98
USG ABDOMEN - NORMAL.
ECG - SINUS TACHYCARDIA
LVH PRESENT.
This was picture of striae one year ago when it gradually started :
On presentation to opd pictures : 28/05/21
We took dermatologist opinion for tenia corporis where they advised
Ointment AMLORFINE
FUSIDIC ACID CREAM.
SALINE COMPRESS OVER LEISONS.
Plan to start anti fungals on next visit once dose of steroids is reduced .
OPTHAL opinion Was taken to look for visual acuity and cataract .
No features of lens opacities noted .
BUT IOP was high ,where they advised to follow up .
We advised pt to get fasting 8am serum cortisol levels and was planned to start on low dose steroids to avoid adrenal crisis.
8AM S CORTISOL LEVELS (30/5/21)
- 0.46 mcg/dl ( very low) .
( normal range - 4.3-22.4 mcg/dl).
In view of lvh pt was started on tab telma 20 mg od .
On 3/6/21 - ACTH STIMULATION TEST WAS DONE .
BY INJECTING 0.4 ML OF ACTOM PROLONGATUM INJECTION (ACTH) INTRA MUSCULAR @ 7am
1 HR LATER FASTING SERUM CORTISOL SAMPLE WAS SENT .
VALUE - 0.73 mcg/dl
Indicating there was HPA AXIS suppression and pt was started on TAB HIZONE 15 mg per day in three divided doses @ 8am ,12 pm and 4 pm.
Pt was asked to follow up after one month .
ON NEXT VISIT : ( 25/6/21).
Pt was symptomatically better , pedal edema subsided.
Striae were pale in color and we're subsiding.
Weight - 67kg
Ht -175 cm.
Bp- 160/100 mmHg.
Pr -88bpm.
Dose of Tab hizone was reduced to 10 mg per day in divided doses for one month.
In view of low back ache Xray LS spine was done which was normal and pt was advised.:
Tab Shelcal 500 OD and Tab Vit D 3 Od.
Tab ULTRACET /PO/SOS.
Psychiatry opinion was taken and he was diagnosed with moderate depression .
In July 2021 pt was complaining of fever ,sore throat and dry cough since 3 days and he was tested positive for COVID 19 , we advised him home isolation and PCM 650 Mg /po /sos .
He was advised to continue tab hizone tablets as it was advised.
He recovered within one week .
Next visit : ( 6/8/21).
BP- 170/100 - TELMA DOSE WAS INCREASED TO 40 MG OD.
PR - 88bpm.regular , normovolemic.
Wt- 69 kg
Height -
Abdominal girth - 96cm
Pt complaints of excoriation over striae and appearance of erythematous macules over abdomen whenever he takes food he is allergic to.
Took dermatologist opinion for it . They started him on Tab Itraconazole 100 mg bd. And lulifin cream and tab levocitrixine 5mg od.
His brother complaints of depressed mood , pt not going out due to social stigma. Psychiatric counselling was given .
He still complaints of low back ache..othropedics opinion was taken and advised to continue Ultracet and tab Shelcal .
Cbp , cue and electoltes were repeated which were all in normal range .
USG ABDOMEN was done - Normal kidney size bilateral and CMD maintained. No other sonological abnormality noted.
As his lesions dint subside we reduced dose of hisone to 7.5 mg per day ,to see response.
At this point of time we are now in diagnostic dilemma whether endogenous CUSHINGS is also present in this patient , as he is responding slowly to treatment .
We advised him to review after 15 days to see progress . And accordingly plan to evaluate further to rule out endogenous CUSHINGS SYNDROME.
FINAL DIAGNOSIS :
IATROGENIC CUSHINGS SYNDROME SECONDARY TO TOPICAL CLOBETASOL APPLICATION ALL OVER BODY FOR APPROXIMATELY ONE YEAR.
TINEA CORPORIS
DENOVO HTN .
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