"The Darting Tongue in Sydenham's Chorea."

 Hello everyone, This is R Preethi  an intern in the medicine department,  An internship is a hands -on way to learn new skills. Completing an internship can expose you to the workplace, letting you know what really goes on . One of the important terms of getting the internship  completion  is to complete my log book by documenting the cases I saw, along with my daily log . 

The case  presentation:

 A 17 year old lady from jaggayapet, presented with a 1 week history of "shaking". Hailing from an impoverished family,  she was apparently asymptomatic 2 years ago, when she first developed  wheezing and breathlessness on a cold winter night at 1am which woke her up from her sleep. The mother reported that it resolved spontaneously over a short period of time . At this point ,she denied a history  of sweating or palpitations. 

She also  denied a prior history of chest tightness and exertional dyspnea.  

5 days later she  started developing cough and breathlessness with very little sputum. 

The cough was mostly dry, present throughout  the day and increased when she was lying down. 

Her breathlessness too started at around the same time and quickly progressed to a point limiting her functional capacity significantly,  which made them consult a local medical practitioner. 

The mother says that the practitioner suspected a serious  lung problem and referred them to a higher center.

They immediately went to a higher  center where a CXR PA  view was taken  which was diagnosed as "nimmu" ( local lingo for Pneumonia or a lung consolidation). 

At this point ,her sputum was sent for labs ,in suspicion of TB or a fungal infection.  She tested negative for HIV ,HCV ,Hbs Ag. 

She was started on empirical antibiotics  and a short course of steroid therapy was initiated at this point. 

Her symptoms remained unabated and were limiting her functional capacity  to a point where she couldn't continue her studies anymore.

Her sputum tested negative AFB and CBNAAT but was positive for fungal elements on KOH staining. However, she was  initiated on Cat1ATT based on a clinical suspicion of a thick walled cavity lesion in the right upper lobe .

She took the prescribed ATT  for 6 full months and reported resolution for her symptoms ( serial chest x-rays  were  done - images are shared below.). With  this disease rendering her unable to complete school on time , the patient was disappointed that she lagged behind , while her friends had progressed.

 With an increased resolve to finish school, she worked even harder and finally finished her school. Her quality of life was  gradually returning  and she entered Intermediate 1 st year in Bipc . 

On 28 th of June  2020 she started noticing that her right upper limb started having semi voluntary to involuntary violent movements,  which progressed over 2 days to her entire body , including her head movements. Her speech also became erratic and jerky like her limb movements. Those movements are present throughout the day, with a high amplitude of movements, erratic and  non rhythmic, present at rest and during action. She is able to walk but has some gait instability. She has not reported any stiffness or rigidity. She also frequently brings out and takes back her tongue associated with sucking movement of her lips. She has no history of weakness or giddiness. Since then she had great difficulty in consuming food and water, difficulty in dressing and undressing herself, communication has become a tedious task and she simply has been unable to catch any sleep over the past 5 days . However,  in the off chance she catches some sleep ,her movements abate and she only has minimal movement of  her feet occasionally during her sleep.  The mother also reported that she had been having painless, initially itchy, pale and papular lesions on her hands and feet. She says, they became non itchy over a period of time and have remained stable for the past 1 year. She has also noticed a painless bump on her little fingers of both hands.

On psychiatry referral : no hearing of voices, suspiciousness, pervasive mood changes, irrational fears.

No significant psychiatric history in the past and family.

ETEC not maintained 

PMA increased 

Speech : verbal,tone, rhythm normal

Mood, Thought , perception  no abnormality detected.

They prescribed INJ .PHENERGAN 12.5 MG IM

                             INJ. DIAZEPAM    10 MG/IM/ STAT.

She is the eldest of 2 children to a widower, after her father died in a fatal bus crash in 2013 . He was the school bus driver for  Zilla Parishad Hih School. They live in a single large room with a washroom attached. There is a cement factory close by and several weeds and shrubs beside their house. Her mother is the sole  breadwinner and earns 6k rupees for month. The patient has a steely resolve to successfully complete  her education and stand on her feet by herself .

 

PAST HISTORY :

No past history of such movement disorders. 

No past history of fever but she reports a history of multiple large joint pains without any limitation of movements.

No past history of ataxia , giddiness, fatigue , jaundice. 

No past history of seizures.

DRUG HISTORY:

Apart from ATT for 6 months,  she has used IV and inhalational steroids quite frequently over the past 1 year . 

She also recently received a 10 day course of Cefpodoxime. 

She never used OCP's or Antiparkinsonian drugs. 

FAMILY HISTORY:  

Her mother also reports a history of Atopy on exposure to cold climates, wet grass and moldy hay.

PERSONAL HISTORY: 

Diet: mixed

Appetite is decreased since 15days.

