Central Nervous System Crystal-Storing Histiocytosis: Neuroimaging, Neuropathology, and Literature Review

Kaminsky, I.A.; Wang, A.-M.; Olsen, James B; Schechter, S.; Wilson, J. L.; Olson, Roy E.

doi:10.3174/ajnr.a1983

Cited by 21 publications

(27 citation statements)

References 5 publications

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“…In a second case, a 27 year-old woman with Crohn's disease presented with asymmetric tremor and left sided sensory disturbance in the setting of a unifocal gadolinium enhancing brain lesion associated with mild mass effect and edema. Biopsy confirmed the presence of intracytoplasmic crystal inclusions within macrophages (Kaminsky et al, 2011). No treatment was mentioned.…”

Section: Discussionmentioning

confidence: 93%

Crystal-storing histiocytosis: An unusual relapsing inflammatory CNS disorder

Costanzi

Bourdette

Parisi

et al. 2012

Multiple Sclerosis and Related Disorders

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The differential diagnosis of acute leukoencephalopathy often focuses on central nervous system idiopathic inflammatory demyelinating diseases (IIDDs) such as multiple sclerosis (MS). However, a spectrum of conditions mimic IIDDs, therefore it is critical to consider whether symptoms, signs, imaging and/or response to therapies are compatible with the diagnosis. We describe a 32-year-old previously healthy woman presenting with a 2 year history of steroid-responsive relapsing episodes lasting 2–10 days characterized by transient visual blurring, right-hemiparesis, and spells of aphasia. MRI demonstrated multifocal, relapsing, predominantly white matter enhancing brain lesions, a longitudinally extensive cord lesion, and abnormal visual evoked potentials. Notably, some lesions persistently enhanced whereas others demonstrated progressive T2W hypointensity. Brain biopsy revealed an atypical plasma cell infiltrate and crystal-storing histiocytosis, which by mass spectrometry confirmed the presence of macrophages containing intracellular kappa-light chain restricted crystals. Bone marrow was negative. The patient did well for several years on pulse dexamethasone, however subsequent scans demonstrated increasing enhancement. Repeat biopsy demonstrated a clonal plasma cell proliferation. She was treated with melphalan, and has remained stable. Although this patient initially met McDonald criteria, atypical imaging prompted further workup, and advanced proteomic technology helped secured an accurate diagnosis. Crystal-storing histiocytosis should be considered in the differential diagnosis of inflammatory CNS disorders.

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Section: Discussionmentioning

confidence: 93%

Crystal-storing histiocytosis: An unusual relapsing inflammatory CNS disorder

Costanzi

Bourdette

Parisi

et al. 2012

Multiple Sclerosis and Related Disorders

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“…However, occasionally it develops in a setting of plasma cell granulomas, atypical infections, and other inflammatory processes such as rheumatoid arthritis, mastocytosis, Crohn's disease, and hypereosinophilic syndrome. [12] The majority of CSH without an identified lymphoproliferative disorders occur in women[1] [Table 1]. Consequently, the diagnosis of CSH warrants careful evaluation of the underlying cause.…”

Section: Discussionmentioning

confidence: 99%

“…Most reported and the present case of CSH presented as mass lesions in the cerebral hemispheres. [245] One case presented as a serpentine enhancing lesion of the centrum semiovale. [2] The present case presented as a left temporal lobe mass.…”

Section: Discussionmentioning

confidence: 99%

“…The use of electron microscopy, immunofluorescence, in situ hybridization for immunoglobulins and light chains and, if available, liquid chromatography-electrospray tandem mass spectroscopy may facilitate recognition. [12]…”

Section: Discussionmentioning

confidence: 99%

“…To our knowledge, only three well-documented cases involving the CNS have been reported. [2345] We describe an atypical case of CSH and review the literature.…”

Section: Introductionmentioning

confidence: 99%

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Crystal storing histiocytosis presenting as a temporal lobe mass lesion

Johnson

Mazariegos²,

Lewis³

et al. 2013

Surg Neurol Int

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Background:Crystal storing histiocytosis (CSH) is a disorder characterized by local or diffuse infiltration of histiocytes containing crystalline inclusions most commonly of immunoglobulin light chain. Involvement of the central nervous system is extremely rare. CSH may be misdiagnosed as an infection or tumor. In patients with involvement of other organs, it is frequently associated with lymphoplasmacytic diseases.Case Description:A 20-year-old female was evaluated for 2 weeks of progressively worsening headaches. At presentation, she had no history of fevers but reported a sore throat without cough 3-4 days prior. Her past medical history was unremarkable. She denied intravenous drug use or sexually transmitted diseases but lived with an individual with a history of fungal meningitis. On examination she was afebrile, alert, and oriented with a blood pressure of 110/70 mmHg. She had no adenopathy or neurological deficits. Her white blood cell count was minimally elevated. Magnetic resonance imaging revealed a 3.5 × 1.3 × 1.9 cm contrast enhancing lesion of the left temporal lobe with a mild midline shift. Evaluation by multiple specialists suggested a differential diagnosis of an infectious or neoplastic process. Cultures for infectious agents were negative. The biopsy showed CSH. Postoperatively and at 1 month follow up, she was neurologically intact.Conclusion:Radiographically and intraoperatively, CSH may mimic an infectious process or neoplasm. Its recognition is critical to facilitate appropriate therapy and prompt screening for an occult lymphoplasmacytic neoplasm, plasma cell dyscrasia or other underlying disease.

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Autoimmune dementias

McKeon¹,

Pittock²

2016

Non‐Alzheimer's and Atypical Dementia

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Central Nervous System Crystal-Storing Histiocytosis: Neuroimaging, Neuropathology, and Literature Review

Cited by 21 publications

References 5 publications

Crystal-storing histiocytosis: An unusual relapsing inflammatory CNS disorder

Crystal-storing histiocytosis: An unusual relapsing inflammatory CNS disorder

Crystal storing histiocytosis presenting as a temporal lobe mass lesion

Autoimmune dementias

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