Sarcoidosis presenting as late-onset dementia

Sanson, Marc; Duyckaerts, Charles; Thibault, Jean-Luc; Delattre, Jean‐Yves

doi:10.1007/bf00900506

Cited by 8 publications

(5 citation statements)

References 10 publications

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“…Corticosteroids are the cornerstone of therapy for neurosarcoidosis, and improvement was achieved in the vast majority of reported patients with dementia whereas nearly all of those who did not receive therapy had a fatal course [2,3,4,6,7,8,10,11,12,14]. However, steroid treatment would be devastating if CNS tuberculosis was missed.…”

mentioning

confidence: 97%

Sarcoidosis presenting as rapidly progressive dementia: clinical and neuropathological evaluation

Schielke¹,

Nolte²,

Müller

et al. 2001

Journal of Neurology

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mentioning

confidence: 97%

Sarcoidosis presenting as rapidly progressive dementia: clinical and neuropathological evaluation

Schielke¹,

Nolte²,

Müller

et al. 2001

Journal of Neurology

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“…Mood and behavioural symptoms are often present but are rarely predominant [ 13 ]. In elderly patients, a progressive onset can mimic a degenerative dementia [ 11 ]. The diagnosis of sarcoidosis may then be very difficult when systemic signs are missing or latent.…”

Section: Discussionmentioning

confidence: 99%

Systemic Sarcoidosis Mimicking a Behavioural Variant of Frontotemporal Dementia

Maindreville

Bedos

Bakchine

2015

Case Reports in Neurological Medicine

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Among rare neurological manifestations, a progressive dementia may exceptionally be the revealing clinical feature of a sarcoidosis. Diagnosis may then be difficult, especially when systemic signs are missing or latent, with a risk of therapeutic delay. We report the first case of sarcoidosis mimicking a frontotemporal dementia. A 53-year-old man presented with a dementia clinically suggestive of frontotemporal dementia, progressing slowly for about 2 years. However, MRI revealed unusual aspects, mainly large areas of T2/FLAIR hypersignal within temporal regions and cerebellum, with nodular leptomeningeal and juxtacortical Gadolinium enhancement. The patient was finally diagnosed with a systemic sarcoidosis. We discuss the differential diagnosis based on MRI aspects and review the literature on the clinical, biological, and imaging features of sarcoidosis presenting with dementia. This case demonstrates that brain imaging remains mandatory in the exploration process of a patient with dementia. Although the patient presented with rather typical features of a behavioural variant of frontotemporal dementia, the MRI aspect was the key exploration that leaded to the diagnosis.

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“…Although it usually presents in young adults, late presentation as dementia has been reported 2. However, serum angiotensin-converting enzyme levels were normal and there was no hilar lymphadenopathy on the chest x ray.…”

Section: Commentmentioning

confidence: 98%

An exotic cause for confusion in the garden: Figure

Williams

Ginsberg²,

Brenner³

et al. 2008

Pract Neurol

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A 70-year-old retired accountant presented with progressive confusion. He was unable to give a clear account of recent events so his wife provided most of the history. She had first noticed four months previously that he had been unable to organise social events for his local Rotary club, and also had lost his ability to manage the household finances. There was then a progressive decline in his memory and organisational skills with increasing confusion about everyday events. In addition he had developed a tremor when holding the newspaper or trying to write. He also complained of muscle cramps in his legs and a band of sensitivity around his abdomen, which were severe enough to wake him at night. He appeared anxious and confused at social occasions, and was sweating and complaining that his head felt hot. The only significant medical history was a raised blood cholesterol for which he was taking simvastatin. There was no relevant family history.General examination was normal and he was afebrile. On cranial nerve examination he had jerky pursuit eye movements and slow tongue movements. On examination of the limbs he had normal tone, power, reflexes and sensation, but impaired coordination; there was a fine postural tremor in the hands and a bilateral intention tremor, left worse than right. His gait was slow and broad-based. He scored 20/30 on Folstein's Mini-Mental State Examination (MMSE), and 71% on the Addenbrooke's cognitive examination (ACE). Detailed neuropsychological testing showed global cognitive impairment suggesting both cortical and subcortical involvement.Initial blood tests showed a sodium of 127 mmol/l and a raised ESR (37 mm/h). Further evaluation of the hyponatraemia revealed a low plasma osmolality (268 mOsm/kg) (normal 275-295 mOsm/kg), inappropriately high urine osmolality (676 mOsm/kg) and high urine sodium (67 mmol/l) in the absence of hypovolaemia, confirming the diagnosis of SIADH (Syndrome of Inappropriate AntiDiuretic Hormone secretion). CT and MR scans of the brain were normal. Practical Neurology

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Sarcoidosis presenting as late-onset dementia

Cited by 8 publications

References 10 publications

Sarcoidosis presenting as rapidly progressive dementia: clinical and neuropathological evaluation

Sarcoidosis presenting as rapidly progressive dementia: clinical and neuropathological evaluation

Systemic Sarcoidosis Mimicking a Behavioural Variant of Frontotemporal Dementia

An exotic cause for confusion in the garden: Figure

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