18100006005 CASE PRESENTATIONS

LONG CASE:


History taken from patient's wife and younger son and is reliable .

A 59yr old male patient who is a right handed, resident of Hayathnagar, carpenter by occupation presented with

 chief complaints of  

-decreased appetite since 6 months 

-yellowish discolouration of eyes and urine since 6months

-difficulty in passing stools since 3months

-altered behaviour since 1month

History of present illness :

Patient was apparently alright 6months back .Then he had fever -low grade ,Intermittent,not associated with chills and rigors,lasting for 45days ,relieved on taking medication .

Associated with decreased appetite .

Complaints of yellowish discolouration of eyes and urine for which he consumed Ayurvedic medicine powder.

 In April 2021 he consumed leaf medicine for prevention of corona ,after that he had difficulty in passing stools (passed stools every 4-5days), not relieved by taking laxatives and enema .

Then he developed abdominal distention-acute in onset,diffuse ,gradually progressive ,associated with pedal edema and scrotal swelling, relieved on paracentesis (2-2.5litres ) and albumin infusion.

Not associated with facial puffiness.Associated with vomiting-greenish,4-5episodes ,non projectile, contained bile and food particles what ever he consumed.

Complaints of altered behaviour, staring look ,not responding to speech ,irritable since 1 month and got hospitalized ,improved on medication and got discharged .

Since 6days patient became drowsy ,excessive sleeping, and constipation and loss of appetite .

Complaints of rash over upper thorax since 6days .

No h/o pain abdomen

No h/o Melena.

No h/o hematemesis .

No h/o breathlessness, chest pain 

No h/o dysphagia

No h/o weight loss

No h/o decreased urine output


Past history:

History of jaundice every 6-8months since 20yrs

History of surgery for sinusitis 7yrs back

History of RTA (fall from byke) and injury to left thigh 3yrs back

Not a known case of hypertension , diabetes,epilepsy ,asthma, Tuberculosis,thyroid disorder , CAD.

Family history:

No similar complaints in family 

Personal history :

Marietal status: married

-Mixed diet

-Loss of appetite

Alcoholic -since 40yrs (consumed 2beers(24gm of alcohol)daily 

No habit of smoking or tobacco chewing

Bladder habits regular and normal 

Bowels -constipation since 4months

Sleep -excessive day time sleepiness.

Socio economic status: upper middle class

Drug history :

Took ayurvedic leafs 4months back 

And consumes Ayurvedic powder every time he had jaundice .

No h/o any drug allergy

Summary: A 59 year old male,carpenter by occupation,who is a chronic alcoholic  and non hypertensive and non Diabetic presented with decreased appetite and yellowish discolouration of eyes and urine since 6months, difficulty in passing stools since 3months and altered behaviour since 1month and became drowsy since 6days with history of ascites 1month back.

Provisional diagnosis :  

1. Decompensated chronic liver disease with cirrhosis secondary to alcohol -stage 3 hepatic encephalopathy.

Differential Diagnosis

1. Chronic liver disease secondary to alcoholism

2.acute liver injury secondary to leaf medicine 

3.cirrhosis secondary to NAFLD 

4.Cirrhosis secondary to hepatitis 

General examination:

Moderately built and nourished

Patient is drowsy ,arousable,not cooperative and not responding to commands.

GCS: E3V1M5

BMI: 24.8kg/m²

Vitals :

Pulse -

95beats per minute , regular, normal volume ,vessel wall normal ,no radio-radial delay ,no radio femoral delay, all peripheral pulses felt.

Blood pressure:

right arm -110/80mmHg ,supine position 

Left arm -110/80mmHg ,supine position 

Right leg - 130/80mmHg 

Left leg -130/80mmHg 

Respiratory rate- 18cycles per minute, abdominothoracic ,no usage of accessory muscles.

Temperature - 98.2F

SpO2-98% at room air

JVP - not elevated

Physical examination: 

Pallor +

Icterus+

No cyanosis

No clubbing 

No generalized lymphadenopathy

Pedal edema +

B/l Parotid enlargement +

No alopecia

No fetor hepaticus

No gynecomastia

No asterexis 

Axillary hair sparse

Spider never +

No Palmar erythema 

Flanks full

Dilated veins over abdomen

No testicular atrophy

No loss of pubic hair


Systemic examination:

Gastrointestinal system 

Inspection

Oral cavity - no dental caries, no tobacco staining. 

