Acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM) associated with choroidal melanoma

Abstract Background To report a case of acute exudative polymorphous paraneoplastic vitelliform maculopathy in a patient with a history of choroidal melanoma, with metastases to the pancreas, liver, and central nervous system. Case presentation A 63-year-old patient, with a history of enucleation of the right eye due to choroidal melanoma, complained of progressive visual loss during a follow-up visit. Fundoscopic examination revealed multiple small areas of serous retinal detachment scattered throughout the posterior pole and ancillary tests confirmed the diagnosis of acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM). Screening for systemic metastases showed pancreatic, hepatic, and central nervous system involvement. Conclusions We describe a rare case of acute exudative polymorphous paraneoplastic vitelliform maculopathy, which should be considered in patients with or without a history of melanoma, who have vitelliform retinal detachments. Nevertheless, no previous reviews of literature have shown a correlation between AEPPVM and pancreatic metastasis.

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Towards Central Nervous System Involvement in Adults with Hereditary Myopathies

Journal of Neuromuscular Diseases ◽

10.3233/jnd-200507 ◽

2020 ◽

Vol 7 (4) ◽

pp. 367-393

Author(s):

Jens Reimann ◽

Cornelia Kornblum

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Neuromuscular Disorders ◽

Clinical Manifestations ◽

Specific Treatment ◽

Central Nervous System Involvement ◽

Epileptic Seizures ◽

System Involvement ◽

History Of ◽

Neuroimaging Techniques

There is increasing evidence of central nervous system involvement in numerous neuromuscular disorders primarily considered diseases of skeletal muscle. Our knowledge on cerebral affection in myopathies is expanding continuously due to a better understanding of the genetic background and underlying pathophysiological mechanisms. Intriguingly, there is a remarkable overlap of brain pathology in muscular diseases with pathomechanisms involved in neurodegenerative or neurodevelopmental disorders. A rapid progress in advanced neuroimaging techniques results in further detailed insight into structural and functional cerebral abnormalities. The spectrum of clinical manifestations is broad and includes movement disorders, neurovascular complications, paroxysmal neurological symptoms like migraine and epileptic seizures, but also behavioural abnormalities and cognitive dysfunction. Cerebral involvement implies a high socio-economic and personal burden in adult patients sometimes exceeding the everyday challenges associated with muscle weakness. It is especially important to clarify the nature and natural history of brain affection against the background of upcoming specific treatment regimen in hereditary myopathies that should address the brain as a secondary target. This review aims to highlight the character and extent of central nervous system involvement in patients with hereditary myopathies manifesting in adulthood, however also includes some childhood-onset diseases with brain abnormalities that transfer into adult neurological care.

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Trigeminal ganglion involvement in sarcoidosis

JBNC - JORNAL BRASILEIRO DE NEUROCIRURGIA ◽

10.22290/jbnc.v20i4.901 ◽

2018 ◽

Vol 20 (4) ◽

pp. 439-441

Author(s):

Jairo Silva Dos Ângelos ◽

Guilherme Brasileiro De Aguiar ◽

Edgar Manuel Garcete Fariña ◽

Maud Parise ◽

Alexandre Martins Cunha ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Magnetic Resonance ◽

Parietal Cortex ◽

Central Nervous System Involvement ◽

Corticosteroid Treatment ◽

Unknown Etiology ◽

Histopathological Study ◽

Gasserian Ganglion ◽

The Right

Sarcoidosis is a disease of unknown etiology presenting as noncaseating granulomatous infiltration in tissues. Central Nervous system is rarely affected , usually as dissemination from other organs, mainly lungs. We report on a case in which the patient had only central nervous system involvement, presenting with a mass in the right gasserian ganglion and another lesion in right parietal cortex, observed in magnetic resonance. Diagnosis was given by histopathological study of the lesion and the patient improved with corticosteroid treatment.

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Peripheral neuropathies and motor neuron disorders

Oxford Textbook of Geriatric Medicine ◽

10.1093/med/9780198701590.003.0112_update_001 ◽

2017 ◽

pp. 871-878

Author(s):

Stéphane Mathis ◽

Jean-Michel Vallat

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Peripheral Nervous System ◽

Motor Neurons ◽

Central Nervous System Involvement ◽

Nerve Biopsy ◽

Motor Disorder ◽

Peripheral Neuropathies ◽

The Right ◽

Lateral Sclerosis

Peripheral nervous system disorders are common in older people. Most peripheral neuropathies are secondary to metabolic (diabetes) or toxic causes (medications, alcohol), but several other aetiologies are possible, requiring clinical, electrophysiological, biological, and sometimes pathological (nerve biopsy) examinations to make the right diagnosis and instigate effective treatments. However, despite using this strategy, no cause is found in some axonal polyneuropathies, leading to the concept of chronic idiopathic axonal polyneuropathy (CIAP). Amyotrophic lateral sclerosis (ALS) affects the peripheral nervous system, but is also characterized by central nervous system involvement: it is a strictly motor disorder affecting both the first and second motor neurons, usually presenting during the sixth or seventh decade. This rapidly progressive, still incurable neurodegenerative disease leads to death in all cases.