Sleep  disturbances since 1 week.

No bowel and bladder disturbances.

No addictions. 

GENERAL EXAMINATION:

Patient is conscious, coherent , cooperative , well built and well nourished. 

VITALS: 

BP:120/80 mm of Hg measured in the right arm in the sitting position .

PR:130 bpm , her PR has been consistently high and during sleep:115 bpm.

Temperature: 97F

Spo2 varying between 91 to 99 on room air.

GRBS 75mg/dl

No signs of pallor, icterus,cyanosis, clubbing,lymphadenopathy,edema or any obvious rash or neurocutaneous markers.

 

Respiratory system examination - Chest expansion was equal on both sides but it was reduced in the right suprascapular area. Percussion revealed a subtympanitic / impaired note in the right anterior lung fields and the right suprascapular area. Auscultating the lungs proved extreme difficult, however bronchovesicular breathingwas heard.

Per abdomen examination: abdomen scaphoid,soft  , non tender, no local rise of temperature, no organomegaly, no visible pulsations, hernial orifices free, bowel sounds are heard.

CVS EXAMINATION: Touch and go with the heave but RV pulsations present.

Palpable heart sounds in pulmonic area and tricuspid area. 

Loud S1 with a loud and narrow S2 were audible. No murmurs rubs  or gallops.

CNS  EXAMINATION: 

MMSE: 19/30 - Moderate MR.

Higher mental functions - conscious, coherent and oriented to time, place, person.

Memory intact short term and long term.

Recall intact 

Calculation and attention 3/5.

Language-  Comprehension of verbal and visual commands intact. Fluency and latency impaired. Naming and repetition intact. Dysarthria with erratic and monotonic speech.

Gait - Difficulty to walk alone, needs an assistant but improved after the drug therapy walking alone ( video shared below).

Cranial nerves intact 

No meningeal signs . 

Motor system           Right    Left ( in cms)

              Bulk    UL      26        25 

                          LL      28        28

              Tone    UL      N           N

                           LL      N           N 

 Power couldn't be elicited. 

REFLEXES  

Biceps,  Triceps,Supinator, Knee, Ankle ,Plantar : present.

SENSORY SYSTEM

              Pain       

             Touch                  :       N        N

            Temperature

INVESTIGATIONS:

  HB: 10.2 g/dl 

  TLC 7700

   HCT 29.6

   PLC 1.2 

   RBC 4.01          

Ph:    7.3

Pco2 :40.6

PO2: 35.7

O2 sat : 54.8

Hco3:23.6

St.Hco3: 22.9

HIV ,HbsAg,HCV } negative .

Serum.Mg 2+: 2.3

T3 :0.99

T4 :14.46

TSH :0.56      

RFT

Urea :31

Creatinine: 0.4 

Calcium : 10.2

Phosphate: 3.3

Uric acid :5.3

Sodium :139

Potassium: 3.5 

Chlorine :10.3 

LFT

Total bilirubin: 1.93

Direct bilirubin:  0.30

AST: 38

ALT: 21

ALP :150

Total protein :6.6

Albumin :3.8 

A/G ratio: 1.37

ECHO REPORT 

 OS ASD (+) Size 1.3cms(left to right shunt) 

?RA clot present 

Good LV function  (+)

No RWMA

Mild TR, Moderate to Severe PAH with RVSP 65 mm Hg.

No MR/AR

No PE and LV  clot

Rheumatic myocarditis

Posterior mitral leaflet prolapse

Myocardial asynchrony 

 

DIAGNOSIS 

ACUTE RHEUMATIC FEVER-SYDENHAM'S CHOREA AND MODERATE COGNITIVE IMPAIRMENT WITH RHEUMATIC CARDITIS

?ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS /?PULMONARY KOCH'S (TREATMENT COMPLETED FOR 6MONTHS)-RIGHT UPPER LOBE HEALED FIBROCAVITORY LESION WITH CENTRAL PROXIMAL AIRWAY BRONCHIECTASIS ASSOCIATED WITH LEFT PARIETAL LOBE HEALED GRANULOMA.

TREATMENT

INJ Monocef 1gm/ IV/ BD

INJ Pantop 40mg/IV/OD

TAB Tetrabenazine 25mg /PO/BD (after food)

Protein powder 2 tablespoons in one glass of milk/PO/TID

INJ Lorazepam 2cc/IV/SOS

BP/PR/Spo2 charting 4th hrly

GRBS charting 6th hrly

Advice at discharge 

TAB ENMOX 500mg/PO/BD after food for 14 days

TAB Naproxen 250mg/PO/BD after food for 2weeks

TAB Tetrabenzene 25mg/BD for 1month

TAB Pantop 40mg/PO/OD at 7am

INJ benzyl penicillin 1.2million units IM every four weeks for 5 years .