Abdomen- Flanks full, mild distenstion+

Skin over abdomen normal

Umbilical-normal in position

Movement of corresponding quadrants normal with respiration

Dilated veins +

No visible peristalsis, no visible pulsation,

No scars or sinuses. 

Hernias orifices -normal

Palpation

No local rise of temperature

Abdomen soft 

No guarding and rigidity

All inspection findings confirmed

Lower border of liver not palpable 

Spleen not palpable 

Kidneys- bimanually palpable, ballotable

Fluid thrill absent

Abdominal girth -92cms

Spino umbilical distance - 

.                           right-  46cm  left-46cm

Xiphisternum to umbilucus -16cms

Pubic symphisis to umbilicus-11cms

Per rectal examination- no mass felt , no blood staining ,hard pellets+

Hernias orifices -normal 

Percussion:

Shifting dullness +

Liver span - 11cm

Traubes space - resonance

Auscultation:

Bowel sounds +( 6 per minute)

No hepatic bruit , no venous hum

Examination of scrotum: No testicular atrophy

Examination of spine -  normal 

CENTRAL NERVOUS SYSTEM :

Higher mental functions:

 Patient is drowsy , non cooperative

Not oriented to time ,place ,person

Speech - aphasia

MMSE - Cannot be done

Kirby's method : 

General reaction and posture :

Patient is drowsy ,apathetic 

Patient is not responding and moving when placed in awkward position.

Eyes and pupils : 

Eyes open with decreased blinking

Reaction to examiners questions and tests:

Not responding to commands

Cranial nerves - intact 

Motor examination: 

Attitude  - lying over bed with knees flexed 

Bulk -no apparent wasting present 

Tone -                    right.                    Left 

           UL.             Hypo.                 Hypo

           LL.              Hypo.                Hypo

Power-

           UL.            3/5.                      3/5

           LL.            3/5.                      3/5

Reflexes-     

     Biceps.          ++                        ++

   Triceps.          ++                        ++

Supinator.         +                           +

        Knee.          -                            -

      Ankle.           -                            -

Superficial reflexes 

Corneal.           +                             +

Conjunctival.   +                             +

Plantars.         Flexion.                Flexion

Abdominal.       +   

Cremastric.        +     

Perianal.             +  

Sensory examination:

Spino thalamic : 

Crude touch:    +                         +

Pain.                  +                          +

 temperature.   Cannot be examined

Posterior column : cannot be examined 

Fine touch.          -

Vibration.            - 

Joint position.    -

Rombergs.          -

Cortical : Cannot be elicited 

Two point discrimination    -

Tactile localization.             -

Stereognosis.                       -

Graphesthesia.                    -

Gait : cannot be examined 

Spine : No bony tenderness no kyphosis ,scoliosis

Cranium - no bony deformities

Peripheral nerves- no thickened nerves, no foot drop ,no wrist drop

Other systems : 

CARDIOVASCULAR SYSTEM:

 S1S2 heard

No murmers 

Apex -normal 

Respiratory system:

Inspection:-
Upper respiratory tract - oral cavity, nose & oropharynx appears normal. 
 Rules tube in place .
Chest appears Bilaterally symmetrical & elliptical in shape
Respiratory movements decreased on right Infra Mammary and right infrascapupar region.
Trachea central in position 
 Nipples are in 4th Intercoastal space
No signs of volume loss
No dilated veins, scars, sinuses, visible pulsations. 