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Central Nervous System Demyelination in a Charcot-Marie-Tooth Type 1A Patient

Case Reports in Neurological Medicine ◽

10.1155/2013/243652 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 2

Author(s):

Christos Koros ◽

Maria-Eleftheria Evangelopoulos ◽

Costas Kilidireas ◽

Elisabeth Andreadou

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Molecular Mimicry ◽

Central Nervous System Involvement ◽

Laboratory Findings ◽

Charcot Marie Tooth ◽

Demyelinating Disorder ◽

History Of ◽

Tooth Type ◽

Simultaneous Existence

Introduction. Central nervous system involvement, either clinical or subclinical, has been reported mainly in X-linked Charcot-Marie-Tooth (CMT-X) patients.Case Presentation. We present the case of a 31-year-old man with a genetically confirmed history of CMT1A who developed CNS involvement mimicking multiple sclerosis (MS). Clinical, imaging, and laboratory findings suggested an autoimmune CNS demyelination.Discussion. Although the simultaneous existence of CMT1A and MS could be coincidental we postulate that overexpression of PMP22, the target protein in CMT1A, might influence the immunological self-tolerance to CNS proteins via molecular mimicry, leading to a CNS autoimmune demyelinating disorder.

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Peripheral neuropathies and motor neuron disorders

Oxford Textbook of Geriatric Medicine ◽

10.1093/med/9780198701590.003.0112 ◽

2017 ◽

pp. 871-878

Author(s):

Stéphane Mathis ◽

Pierre Bouche ◽

Jean-Michel Vallat

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Peripheral Nervous System ◽

Motor Neurons ◽

Central Nervous System Involvement ◽

Nerve Biopsy ◽

Motor Disorder ◽

Peripheral Neuropathies ◽

The Right ◽

Lateral Sclerosis

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A 75-Year-Old Man With 5 Days of Progressive Gait Difficulties and Confusion

10.1093/med/9780197583425.003.0063 ◽

2021 ◽

pp. 195-198

Author(s):

Julie E. Hammack

Keyword(s):

Central Nervous System ◽

Nervous System ◽

B Cell ◽

Blood Cells ◽

Central Nervous System Lymphoma ◽

High Dose ◽

Normal Glucose ◽

History Of ◽

The Right ◽

Large B Cell

A 75-year-old man with a history of chronic obstructive pulmonary disease and ischemic cardiomyopathy was brought to the emergency department after a fall at home. He had a 5-day history of progressive gait disturbance, right-sided weakness, and confusion. He had reported floaters in the right eye for the past month. He was previously well with no history of trauma, fever, anorexia, or change in body weight. Cerebrospinal fluid evaluation showed an increased protein concentration, normal glucose level, no red blood cells, and 4 white blood cells/µL. Cytologic and flow cytometry evaluations were negative. Computed tomograph of the chest, abdomen, and pelvis indicated no adenopathy or visceral lesions. Slitlamp examination of the right eye showed clumps of cells in the vitreous. Vitrectomy was performed, and analysis showed atypical monoclonal B cells consistent with large B-cell lymphoma. The patient was diagnosed with primary central nervous system lymphoma. The patient had an excellent initial clinical response to intravenous corticosteroids (dexamethasone) administered after vitrectomy. Subsequent staging showed no systemic lymphoma. He had hematology-oncology evaluations and was treated with chemotherapy (high-dose intravenous methotrexate, temozolomide, and rituximab) together with intravitreal rituximab for 1 year. He had an excellent clinical and radiographic response to treatment. He remained in complete remission until his death 6 years later (age 81 years) of pneumonia. Primary central nervous system lymphoma accounts for approximately 4% of primary brain tumors and occurs more commonly in persons older than 60 years and those with compromised immune systems. The tumor represents an extranodal form of non-Hodgkin lymphoma and is typically of the diffuse large B-cell type.

