Catatonia – not only a schizophrenia subtype: a case report of Wilson’s disease

Catatonia is a psychomotor syndrome first described by Kahlbaum, who acknowledged psychiatric, neurologic, and general medical etiologies.¹ Under the influence of Kraepelin, catatonia was considered a schizophrenia subtype for many years. The DSM-5 described catatonia across the manual, regardless of associated conditions.² Wilson’s disease (WD) is a genetic condition that causes copper to accumulate in several organs. Approximately 20% of patients present with behavioral symptoms at disease onset. Psychotic phenomena and catatonia are rare,³,⁴ but may occur due to copper accumulating in the brain.⁵-⁷

A 24-year-old single man was referred to the psychiatric ward of the hospital of Universidade Federal de Pernambuco due to reports of unusual and refractory depression. Two years before admission, the patient developed insomnia and refused food. During the first year, he still interacted with friends and family. After 6 months, he isolated himself, became mute, and developed self-injurious behavior. He was taken to a primary care service where escitalopram 20 mg/day, levomepromazine 25 mg/day, clonazepam 2 mg/day, and lithium carbonate 300 mg/day were prescribed. Twenty months after the initial onset of symptoms, he had significant impairment in activities of daily living, reduced mobility, repetitive masticatory movements, mutism, and posturing. The patient had no previous history of psychiatric disorders or psychoactive substance use. During the admission evaluation, he remained mute, with his head down and fists clenched. When called, he glanced at the doctor. Upon request, he tried to stand up, but failed. He scored 27 on the Bush-Francis Catatonia Rating Scale (BFCRS), and 0 on the Katz scale.

In hospital, the patient was treated with lorazepam 12 mg/day (progressing over 2 weeks and continuing for up to 4 weeks) and olanzapine 5 mg/day, considering a possible psychotic etiology. Throughout the first 2 weeks of hospitalization, we noticed an improvement in psychomotor stiffness. The relevant lab results were: C-reactive protein, 10 mg/L; gamma-glutamyl transferase, 150.4 U/L; and ferritin, 465.9 ng/mL, with normal transaminases. Electroencephalography was unremarkable. Abdominal ultrasound showed a liver with heterogeneous texture and regeneration nodules. MRI of the brain (Figure 1) showed diffuse volumetric reduction and bilateral hyperintensities in the basal ganglia on T2/FLAIR sequences, especially in the periphery of the putamen. A 24-hour urine copper test (128.8 μg) and ceruloplasmin level (6.7 mg/dL) were requested, as well as an ophthalmologic evaluation, which revealed Kayser-Fleischer rings. Olanzapine was discontinued immediately upon diagnosis of WD. Due to a limited response to benzodiazepines after 4 weeks, bitemporal electroconvulsive therapy was performed, which did not show any benefit after six sessions. Specific treatment for WD with the use of copper chelation therapy agents was initiated.⁵ After 6 months, the patient was able to sit, eat, and cooperate with caregivers. Nine months after starting chelation therapy, the patient scored 10 on the BFCRS and 2 on the Katz scale. In three previously described cases, chelation had mixed.⁴,⁸,⁹

Figure 1
MRI of brain showing diffuse volumetric reduction with adaptive dilation of the ventricular system and bilateral hyperintensity in the basal ganglia on T2/FLAIR sequences, especially in the periphery of the putamen, alterations which are consistent with Wilson’s disease.

It is important to remember that catatonia is not exclusively a subtype of schizophrenia, and may be a manifestation of other clinical conditions. Clinicians should be aware of this and institute treatment for the underlying cause of catatonia whenever one is identified.

References

• ¹
  Carroll BT. Kahlbaum’s catatonia revisited. Psychiatry Clin Neurosci. 2001;55:431-6.
• ²
  Walther S, Stegmayer K, Wilson JE, Heckers S. Structure and neural mechanisms of catatonia. Lancet Psychiatry. 2019;6:610-9.
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• ⁴
  Basu A, Thanapal S, Sood M, Khandelwal SK. Catatonia: an unusual manifestation of Wilson’s disease. J Neuropsychiatry Clin Neurosci. 2015;27:72-3.
• ⁵
  Poujois A, Woimant F. Wilson’s disease: a 2017 update. Clin Res Hepatol Gastroenterol. 2018;42:512-20.
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  European Association for Study of Liver. EASL clinical practice guidelines: Wilson’s disease. J Hepatol. 2012;56:671-85.
• ⁸
  Davis EJ, Borde M. Wilson’s disease and catatonia. Br J Psychiatry. 1993;162:256-9.
• ⁹
  Nayak RB, Shetageri VN, Bhogale GS, Patil NM, Chate SS, Chattopadhyay S. Catatonia: a rare presenting symptom of Wilson's disease. J Neuropsychiatry Clin Neurosci. 2012;24:E34-5.

Publication Dates

• Publication in this collection
  17 Oct 2019
• Date of issue
  Sep-Oct 2019

History

• Received
  27 May 2019
• Accepted
  1 Aug 2019