Palpation:-
All inspection findings confirmed
No local rise of temperature. 
Trachea central in position
Apical impulse in left 5th ICS, 1cm medial to mid clavicular line.
MEASUREMENTS-
chest circumference- 
82cms at expiration 
85.5 cms at  inspiration
Chest expansion- 3.5cm
                                         Right                   left
Hemithorax-              41cms                  41cms

Hemithorax expansion-  1.5cms           2cms

AP diameter-                 20 cms 

Transverse diameter-   35cms

AP/Transverse ratio - 0.57

Percussion:-
                                       Right                     left

Supraclavicular-    Resonant              resonant

Infraclavicular-      Resonant.           Resonant

Mammary-              Resonant            Resonant

Inframammary.      Stony Dull.         Resonant 

Axillary-                   Resonant             Resonant

Infra axillary-           stony dull           Resonant

Suprascapular-         Resonant           Resonant

Interscapular-          Resonant            Resonant

Infrascapular-            stony Dull         Resonant

Auscultation:-

                                      Right                     Left

Supraclavicular-       NVBS.               NVBS 

Infraclavicular-         NVBS                NVBS

Mammary-                 NVBS                 NVBS

Infra Mammary-       decreased           NVBS

Axillary-                      NVBS               NVBS

Infra axillary-             Decreased.       NVBS                                           
Suprascapular-          NVBS             NVBS

Interscapular-            NVBS               NVBS

Infrascapular-         decreased         NVBS
                                   

Provisional Diagnosis 

1.Decompensated chronic liver disease with cirrhosis secondary to alcohol with stage 3 hepatic encephalopathy without portal hypertension and with moderate ascites and no signs of spontaneous bacterial peritonitis.

2. Anemia of chronic disease 

3. right sided pleural effusion secondary to hydrothorax or hypoalbumunemia .

Based on history and examination we advised following  investigations

Hemogram : 

Hemoglobin-9.9 gm/dl

Total counts - 8500 cells/cumm

Neutrophils-74%

Lymphocyte-10%

Platelets- 1.79 lakhs/cumm

Peripheral smear - NC/NC Anemia

Complete urine examination:

Colour -pale yellow 

Albumin nil

Pus cells nil

RBC - nil

Renal function tests :

Urea - 73mg/dl

Creatinine - 1.2mg/dl

Uric acid - 8mg/dl

Calcium - 10mg/dl

Phosphorus- 7mg/dl

Sodium - 128 mEq/l

Potassium - 3.8mEq/l

Chloride- 92mEq/l

LFT :

Total bilirubin -1.82

Direct bilirubin- 0.48

AST- 20

ALT- 10 

ALP- 207

Total proteins -6.1

Albumin -2.2

A/G ratio - 0.51

PT -16 seconds

INR-1.11

aPTT- 32 seconds 

Blood group - O positive 

ÀBG :

PH : 7.44 

PCO2 :34.2 mmHg

PO2:      89mmHg

HCO3 :  24.2mmol/L

BEB : -0.2mmol/L

O2 sat :96.% 

USG abdomen : mild ascites

ECG 

Chest x-ray:


MRI BRAIN - NORMAL

Discussion

What is hepatic encephalopathy? 

Hepatic encephalopathy is the term used to describe the complex and variable changes in neuropsychiatric status which complicate liver disease. This syndrome is the defining feature of fulminant hepatic failure and, in this setting, is only one of a multitude of metabolic abnormalities caused by loss of functioning hepatocyte mass.

Precipitating factors :
1. Infections
2. Gastrointestinal Bleeds(GI Bleeds)
3. Dyselectrolytemia –Hyponatremia and Hypokalaemia
4. Dehydration – Fluid Restriction, Diarrhea/Vomiting, Excessive Diuresis
And Paracentesis
5. Constipation
6. High Protein Diet 

Guideline Based Management of Hepatic Encephalopathy
1. Primary Intervention – 
  Correct or Eliminate the Precipitating Factor.
2. Dietary Advice
     a. Dietary restriction of Protein is NOT       RECOMMENDED. Conclusively demonstrated that limiting protein-calorie intake is not of benefit in patients with HE.
The recommended daily dietary allowance is 60-80g.
     b.Vegetable and Dairy protein is recommended over animal protein because of a favourable calorie-to-nitrogen ratio.
3. BCAA (Branched Chain Amino Acids) supplementation has modest efficacy and it’s routine use is not recommended.
Medical Management
1. Non-Absorbable Disaccharides
Lactulose , Lactitol -  First line agents; Symptomatic improvement only, no improvement in Psychometric test performance 
Goal of Therapy – 2 to 3 Soft Stools per day.
2. Oral Antibiotics – Rifaximin 550 mg BD. Other Drugs – Neomycin, Metronidazole and Vancomycin.
3. Acarbose – An Intestinal α-Glucosidase Inhibitor 
 •Used in patients with cirrhosis and adult-onset Diabetes Mellitus
• Good fasting and post-prandial glucose control
•Lowered HbA1c (and post-prandial C-peptide levels)
• No alteration in biochemical parameters of Liver function.
4. Other Drugs (requiring conclusive evidence for use in clinical practise)
•Sodium benzoate, Sodium Phenylbutyrate and Sodium Phenylacetate (Urea cycle defects), Zinc supplementation,
Extracorporeal Albumin Dialysis using MARS (Molecular Adsorbent Recirculating System)
5. L-Ornithine-L-Aspartate (LOLA) 
•Significant decrease in Ammonia
Levels (Independent Predictor of
Survival)
• Improvement in Mental Status
• Better performance in
Psychometric tests
• Improved EEG Activity

