CME INDIA Presentation by Dr. Ashok Kumar, DM (Neurology), Neuron Clinic, Ranchi.

An Enchanting Encounter with Late Dr. Bidhan Chandra Roy

What happened 30 years ago was a wonder

  • Some 30 years ago (1993-94) during my Senior Residency as DM (Neurology) student – I saw a middle-aged patient in OPD. It was in SGPGIMS, Lucknow.
  • The patient was 56 years old Male from Bihar. He belonged to an old Jamindar family and was relatively well-off. He complained of “tremor” (कम्पन ) in the right hand for several decades.
  • He had these tremors first at 16 in 1953 -54. He consulted doctors at his native place and Patna. No Details or papers were available. Apparently local treatment did not satisfy him. He approached the then Bihar Chief Minister late S. K. Sinha (Shree Babu) and got a letter for Dr. B. C. Roy, then Chief Minister of West Bengal.

The Letter Blues

  • The letter was “From” CM, Bihar to Dr. B. C. Roy, CM West. It contained a single sentence.

 “Dear Sir, kindly lend the magic touch of your hand to this unfortunate boy.” The patient was able get immediate appointment from Dr. B. C. Roy. He examined him briefly and wrote a prescription. The prescription was in typical old doctor style, very difficult to read. But, two things were clear. First was the test, Protein-bound Iodine (PBI). Second was a medicine – Thyroid Extract (French Codex). The patient was advised to get this imported medicine from Dey’s Medical Stores, Calcutta.

  • He had kept it from Dey’s.
  • There was dramatic improvement in “tremor” after taking the Thyroid extract.
  • The medicine was discontinued after a few months. The patient remained symptom-free for several years.
  • In late 1960’s, he had recurrence of the same tremor. But Dr. B. C. Roy was not available to treat him. He had been consulting doctors in all big cities and institutions in India. But no one had a clue.

When I Examined

  • I examined the patient. General examination was unremarkable. So-called tremor was not a tremor – his Right had irregular, infrequent movements – occasionally slightly jerky and stiff posturing. As usual in Neurology l, the problem was how to describe these movements.
  • It was decided that we should first get an MRI Brain. Later, some SPECT will be planned. Plus, blood tests including Hematology, Autoimmune related tests, Thyroid, Biochemistry.
  • But the patient was not keen on this costly workup. He didn’t turn up next day.

Looking back to Neurology Text books

  • We looked up Neurology textbooks for Thyroid. Hyperthyroidism may cause Hyperkinetic movement disorders like Chorea, tremors etc. Hashimoto Thyroiditis had many neurological complications – Encephalopathy to movement disorders of all types. But, Hypothyroid was an exception. Some hypokinetic disorders e.g. Parkinsonism had been described. But, NONE of iconic textbooks (Aspen course, Jancovic, etc) have described chorea or choreoathetosis in Hypothyroid. Of course, everyone was aware of Myxedema Coma.
  • Some 2 decades later, I saw some case reports of movement disorders in Thyroid diseases with new ones in Hypothyroid patients. One case, reported from Thailand described unilateral upper limb ChoreoAthetoid movements.

Was it SREAT?

  • Hypothyroid-associated Hyperkinetic disorders may occur in the context of Hashimoto disease (Steroid-responsive Encephalopathy with Autoimmune Thyroiditis, SREAT) — tremor, Myoclonus and Ataxia.
  • Picture here was not like SREAT.
  • Some Hypothyroid patients may develop Dyskinesia after taking certain Neuroleptics (Sulpiride ) and Baclofen . Our patient didn’t give history of neuroleptics.
  • Myoclonus and Encephalopathy have a reported in Hypothyroid patients who had discontinued Levothyroxine.
  • Rare Genetic diseases have been described with combination of Hypothyroidism and Chorea-dyskinesia. One such is Brain-Lung-Thyroid Syndrome (Benign Hereditary Chorea, BHC) due to mutation in NKX-1 gene. Children have Delayed milestones, Ataxia gait, Chorea, Dystonia and Tics. Comorbid Depression, Psychosis, ADHD and poor school performance occur. 2/3 develop Hypothyroidism. Later lung fibrosis may occur. But, none of these was present in the case described here.

It is mystery how Dr. B. C. Roy diagnosed?

  • It is mystery how Dr. B. C. Roy diagnosed and came to the treatment decision in this patient. What are the possibilities? One, he had experience of such a patient in practice. Very rare, but not impossible. He used to see referred, rare, unusual cases. Second, he might have some sign of Hypothyroidism in the patient.
  • But, one thing is almost sure. No one had described such a movement disorder in Hypothyroid before 1989.
  • संस्मरण एक तीर्थयात्रा है । (महादेवी वर्मा)

CME INDIA Comments

Dr. Shubhankar Chatterjee, DM (Std) ,Neuro,Kolkata (as per reference 1)

  • A patient with primary hypothyroidism presented with hemichoreoathetoid movement.
  • After a few weeks of thyroid hormone replacement therapy, the movement disorder significantly improved.
  • This abnormal movement is likely one of the neurological manifestations of hypothyroidism.

