A 34-year-old woman presents to accident and emergency claiming that the
devil has returned to earth and is hunting her through her neighbours, who are
recording her every movement. The psychiatric assessment shows florid delusions
and auditory hallucinations. She has no past psychiatric history. Her husband tells
you that she was fine up until 2 weeks ago. Her hands have also been shaking
and she has complained that the devil has been torturing her muscles. She has
widespread lymphadenopathy and an enlarged spleen. An unusual rash is present
across her cheeks and nose, which she says is the brand of the devil. What is the
most likely diagnosis?
A. Behçet’s disease
B. CREST syndrome
C. Graves’ disease
D. Systemic lupus erythematosus (SLE)
E. Wegener’s granulomatosis
Answer
D SLE (D) is an autoimmune connective tissue disorder that may affect any
organ in the body. It commonly presents in women (9:1 female to male
ratio), usually in the third or fourth decades. Neuropsychiatric symptoms
may occur at the beginning of the natural course of the disorder,
without any seeming involvement of other organ systems. Unexplained
psychotic symptoms, which may closely resemble schizophrenia, may
occur, as may a dementia-like illness or affective disorders. This woman
is also displaying neurological signs, making a diagnosis of a functional
psychiatric disorder unlikely. The presence of Parkinsonism, widespread
muscle pain, lymphadenopathy and splenomegaly are all consistent
with the diagnosis. The ‘malar rash’, which is highly indicative of SLE,
is present in this woman but its absence does not negate the diagnosis.
Behçet’s disease (A) is an autoimmune disorder characterized by recurrent
mouth ulcers, genital ulcers and uveitis. Neurological or psychiatric
symptoms are uncommon and usually a late presentation. It also tends
to present in the third or fourth decades, but tends to be more common
in men. The CREST syndrome (B) is a form of systemic scleroderma
characterized by the following five cardinal features from which the
acronym is derived: calcinosis, in which there is calcium deposits in
the soft tissue, usually under the skin; Raynaud’s phenomenon, in
which there is bilateral cyanosis of the hands in response to cold or
stress; oesophageal atresia, which presents with symptoms of gastrooesophageal
reflux; sclerodactyly, in which there is thickening of the
skin of the hands and feet, taking on a ‘shiny’ appearance; finally,
telangiectasia, which are lesions caused by dilated blood vessels and
usually present on the face or hands. Psychiatric complications have been
little studied in this disorder, although depression certainly does occur.
Graves’ disease (C) is an autoimmune thyroiditis, which incidentally
may occur co-morbidly with SLE, although the greatest association is
with rheumatoid arthritis. The characteristic symptoms include those of
hyperthyroidism, which in psychiatric terms may resemble an anxiety
disorder, with tachycardia and palpitations. There may be unexplained
weight loss. Systemic symptoms, such as malaise, are also common, as is
exophthalmos and pretibial myxoedema (‘orange peel’ skin on the shin).
The psychiatric symptoms tend to either resemble an anxiety disorder,
or other affective symptoms. Like SLE, women are more commonly
affected, and usually in their 20s or 30s. Wegener’s granulomatosis (E)
is an autoimmune vasculitis that typically affects the lungs, kidneys and
nervous system. It affects males slightly more often with a slightly later
peak of incidence (fourth and fifth decades). The typical presentations are
with dyspnoea, cough, haemoptysis, nasal ulceration, sinusitis, systemic
symptoms, haematuria, and neurological symptoms – typically peripheral
neuropathy but sometimes strokes and seizures. Psychiatric complications
have not typically been described except in case reports.
devil has returned to earth and is hunting her through her neighbours, who are
recording her every movement. The psychiatric assessment shows florid delusions
and auditory hallucinations. She has no past psychiatric history. Her husband tells
you that she was fine up until 2 weeks ago. Her hands have also been shaking
and she has complained that the devil has been torturing her muscles. She has
widespread lymphadenopathy and an enlarged spleen. An unusual rash is present
across her cheeks and nose, which she says is the brand of the devil. What is the
most likely diagnosis?
A. Behçet’s disease
B. CREST syndrome
C. Graves’ disease
D. Systemic lupus erythematosus (SLE)
E. Wegener’s granulomatosis
Answer
D SLE (D) is an autoimmune connective tissue disorder that may affect any
organ in the body. It commonly presents in women (9:1 female to male
ratio), usually in the third or fourth decades. Neuropsychiatric symptoms
may occur at the beginning of the natural course of the disorder,
without any seeming involvement of other organ systems. Unexplained
psychotic symptoms, which may closely resemble schizophrenia, may
occur, as may a dementia-like illness or affective disorders. This woman
is also displaying neurological signs, making a diagnosis of a functional
psychiatric disorder unlikely. The presence of Parkinsonism, widespread
muscle pain, lymphadenopathy and splenomegaly are all consistent
with the diagnosis. The ‘malar rash’, which is highly indicative of SLE,
is present in this woman but its absence does not negate the diagnosis.
Behçet’s disease (A) is an autoimmune disorder characterized by recurrent
mouth ulcers, genital ulcers and uveitis. Neurological or psychiatric
symptoms are uncommon and usually a late presentation. It also tends
to present in the third or fourth decades, but tends to be more common
in men. The CREST syndrome (B) is a form of systemic scleroderma
characterized by the following five cardinal features from which the
acronym is derived: calcinosis, in which there is calcium deposits in
the soft tissue, usually under the skin; Raynaud’s phenomenon, in
which there is bilateral cyanosis of the hands in response to cold or
stress; oesophageal atresia, which presents with symptoms of gastrooesophageal
reflux; sclerodactyly, in which there is thickening of the
skin of the hands and feet, taking on a ‘shiny’ appearance; finally,
telangiectasia, which are lesions caused by dilated blood vessels and
usually present on the face or hands. Psychiatric complications have been
little studied in this disorder, although depression certainly does occur.
Graves’ disease (C) is an autoimmune thyroiditis, which incidentally
may occur co-morbidly with SLE, although the greatest association is
with rheumatoid arthritis. The characteristic symptoms include those of
hyperthyroidism, which in psychiatric terms may resemble an anxiety
disorder, with tachycardia and palpitations. There may be unexplained
weight loss. Systemic symptoms, such as malaise, are also common, as is
exophthalmos and pretibial myxoedema (‘orange peel’ skin on the shin).
The psychiatric symptoms tend to either resemble an anxiety disorder,
or other affective symptoms. Like SLE, women are more commonly
affected, and usually in their 20s or 30s. Wegener’s granulomatosis (E)
is an autoimmune vasculitis that typically affects the lungs, kidneys and
nervous system. It affects males slightly more often with a slightly later
peak of incidence (fourth and fifth decades). The typical presentations are
with dyspnoea, cough, haemoptysis, nasal ulceration, sinusitis, systemic
symptoms, haematuria, and neurological symptoms – typically peripheral
neuropathy but sometimes strokes and seizures. Psychiatric complications
have not typically been described except in case reports.
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