Rifaximin vs placebo treatment in hepatic encephalopathy:

Polyethylene glycol vs lactulose in hepatic encephalopathy:


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SHORT CASE- 1:


History given by patient and her husband and is reliable


 A 45year old female, right handed ,housewife, resident of Nalgonda  presented with 

chief complaints of 

-headache since 2years 

-Bilateral Knee,ankle,elbow joint pains since 6months 

-weakness of right upper and lower limbs since 30days

-deviation of mouth to left since 30days

-Double vision since 10days

History of present illness:

Patient was apparently alright 2years back ,then she developed headache-acute in onset, Intermittent ,throbbing type,diffuse and bilateral .Not associated with nausea ,vomitings,photophobia,phonophobia, laceration,blurring of vision. 

Complaints of bilateral Knee,ankle,elbow joint pains since 6months -not associated with fever,restriction of movements,early morning stiffness,swelling of joints -relieved by taking analgesics and aggrevated on exertion. 

Complaints of weakness of right upper and lower limb since 1month -acute in onset and gradually progressive - initially she had mild symptoms ,that gradually progressed over  1 month to current status .Initially she used to walk  alone till the bathroom With some difficulty ,later patient found difficulty in walking without support and patient felt more giddiness while walking.

Complaints of mild deviation of mouth to left side , not associated with drooling of saliva 

Complaints of Double vision since 1month- intermittent ,horizontal and binocular ,no history of black spots,colored halos,floaters,blurring of vision.

No history of tingling and numbness

No history of difficulty in perceiving hot and cold sensation while bathing.

No history of perceiving band link sensation/girdle like sensation.

No history of electric shock like sensation.

No history of sensation of walking on cotton wool.

No history of washbasin attacks.

No history of loss of consciousness. 

No history of memory loss.

No history of delusions ,hallucinations,behavioral disturbances. 

No history of alteration in smell.

No history of blurring of vision /able to differentiate colors.

No history of drooping of eyelids.

No history of loss of sensation over face.

No history of difficulty in closing eyes,lips

Able to perceive taste sensation.

No history of hard of hearing ,ringing sensation in ears.

No history of regurgitation of food and fluids.

No history of difficulty in swallowing foods/nasal twang in speech.

No history of difficulty in moving neck in all directions, shrugging the shoulders.

No history of difficulty in pushing the food backwards,able to roll the tongue and clear the food.

No history of giddiness after getting up from bed.

No history of excessive sweating.

No history of Bowel and Bladder disturbances .

No history of recurrent infections. 

No history of ear discharge,fever,neck stiffness,weightloss.

No history of drug intake.

No history of trauma and head injury.

Past history:

Non Diabetic,Non Hypertensive, No history of Asthma,CAD,Epilepsy.
She underwent hysterectomy 20 years back.

Family history :

She was married at the age of 18 years ,3rd degree consanguinity 

1 st child- spontaneous vaginal delivery - 25 yrs old
2 nd child-vaginal delivery, died at 1 month of age due to unknown reason
3rd child - died at the age of 21 due to kidney failure

No history of similar complaints in family.