Dr. Ashok Kumar

  • Pure acute onset chorea without encephalopathy has rarely been reported in anti-thyroid peroxidase (anti-TPO) and anti-thyroglobulin (anti-TG) antibody-related neurologic disorders that respond to steroids (ATANDS).
  • ATANDS with associated movement disorders have been described previously. Ghosh et al have reported a 16-year-old female with ATANDS who presented with acute pure chorea without encephalopathy.
  • ATANDS presenting with chorea is exceedingly rare. For example, Miranda et al. described a middle-aged female with acute onset rapidly worsening choreo-athetosis with dystonia and slurred speech, which was identified as a case of ATANDS.
  • Sharan A et al. reported an elderly female with ATANDS who developed abrupt onset behavioral changes along with asymmetric florid chorea. Taurin G et al. narrated a case involving an elderly female who exhibited behavioral abnormalities with psychotic features and bilateral axial choreic movements.

What was Wonder in this Case?

  • Anti-TPO Antibodies are associated with several neurological problems. The most well-known is Hashimoto Encephalopathy (SREAT). But others are Tremors, Ataxia, Choreoathetosis, etc. These are known to respond to Corticosteroids. Thus, causative mechanism has little to do with hormone imbalance. We are reminded of Autoimmune Encephalitis due to NMDA -R antibodies and others.
  • The case here in question did not need Steroids. He responded to Thyroid extract (containing T4 and T3). This is not analogous to Autoimmune diseases. It was Hypothyroid-associated movement disorder. This is extremely rare. Many authors are probably even skeptical about its existence. Still, 70 years ago, someone “diagnosed” and treated such a condition successfully. That is the Wonder.

CME INDIA Learning Points

  • In autoimmune thyroid disorders, although known to affect only 1% of the population, focal or subclinical autoimmune thyroid inflammation can be found in around 15% of the biochemically euthyroid population. Anti-thyroid peroxidase (anti-TPO) and anti-thyroglobulin (anti-TG) antibodies are considered diagnostic markers of autoimmune thyroid disorders.
  • Neurological manifestations associated with autoimmune thyroid disorders have been frequently under-documented in the literature, with Hashimoto’s encephalopathy being the most protean among these disorders.
  • The spectrum of this disorder can range from subtle behavioral/personality changes to movement disorders, seizures, dementia, encephalopathy, stroke, coma, and death. Patients can also present with movement disorders without encephalopathy and cognitive impairment.
  • There are no pathognomonic clinical, serological, biochemical, electrophysiological, or imaging markers. Additionally, there are no good predictors of treatment response to steroids; in fact, in a recent study, only 31% of patients completely responded to these drugs. Similarly, in other studies, only 56% and 36% of patients with suspected Hashimoto’s encephalopathy responded to steroids. Despite this, response to steroids seems to be the only partially consistent feature of this disorder, hence the renaming as “steroid-responsive encephalopathy associated with autoimmune thyroiditis” (SREAT).
  • However, neither response to steroids nor association with thyroiditis is steadfast. Termasarasab et al. have recently proposed “anti-TPO/TG antibody-related neurologic disorders responsive to steroids (ATANDS)” to encompass the complete spectrum. Reported movement disorders associated with ATANDS can be either “encephalopathic” or “non-encephalopathic.”

CME INDIA Tail-Piece

  • Dr. Bidhan Chandra Roy:
    • Born on July 1, 1882, in Bankipore, Patna, in British India.Completed his undergraduate studies at Patna College.Pursued medical education at Calcutta Medical College and later went to the United Kingdom, where he earned his MRCP (Member of the Royal College of Physicians) and FRCS (Fellow of the Royal College of Surgeons) degrees.Renowned for his contributions to the medical field in India.Worked tirelessly to improve healthcare facilities and medical education in the country.Played a pivotal role in establishing several medical institutions, including the Indian Medical Association and the Medical Council of India.His contributions led to the foundation of institutions like the Indian Institute of Mental Health and the Infectious Disease Hospital.Active in Indian politics and the Indian National Congress.Served as the Chief Minister of West Bengal from 1948 to 1962.Worked towards the socio-economic development of West Bengal post-independence.Instrumental in the establishment of numerous industrial, educational, and healthcare institutions in West Bengal.Honored with the Bharat Ratna, India’s highest civilian award, in 1961.His birth anniversary, July 1, is celebrated as National Doctors’ Day in India to honor his contributions to the medical profession.
    • Left a lasting legacy in both the medical and political fields, remembered for his dedication to public service and healthcare.
  • Dr. Bidhan Chandra Roy’s life and work continue to inspire many in the fields of medicine and public service. His efforts laid the groundwork for the advancement of medical education and healthcare infrastructure in India.

References:

  1. Chotmongkol V, Bhuripanyo P. Movement disorder in hypothyroidism: a case report. J Med Assoc Thai. 1989 May;72(5):288-90. PMID: 2769124.
  2. Ghosh R, Chatterjee S, Dubey S, Pandit A, Ray BK, Benito-León J. Anti-Thyroid Peroxidase/Anti-Thyroglobulin Antibody-Related Neurologic Disorder Responsive to Steroids Presenting with Pure Acute Onset Chorea. Tremor Other Hyperkinet Mov (N Y). 2020 Jul 8;10:19. doi: 10.5334/tohm.175. PMID: 32775033; PMCID: PMC7394228.
  3. Sharan A, Sengupta S, Mukhopadhyay S, Ghosh B. Hashimoto’s Encephalopathy Presenting with Chorea. The Journal of the Association of Physicians of India. 2015; 63: 83–84. DOI: 10.4103/0028-3886.170064
  4. Termsarasab P, Pitakpatapee Y, Frucht SJ, Srivanitchapoom P. Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) Presenting with Pure Cerebellar Ataxia. Tremor Other Hyperkinet Mov (N Y). 2018 Aug 9;8:585. doi: 10.7916/D8CZ4QQQ. PMID: 30191089; PMCID: PMC6125737.


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