Wilson's Disease

Dening, Tom; Berríos, Germán E.

doi:10.1001/archpsyc.1989.01810120068011

Cited by 205 publications

(42 citation statements)

References 61 publications

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“…Psychiatric symptoms have been reported in about 50% of patients with WD and include abnormal behavior and personality, depression, elation, irritability and hallucinations [9, 25]. Many patients with WD are first visited by psychiatrists, and in our series 2 patients even received electroconvulsive therapy.…”

Section: Discussionmentioning

confidence: 86%

Wilson’s Disease: A Great Masquerader

Soltanzadeh

Nafissi

et al. 2006

Eur Neurol

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Background: Wilson’s disease (WD) is a treatable autosomal recessive metabolic disorder which could lead to protean hepatic or neurologic manifestations. WD could mimic many neurologic disorders and is often diagnosed with a long delay. This study describes central nervous system manifestations of a group of Iranian patients with neurologic WD. Methods: Data from case records of patients with neurologic WD presenting at a referral university hospital and a private clinic in Tehran from 1984 to 2004 were analyzed. Results: Fifty patients from 44 unrelated families with WD were identified, whose mean duration of follow-up was 51.8 (±58.5) months. The median age of onset of neurologic symptoms in 37 patients with primary neurologic or simultaneous hepatic-neurologic presentation was 16 (10–38) years, whereas in 13 patients with prior hepatic damage, this was 18.5 (11–34) years. The 6 most common manifestations were dysarthria (80%), drooling (48%), tremor in limbs (44%), abnormal gait (44%), psychiatric and/or sleep symptoms (44%), and dystonia in limbs (42%). Conclusion: Neurologic WD has heterogeneous manifestations and should be considered in young patients presenting with dysarthria, drooling, any kind of movement disorders or psychiatric symptoms.

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Section: Discussionmentioning

confidence: 86%

Wilson’s Disease: A Great Masquerader

Soltanzadeh

Nafissi

et al. 2006

Eur Neurol

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“…Between one- and two-thirds of patients report psychiatric symptoms at initial presentation [56-58]. Psychiatric signs are present in almost 50% of patients at any one time [59], and present before motor signs in 20% of cases; up to half of patients may be seen initially by a psychiatrist [60]. …”

Section: Resultsmentioning

confidence: 99%

“…Mood disturbance, including both depression and mania, is the most common formal neuropsychiatric illness [63-67]. Psychosis, delusional states and catatonia, while less frequent in WD, can be extremely disabling [56,57,60,61,68]. Schizophrenia-like symptoms were reported to be present in up to 10% of patients [63], but were less prevalent in one case series (2.4%) [69].…”

Section: Resultsmentioning

confidence: 99%

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Diagnostic and treatment implications of psychosis secondary to treatable metabolic disorders in adults: a systematic review

Bonnot

Klünemann

Sedel

et al. 2014

Orphanet Journal of Rare Diseases

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ObjectiveIt is important for psychiatrists to be aware of certain inborn errors of metabolism (IEMs) as these rare disorders can present as psychosis, and because definitive treatments may be available for treating the underlying metabolic cause. A systematic review was conducted to examine IEMs that often present with schizophrenia-like symptoms.Data sourcesPublished literature on MEDLINE was assessed regarding diseases of homocysteine metabolism (DHM; cystathionine beta-synthase deficiency [CbS-D] and homocysteinemia due to methyltetrahydrofolate reductase deficiency [MTHFR-D]), urea cycle disorders (UCD), acute porphyria (POR), Wilson disease (WD), cerebrotendinous-xanthomatosis (CTX) and Niemann-Pick disease type C (NP-C).Study selectionCase reports, case series or reviews with original data regarding psychiatric manifestations and cognitive impairment published between January 1967 and June 2012 were included based on a standardized four-step selection process.Data extractionAll selected articles were evaluated for descriptions of psychiatric signs (type, severity, natural history and treatment) in addition to key disease features.ResultsA total of 611 records were identified. Information from CbS-D (n = 2), MTHFR-D (n = 3), UCD (n = 8), POR (n = 12), WD (n = 11), CTX (n = 14) and NP-C publications (n = 9) were evaluated. Six non-systematic literature review publications were also included. In general, published reports did not provide explicit descriptions of psychiatric symptoms. The literature search findings are presented with a didactic perspective, showing key features for each disease and psychiatric signs that should trigger psychiatrists to suspect that psychotic symptoms may be secondary to an IEM.ConclusionIEMs with a psychiatric presentation and a lack of, or sub-clinical, neurological signs are rare, but should be considered in patients with atypical psychiatric symptoms.

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“…Schizophreniform disorders, catatonia, and hallucinations are no more common in WD than in the general population, but psychosis and catatonia occur more commonly in neurological WD [1]. Patients with WD having predominantly neuropsychiatric symptoms, present with symptoms later in life, have a longer time delay from onset of symptoms until definitive diagnosis, and have a poorer outcome compared with patients with hepatic symptoms [8]. …”

Section: Wilson’s Diseasementioning

confidence: 99%

Psychiatric signs and symptoms in treatable inborn errors of metabolism

Nia¹

2014

J Neurol

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Possible underlying organic causes of psychiatric symptoms can be overlooked in the clinical setting. It is important to increase awareness amongst psychiatric and neurological professionals with regard to certain inborn errors of metabolism as, in some cases, disease-specific therapies are available that can, for instance, treat underlying metabolic causes. The following article describes the basic pathophysiology, clinical and neurological features, and available diagnostic procedures of six treatable metabolic diseases that are associated with neuropsychiatric symptoms: Wilson’s disease, cerebrotendinous xanthomatosis, porphyrias, homocysteinemia, urea cycle disorders, and Niemann-Pick disease type C (NP-C). NP-C is taken as a particularly relevant example because, while it is traditionally considered to be a condition that presents with severe neurological and systemic manifestations in children, an increasing number of patients are being detected who have the adolescent- or adult-onset form, which is frequently associated with neuropsychiatric signs. A notable proportion of adult-onset cases have been reported where NP-C has mistakenly been diagnosed and treated as a psychiatric condition, usually based on patients’ initial presentation with psychotic or schizophrenia-like symptoms. Underlying organic causes of psychiatric disorders such as psychosis should be considered among patients with atypical symptoms and/or resistance to standard therapy. Alongside improved frameworks for additional multidisciplinary diagnostic work in patients with suspected organic disease, the development of convenient and affordable biochemical screening and/or diagnostic methods has enabled new ways to narrow down differential diagnoses.

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Wilson's Disease

Cited by 205 publications

References 61 publications

Wilson’s Disease: A Great Masquerader

Wilson’s Disease: A Great Masquerader

Diagnostic and treatment implications of psychosis secondary to treatable metabolic disorders in adults: a systematic review

Psychiatric signs and symptoms in treatable inborn errors of metabolism

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