Personal history:

Mixed diet

Sleep adequate

Bowel and Bladder habits regular

No addictions

Socio economic status - lower middle class

Menstrual history:

Age of menarche : 15 years ,regular cycles-5/30 ,no dysmenorrhoea 

Drug history :

Patient was on 
T.ECOSPIRIN 75mg/PO/OD
T.ATORVAS 20mg/PO/HS

Summary:A 45 year old right handed female patient, who is non hypertensive and non Diabetic with no addictions  presented with headache since 2years  and progressive weakness of right upper and lower limb since 1month  and double vision double since 10days.
Differential Diagnosis: 
 1.Cerebro vascular accident involving mid brain and Pons 
2. Basilar artery occlusion 
3.Demyelinating disorders
General examination:

Pt conscious,coherent ,cooperative 

oriented to time ,place and person .

 Moderately built and nourished .

BMI: 24.6kg/m²

Vitals :

Pulse :

82beats per minute , regular, normal volume ,vessel wall normal ,no radio-radial delay ,no radio femoral delay, all peripheral pulses felt.

Blood pressure:

right arm -120/80mmHg ,supine position 

Left arm -120/80mmHg ,supine position 

Right leg - 130/80mmHg 

Left leg -130/80mmHg 

Respiratory rate- 16cycles per minute, thoracoabdominal ,no usage of accessory muscles.

Temperature - 98.2F

SpO2-98% at room air

JVP - not elevated

Physical examination

No Pallor, icterus, cyanosis, clubbing, generalized lymphadenopathy, edema.

Systemic examination:

Patient is conscious,coherent and cooperative 

Right handed person

MMSE - 30/30

Nystagmus -absent

Speech - spontaneous with intact naming ,repetition,fluency.

Spine -normal

Cranium -normal

gait -hemiplegic gait

Cranial nerves -               right.           Left 

1.Sense of smell -        normal.         Normal

2.Visual acuity -           normal.         Normal

 Field of vision-            normal.         Normal

colour vision.              Normal.         Normal

fundus.                         Normal.         Normal

3,4,6 :   extra ocular movements:

 -restriction in adduction elevation,depression on right side  and normal on left side

-pupils: Normal size and reacting to light on both sides

-direct and consensual light refleces normal in both eyes

No Nystagmus 

no ptosis 

5. Sensory : sensations over face  normal on both sides

Motor - massager,temporarily, pterygoids normal

7.motor : loss of nasolabial fold on right . Orbicularis ocular, orbicularis Oris ,occipital frontalis, buccinator -normal on both sides

 Sensory : taste over anterior 2/3rd of tongue normal on both sides

8.rinnes test normal on both sides 

. Webers test normal on both sides

9.10.uvula , palatal arch movements  normal.

         Gag reflex.      -      normal 

         Palatial reflex -      normal

11.tarpezium and sternocleidomastoid -normal

12. No  wasting and fasciculations 

        Tongue protrusion to midline.

Motor system examination : 

1.Bulk (nutrition):         right.             Left

Inspection.              Normal.         Normal

Measurements :U/L  26/22cm      26/22cm

                            L/L. 46/ 34cm.     46/34cm

2.Tone :        U/L       hypotonia.        Normal

                      L/L.      Hyportonia.        Normal

3.power: 

Neck muscles.         5/5.                  5/5

upperlimb:

Shoulder-                -4/5.                  5/5

Elbow -                   -4/5.                   5/5

Wrist -                    -4/5.                    5/5

Handgrip-               50%                    100%

Lower limb: 

Hip -                       3/5.                     5/5

Knee-                     3/5.                      5/5

Ankle -                   3/5.                      5/5

Trunk muscles-              normal. 

Deep tendon Reflexes -   right             left

Biceps                                 +++            +++

Triceps                                +++             +++

Supinator                             ++              ++           

Knee.                                   +++              +++     

Ankle.                                  +++             +++        

Jaw jerk.                       Present 

Superficial reflexes -

Corneal.                              +                      +

Conjunctival.                       +                      +

Pharyngeal.                         +                      +

Palatal.                                +                      +

Abdominal.                          +                      +

Cremastric.                         +                       +

Plantar.                    Extensor             extensor 

Sensory system examination:

Spinothalamic:   right.           Left

Crude touch -     normal.     Normal

Pain-                    normal.      Normal

Temperature-    normal.      Normal

Posterior column

Vibration sense-         normal                Normal

Fine touch -                 normal.              Normal 

Position sense -          normal.              Normal

Cortical senses

2point descrimination- normal.           Normal

Tactile localization -     normal             normal 

Stereognosis   -             normal.           Normal 

Graphesthesia -             normal.           Normal

Cerebellar examination: right                  left 

Finger nose  test -        normal.            Normal

Finger nose finger test- normal.          normal

Disdiadokinesia   -          no                     no 

Heel knee test -            normal.           normal

Tandom walking. -     could not be performed

Rombergs sign -        could not be performed

Gait examination-    hemiplegic gait

Spine examination- normal

Peripheral nerves - no nerve thickening,no foot or wrist drop . 

