Saturday, 08.08.2020

Carlos Hojaij’s comment on Donald F. Klein, Max Fink, Edward Shorter and Thomas A. Ban’s Comment Exchange on Flagrant Catatonic Behavior

 

            I was invited to participate in this exchange under the title of "Flagrant Catatonic Behavior.”

            According to the English Cambridge Dictionary,  “flagrant” means "shocking because of being so obvious” (so intense and evident like the light of a fire). I mention the definition of “flagrant” because, in contrast, we may find catatonic manifestation that is very discreet (subtle), like a simple repetitive finger movement, or a reiterated word present in an extended speech disconnected to the whole context.

            Automatically, I went back to my years at the Hospital Franco da Rocha (also known as Hospital do Juqueri), a psychiatric institution founded by Dr. Franco da Rocha in the late 19th century, 40 km from the city of São Paulo (Brazil), where I studied and worked for years. At that time the hospital complex was composed of several buildings for acute patients and colonies (farm houses) for chronic patients. In 1970s the total population was 11,000 patients! Anyone interested in psychiatry could see anything and everything. I used to take few hours each week to just observe how the patients behaved when outside in the courtyard of the buildings. Of course, I recognized many patients with catatonic signs of all sorts: pacing up and down; sitting for hours in the same position and at the same local, everyday; suddenly standing up and running around a tree; walking while counting the steps and making extravagant gestures with the arms and face; in an absolute mutism, static, not responding to any exterior stimulus; in echopraxia; and in active and passive negativisms. A hospital document from early 1900s describes how an agitated and aggressive patient, having an explosive reaction, rushed to a patient who was in a stuporous state and hit him in the abdomen; the catatonic felt unconscious for few minutes; then, stood up and started walking regularly with an expression of pain but talking to others; this case, known as the “masso-therapy” (boulder-therapy), was registered before the advent of ECT! There was a patient who used to go to the Director's office (he allowed the patient to do so) and for hours would stand beside his desk and move his trunk as a pendulum, while rigidly maintaining his head, face and arms (he was known as “the director's clock”).

            During interviews I had numerous opportunities to observe different stereotypic movements, as well as echolalia, echopraxia and "flexibilitas cerea.” I observed patients going from hyperkinesia to total akinesia (almost like an amentia state); others presented an association of a general akinesia and a localized repetitive movement, like that of just of one finger; and patients with partial catatonic remission, all without any significant treatment. I also observed paranoid patients progressing to catatonia, and vice-versa.

            By the time I left the Hospital Franco da Rocha, and in the early ‘80s concentrated my teaching in another public hospital in São Paulo with 200 patients (acute and chronic), the catatonic patients became less and less frequent to my sight.

            In my private practice (since 1969) I had few schizophrenic catatonic patients, fortunately with favorable outcomes, at least regarding the catatonic symptoms. Association of ECT and haloperidol showed significant degree of effectiveness. However, during these last 20 years working in Australia, I do not remember having seen a catatonic patient in my private practice (which includes in-patients). Of course, I am not mentioning here the cases of organic brain syndrome, or acute psych-organic reactions (Bleuler, Willi and Bülher 1968), etc.

            My main professor of psychiatry, Paulo Fraletti, an inveterate adept of German psychiatry, used to say that some catatonia has a psychogenic factor (he never told me from where and how he formulated this idea). According to my experience, however, and considering the significant reduction of catatonias in the last decades, I may speculate that - like hebephrenic schizophrenia - there are environmental factors influencing the manifestation of catatonic signs/symptoms (here I am excluding the cases of definitive functional disorders and pathologies, i.e., neurological, endocrine, infectious, etc.

            En passant, anyone could question the evolution of R. Gjessing’s (Minde 1966) periodic catatonia hypothesis: are catatonic stupor and catatonic excitement symptoms of a basic similar pathophysiological process linked to nitrogen fluctuation? Of course, to begin is like trying to answer the question: which one comes first, the egg or the chicken? Maybe research like that outlined in the abstract below (Balint et al. 2018) could help us.

            Saying all this, I am not sure whether early treatment does not prevent catatonia from appearing in the course of schizophrenia, as happens with hallucinations and delusions that are aborted by the so-called anti-psychotics, or environmental factors from this agitated world may be providing enough stimulus to counterpose the catatonic functional mechanism. 

 

Abstract

(Movement disorders with neuronal antibodies: syndromic approach, genetic parallels and pathophysiology)

 

Movement disorders are a prominent and common feature in many autoantibody-associated neurological diseases, a group of potentially treatable conditions that can mimic infectious, metabolic or neurodegenerative disease. Certain movement disorders are likely to associate with certain autoantibodies; for example, the characteristic dyskinesias, chorea and dystonia associated with NMDAR antibodies, stiff person spectrum disorders with GAD, glycine receptor, amphiphysin or DPPX antibodies, specific paroxysmal dystonias with LGI1 antibodies, and cerebellar ataxia with various anti-neuronal antibodies. There are also less-recognized movement disorder presentations of antibody-related disease, and a considerable overlap between the clinical phenotypes and the associated antibody spectra. In this review, we first describe the antibodies associated with each syndrome, highlight distinctive clinical or radiological ‘red flags’, and suggest a syndromic approach based on the predominant movement disorder presentation, age, and associated features. We then examine the underlying immunopathophysiology, which may guide treatment decisions in these neuroimmunological disorders, and highlight the exceptional interface between neuronal antibodies and neurodegeneration, such as the tauopathy associated with IgLON5 antibodies. Moreover, we elaborate the emerging pathophysiological parallels between genetic movement disorders and immunological conditions, with proteins being either affected by mutations or targeted by autoantibodies. Hereditary hyperekplexia, for example, is caused by mutations of the alpha subunit of the glycine receptor leading to an infantile-onset disorder with exaggerated startle and stiffness, whereas antibodies targeting glycine receptors can induce acquired hyperekplexia. The spectrum of such immunological and genetic analogies also includes cerebellar ataxias and some encephalopathies. Lastly, we discuss how these pathophysiological considerations could reflect on possible future directions regarding antigen-specific immunotherapies or targeting the pathophysiological cascades downstream of the antibody effects.

 

References:

Balint B, Vincent A, Meinck HM, Irani SR, Bhatia KP. Movement disorders with neuronal antibodies: syndromic approach, genetic parallels and pathophysiology.  Brain, Volume 141, Issue 1, 1 January 2018, pp. 13–36.

 

 

Bleuler M, Willi J, Bülher, HD. Concepción de Bonhoeffer sobre el tipo de reacción exógena aguda. In: Desarrollo y Estado Actual de Nuestros Conocimientos. Sindromes Psiquicos Agudos en Las Enfermedades Somaticas. Ediciones Morata, Madrid, 1968; pp 1-31.

 

Minde K. Periodic catatonia, a review with special reference to Roly Guessing. The Canadian Psychiatric Association Journal / La Revue de L’Association des psychiatres du Canada. 1996; 11 (5), 421-425. April 5, 2018.

 

April 5, 2018