It is relatively uncommon to find a patient presenting with subacute dementia with involuntary movements in clinical practice. Such a presentation requires astute evaluation to diagnose and treat reversible conditions early and improve outcome.
Case Report
A 67‐year‐old male presented with new onset “fidgetiness,” “confusion,” and “restlessness” for 3 weeks. His wife and son felt that he had been “remaining aloof,” in his own world, not communicating much and was confused. He appeared fidgety, kept wriggling his fingers and toes, and had become clumsy, spilling his food on his clothes and the floor while eating and stumbling and needing support while walking. This was preceded by loose watery stools with mild abdominal pain without fever for a week. He did not have any exposure to alcohol, drugs, or toxins or other comorbidities.
On examination, he had hyperpigmentation of the dorsal surface of the hands and feet with patches of desquamation, with hyperkeratosis of the elbows, with clear demarcation from normal skin (see Video 1). He had frontal subcortical executive dysfunction (scored 18/30 on the Mini‐Mental State Examination, and 6/18 on the Frontal Assessment Battery). He made errors in orientation, attention, short‐term memory, calculation, conceptualization, lexical fluency, and motor programming (abnormal motor Luria test; see Video 1 ). His extraocular movements, limb tone, power, reflexes, and sensory examination were normal. He had involuntary patterned, rhythmic, repetitive, nonreflexive movements consisting of flapping and twisting of his hands and feet, with picking and wriggling of his fingers and toes, which were partially controllable volitionally, suggestive of a combination of stereotypy, carphology, and athetosis. He had symmetric ataxia of the gait and trunk with “striatal toes” while walking. These deficits suggested subacute dysfunction of the bilateral basal ganglia with frontal lobe and cerebellar connections. The etiological differentials would include toxic–metabolic/nutritional disorders, infections, and structural, autoimmune, and demyelinating disorders.
Video 1.
Pretreatment video demonstrating combination of distal limb stereotypy, carphology, and athetosis in the toes (0–27 seconds); hyperpigmented skin with desquamation on extensor surfaces of the hands; feet with hyperkeratosis at the elbow (30–57 seconds); and symmetric ataxia of gait and limbs with “striatal toes” with impaired tandem walking (58–120 seconds) and with perseveration on trying to do alternate pronation supination movements of the hands with abnormal motor luria testing suggestive of frontal subcortical executive dysfunction (131–147 seconds).
His hemogram, liver, renal function, thyroid, glucose, electrolytes, vitamin B12, and homocysteine levels and magnetic resonance imaging of the brain and spine were normal. He had the classic triad of diarrhea, dermatitis, and dementia seen in pellagra.1 Hence further details of his food and dietary habits were evaluated. He was a peasant whose crop had failed that season and his cow had died and so his staple diet was restricted to only maize and rice for the past few months. In view of this history and clinical findings, a diagnosis of pellagra was considered. Pellagra is a disease attributed to deficiency of niacin and/or tryptophan, classically described in poor peasants who are isolated maize eaters and do not consume other dietary products rich in niacin (such as dairy products, cereals, wheat). He was treated with oral niacin tablets at a dose of 250 mg 3 time a day for 2 weeks and had complete recovery with treatment (see Video 2 ).
Video 2.
Posttreatment video demonstrating improvement in cognition, gait, balance, and skin rash with remission of involuntary movements.
Discussion
It is important for clinicians to recognize this clinical triad (diarrhea, dermatitis, dementia) of pellagra as a delay in treatment can lead to the fourth D, which is death.1, 2 All patients may not have all of the manifestations of the triad at the same time. The classic “Casal's collar” (or “Casal's necklace”) consisting of erythematous skin changes involving the lower part of neck and chest may not always be present. Neuropsychiatric manifestations such as irritability, anxiety, delusions, hallucinations, depression, psychomotor slowing, mania, fatigue, personality changes, myelopathy, and neuropathy may also be present. Myriad movement disorders described in pellagra include tremors, chorea/choreo‐athetosis, parkinsonism, and gait ataxia (isolated sensory and/or cerebellar ataxia). Pellagra can also occur in the settings of chronic alcoholism with malnutrition, anorexia nervosa, malabsorption syndromes, and chronic isoniazid treatment. Clinicians often are inclined to doing multiple investigations including rare diagnoses of autoimmune disorders in patients presenting with new‐onset dementia. Often such tests such as an autoimmune panel, paraneoplastic panel, and advanced imaging techniques are costly, cumbersome, and take time to be done. A clinical diagnosis such as pellagra can be diagnosed by an astute clinician by a thorough history taking and systemic examination, leading to early treatment and avoidance of delay in treatment, which can lead to disastrous outcomes.2 This case serves as a reminder to neurologists of the importance of good history taking and examination.
Author Roles
(1) Research Project: A. Conception, B. Organization, C. Execution; (2) Manuscript Preparation: A. Writing of the First Draft, B. Review and Critique; (3) Video Editing.
S.D.: 1A, 1B, 1C, 2B, 3
D.D.: 1B, 1C, 2A, 2B
Disclosures
Ethical Compliance Statement
We confirm that we have read the journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines. The Institutional Review Committee of Shree Krishna Hospital Bhaikaka University has approved the study. The patient and his son [who is caregiver and legal representative] have given consent for presentation and publication.
Funding Sources and Conflicts of Interest
No specific funding was received for this work. The authors declare that there are no conflicts of interest relevant to this work.
Financial Disclosures for the Previous 12 Months
The authors have no financial disclosures to report for the previous 12 months.
Relevant disclosures and conflicts of interest are listed at the end of this article.
References
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