Here are some videos of her CNS examination







Other systems 

CVS : S1S2 + , No murmers , Apex normal 

Respiratory system: Normal vesicular breath sounds +, no added breath sounds.

GIT : No abnormalities +

Provisional Diagnosis :
Sudden onset right sided hemiparesis which is gradually progressive , with right  UMN type of Facial palsy and 3rd nerve involvement  due to demyelinating/inflammatory etiology involving midbrain and Pons  .

With the given history and examination we evaluated her further 
MRI BRAIN (Plain and contrast )with Angiogram was done that showed 
                


 T2 hyperintensities noted along short segment of  Cervical cord

 T2 FLAIR  showing hyperintensity right internal capsule


 T2 FLAIR  image showing hyperintensities in midbrain,bilateral thalami

T2 weighted Transverse section MRI BRAIN showing hyperintensity involvement of Midbrain sparing Red Nucleus .

Carotid Artery Doppler: 
  Soft Plaque in left carotid artery without significant stenosis.
ECG  

2D echo : normal 

Chest x-ray PA view:

Treatment:  
 1.Iv methyl prednisolone 1gm /IV was started and continued for 5 days
2.Tab.Ecospirin Av(75/20 mg) /po/OD
3. Supportive treatment

After IV Methylprednisolone Patient improved symptomatically
And objectively her power improved in upper limbs to 4/5 and lower limbs - initially from 2/5 to 4/5 over period of 2-3 days.
         This video is taken after Receiving high dose of steriods , Patient was able to walk alone for some distance ,with some difficulty .But there is significant improvement in Power going by objective evidence of muscle power.
 

LUMBAR PUNCTURE : 
Lumbar puncture was done  ,
and CSF analysis was sent .
Colur - colorless
Appearance- clear
Total counts - 16cells /cumm
Lymphocytes-100%
Neutrophils - nil
CSF glucose -94mg/dl
Protein- 79mg/dl
Chloride -114mmol/lit
High protien and lymphocyte predominant - suggestive of inflammatory process


Anti NMO antibody levels :  Serum Aquaporin levels were sent  and came negative 
                             
Patient was discharged and continuously followed up.
Patient improved symptomatically and is able to walk alone and able to perform her own activities .
Review MRI was done to look for any new lesions.Mri showed regression of hyperintense lesions .








Discussion:  
1.https://academic.oup.com/brain/article/122/11/2171/377380




1.   https://www.ncbi.nlm.nih.gov/pmc/articles/PMC490512/
This shows only few percent of people show Neurologic symptoms prior to systemic manifestations, and most commonly they develop within 6months - 1 year ,( range 6 months -3years ) 

On the other hand, Ikedat7 stressed that the common neurologic features of neuro- Behqet's syndrome were motor impairment especially bilateral pyramidal signs and that the mental changes mainly consisted of loss of emotional control with relative sparing of intelligence and memory.


We performed a systematic review and meta-analysis of studies on neurosarcoidosis published between 1980 and 2016

We identified 29 articles describing 1088 patients diagnosed between 1965 and 2015. Neurosarcoidosis occurred in 5% of patients with systemic sarcoidosis. Mean age at presentation was 43 years and neurological symptoms were the first clinical manifestation of sarcoidosis in 52%. The most commonly reported feature of neurosarcoidosis was cranial neuropathy in 55%, with the facial and optic nerve most commonly affected, followed by headache in 32%. Pleiocytosis and elevated CSF protein were found in 58 and 63%. MRI of the brain showed abnormalities in 70%. Chest X-ray, chest CT, or gallium-67-scintigraphy showed findings consistent with sarcoidosis in 60%, 70% and 69%, respectively.

3. Isolated Neuro sarcoidosis: 


 In all patients, no extranueral sarcoidosis developed during a relatively long follow-up period (mean 58 mo). Compared with the systemic neurosarcoidosis cohort (60), isolated neurosarcoidosispatients had similar demographics and neurological manifestations with a few exceptions including a more common frequency of headache, hemiparesis, and radiculopathy, leptomeningeal involvement on brain MRI, increased cell count in cerebrospinal fluid, and a more favorable clinical outcome (P<0.05).

4: Isolated neurosarcoidosis presenting as meningitis  


The exact etiology of neurosarcoidosis is unknown and multifactorial, involving genetic predisposition and individual and environmental factors  
Exposure to mildew, musty odors, pesticides, and agricultural employment have been associated with the development of sarcoidosis 

Clinical manifestations of neurosarcoidosis can be found in 5–20% of cases of systemic sarcoidosis, and these symptoms can be mild or severe 5 (Table 2). About half of patients with neurosarcoidosis can present with neurologic manifestations sooner than systemic sarcoidosis is apparent
                     

5: https://sarcoidosisnews.com/2016/07/19/isolated-neurosarcoidosis-difficult-to-diagnose-but-easy-to-treat/


6: https://www.hindawi.com/journals/pri/2012/871019/

Articular involvement is characterized by nonerosive and nondeforming arthritis which often presents with monoarticular pattern, although asymmetrical polyarthritis can occur. The articular involvement is usually transient in nature with episodes lasting from a few days to weeks
Parenchymal involvement including brainstem involvement, hemispheric manifestations, spinal cord lesions, and meningoencephalitis is seen in the majority of patients (%80)

Table 6: Summary of evidence-based algorithmic therapy for Neuro-Behc ̧ et’s disease.

1st line.     Corticosteroids
2nd line      Azathioprine, cyclophosphamide, Anti-TNF-α, IFN-α
3rd line       Methotrexate, Anticoagulation

In parenchymal involvement, corticosteroids (100 mg/d or 1 gx 5 days as pulse treatment) should be the first choice. Azathioprine is usually com- bined with corticosteroids. In severe or unresponsive cases, cyclophosphamide can be given additionally [83]. Anti-TNF- α agents and IFN-α are other new effective alternative agents [19]. Methotrexate is another treatment alternative [67, 68].

7. https://www.sciencedirect.com/science/article/pii/B9780702040887001103
Nervous system involvement, known as “neuro-BS” (NBS), is seen in about 5–10% of all cases. Clinical and imaging evidence suggests that primary neurologic involvement in BS may be subclassified into two major forms: the first, which is seen in the majority of patients, may be characterized as a vascular-inflammatory central nervous system disease with focal or multifocal parenchymal involvement, mostly presenting with a subacute brainstem syndrome and hemiparesis (intra-axial NBS); the other, which has few symptoms and a better neurologic prognosis, may be caused by isolated cerebral venous sinus thrombosis and intracranial hypertension(extra-axial NBS), occurring in 10–20% of the cases. 

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SHORT CASE- 2:

History taken from patient and his wife and is reliable
A 45 year old male , right handed , resident of buggilapuram , daily labourer presented with 
chief complaints of 
- fever since 10days 
-difficulty in breathing since 10days
-decreased appetite since 10days 
-burning micturition since 10days
History of present illness : 
-Patient was apparently alright 10days back, then he had fever - high grade , continuous , associated with chills,relieved on medication

 - Complaints of difficulty in breathing since 10days acute in onset , gradually progressed from grade 2 to grade 3 over 10days .       
 -Not associated with cough, chestpain, palpitations .Associated with orthopnea.
 Not associated with PND . 
-Complaints of decreased appetite since 10days. 
-Complaints of burning micturition since 10days. 
History of antibiotics 10days back for fever. 
No history of hematuria, decreased urine output , pedal edema , facial puffiness.
No history of vomitings , loose stools , constipation.
No history of pain abdomen .
No history of joint pains or bone pains.
No history pruritis .
No history of melena , hematemesis. 
No history of analgesic abuse .
Past illness  :
 History of similar complaints of fever , burning micturition 1year back and was diagnosed as having AKI and started him on hemodialysis - 5sessions of hemodialysis done and 3 PRBC transfusions done . 
Not a k/c/o Hypertension, Diabetes , epilepsy,asthma,Tuberculosis,thyroid disorder, CAD. 
Family history: 
No similar complaints in family.
Personal history :  
Married 
Mixed diet 
Appetite -decreased since 10days 
Bowel and Bladder habits - regular 
Alcoholic - since 30yrs , daily (1beer (12gm of alcohol) and toddy)
Non smoker 
Habit of beetle nut chewing since 30yrs.
Drug history : He is taking 
T.SHELCAL 500mg /PO/OD
T.NODOSIS 500mg /PO/OD
T.OROFER -XT /PO/bid since 1year.
Summary : 
A 45 year old male patient   who is a chronic alcoholic ,  non hypertensive and non Diabetic presented with fever , difficulty in breathing , burning micturition since 10days .
Differential Diagnosis
1.Acute kidney injury on  chronic kidney disease 
2.AKI  causing Acute interstitial nephritis secondary to drug induced 
3.AKI secondary to Acute interstitial nephritis due to infection.

General examination : 
Moderately built and nourished

Patient is conscious,coherent and cooperative.

GCS: E4V5M6

BMI: 24.8kg/m²

Vitals :

Pulse -

95beats per minute , regular, normal volume ,vessel wall normal ,no radio-radial delay ,no radio femoral delay, all peripheral pulses felt.

Blood pressure:

right arm -130/70mmHg ,supine position 

Left arm -130/70mmHg ,supine position 

Right leg - 130/80mmHg 

Left leg -130/80mmHg 

Respiratory rate- 24cycles per minute, abdominothoracic ,no usage of accessory muscles.

Temperature - 98.2F

SpO2-98% at room air

JVP - not elevated

Physical examination

Oral cavity -staining of teeth with tobacco

Pallor +

No Icterus

No cyanosis

No clubbing 

No generalized lymphadenopathy

No Pedal edema






Systemic examination

CVS :

S1 and S2 heard 

No murmers 

Apical impulse in left 5th intercoastal space in mid clavicle line.

No thrills , parasternal heave .

Respiratory system

Normal vesicular breath sounds present

No adventitious breath sounds heard

Gastrointestinal system :

Abdomen : 

scaphoid abdomen 

No visible scars, sinuses and peristalsis

Umbilicus- central , circular .

Palpation - soft , non tender , no oraganomegaly

Auscultation : Bowel sounds + 6 per minute 

No audible bruit

Percussion: tympanic note

Central nervous system

Conscious , coherent and cooperative 

Speech - spontaneous , naming, repetition, fluency normal

Normal gait

Cranial nerves intact. 

Sensory system,motor system  autonomic system- normal

Provisional diagnosis: 

1.Acute kidney injury(AKI) on chronic kidney disease (CKD)secondary to Acute interstitial nephritis due to drug induced or infection , without features of uremia

2. severe Anemia 

Investigations:

Hemogram:

Hemoglobin-3gm/dl

Total counts - 16200 cells/cumm

Neutrophils-90%

Lymphocyte-05%

Platelets- 2.08 lakhs/cumm

Peripheral smear - NC/NC Anemia

Blood group - A positive 

Complete urine examination:

Colour -pale yellow 

Albumin +

Pus cells 3-4 /HPF

RBC - nil

Renal function tests :

Urea - 468mg/dl

Creatinine - 25.9mg/dl

Uric acid - 16.4mg/dl

Calcium - 10mg/dl

Phosphorus- 11.3mg/dl

Sodium - 132mEq/l

Potassium - 5.3mEq/l

Chloride- 92mEq/l

ÀBG :

PH : 7.32 

PCO2 :5.5 mmHg

PO2:      113mmHg

HCO3 :  2.8mmol/L

BEB : -24.3mmol/L

O2 sat :96.1% 

USG abdomen : 

Kidneys- 

Right and left - normal size and increased echo texture.

 B/l grade 2 RPD changes 

ECG :

Chest xray : 


Treatment: 
1. started him on hemodialysis 
2.2 PRBC transfusions
3.inj ERYTHROPOIETIN 4000IU/sc twice weekly
4.T.OROFER-Xt /PO/BD
T.SHELCAL 500MG /PO/OD

Commen

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