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Surgical Resection of Isolated Intramedullary Neurocysticercosis

JBNC - JORNAL BRASILEIRO DE NEUROCIRURGIA ◽

10.22290/jbnc.v26i3.1343 ◽

2018 ◽

Vol 26 (3) ◽

pp. 223-226

Author(s):

Leonardo Gilmone Ruschel ◽

Kristel Back Merida ◽

Guilherme José Agnoletto ◽

Guilherme Augusto de Souza Machado ◽

Felipe Andrés Constanzo Navarrete ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Taenia Solium ◽

Central Nervous System Involvement ◽

Underdeveloped Countries ◽

Perilesional Edema ◽

Intracranial Involvement ◽

History Of ◽

The Central Nervous System ◽

Magnetic Resonance Imaging Mri

Introduction: Neurocysticercosis is a parasitic disease caused by the cysticerci of Taenia solium. Central nervous system involvement is endemic in underdeveloped countries and intracranial involvement is the most common presentation. Isolated spinal cord involvement is quite rare and definitive diagnosis in most cases is obtained by anatomopathological evaluation. Case Report: 58 year-old female, from Nicaragua, with a 3-year history of dysesthesia and allodynia on right T5 dermatome, with thoracic spine magnetic resonance imaging (MRI) revealing an intramedullary cystic lesion at the level of T4 and T5 vertebrae with perilesional edema. MRI of the rest of the central nervous system was unremarkable. After initial empirical treatment with intravenous methylprednisolone without clinical or radiological improvement, surgical exploration was proposed, confirming the diagnosis of neurocysticercosis. Conclusion: Diagnosing spinal cysticercosis represents a challenge and should to be considered in patients with intramedullary cystic lesions in endemic areas.

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Neurosarcoidosis with Intraparenchymal Cystic Lesions

The Neuroradiology Journal ◽

10.1177/197140090802100610 ◽

2008 ◽

Vol 21 (6) ◽

pp. 810-816 ◽

Cited By ~ 3

Author(s):

F.A. Al Hajri ◽

A.T. Muqim ◽

T.J.E. Muttikkal

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Rare Case ◽

Clinical Manifestations ◽

Central Nervous System Involvement ◽

Unknown Etiology ◽

Cystic Lesions ◽

Imaging Appearance ◽

Granulomatous Disorder ◽

The Right

Sarcoidosis is a chronic multi-system granulomatous disorder of unknown etiology. Central nervous system involvement is relatively uncommon in sarcoidosis. Clinical manifestations and radiological appearances of neurosarcoidosis vary widely depending on the site and activity of the lesions. In most cases, the imaging appearance is nonspecific. We report a very rare case of extensive neurosarcoidosis with progressively enlarging cystic lesions in the right temporal lobe.

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Central Nervous System Vasculitis as a Rare Presentation of Mycoplasma pneumoniae: A Case Report

Case Reports in Neurology ◽

10.1159/000510632 ◽

2020 ◽

Vol 12 (3) ◽

pp. 402-409

Author(s):

Ashraf Omer Elamin Ahmed ◽

Mona Mohammad Ibraheem Babikir ◽

Amir Elssoni Mahjoup Khojali ◽

Suresh Nalaka Menik Arachchige ◽

Abdirahman Mohamud Abdirahman ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Lower Respiratory Tract ◽

Respiratory Tract Infections ◽

Central Nervous System Involvement ◽

Lower Respiratory Tract Infections ◽

Central Nervous System Vasculitis ◽

Rare Presentation ◽

Systemic Involvement ◽

Tract Infections

<i>Mycobacteria pneumoniae</i> (MP) commonly causes upper and lower respiratory tract infections. The clinical manifestation is classified as pulmonary and extrapulmonary. These manifestations vary according to the involved system. MP may affect one system or more at a time. Commonly prodromal respiratory symptoms precede systemic involvement. Central nervous system involvement in uncommon. This report is presenting a rare case of central nervous system vasculitis secondary to MP, highlighting the diagnosis and management with a succinct literature review.

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Treatment of secondary central nervous system involvement in systemic aggressive B cell lymphoma using R-MIADD chemotherapy: a single-center study

Chinese Neurosurgical Journal ◽

10.1186/s41016-021-00238-0 ◽

2021 ◽

Vol 7 (1) ◽

Author(s):

Yuchen Wu ◽

Xuefei Sun ◽

Xueyan Bai ◽

Jun Qian ◽

Hong Zhu ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Central Nervous System Involvement ◽

Central Nervous System Lymphoma ◽

Progression Free Survival ◽

B Cell Lymphoma ◽

High Dose ◽

Cns Involvement ◽

The Central Nervous System ◽

Secondary Central Nervous System

Abstract Background Secondary central nervous system lymphoma (SCNSL) is defined as lymphoma involvement within the central nervous system (CNS) that originated elsewhere, or a CNS relapse of systemic lymphoma. Prognosis of SCNSL is poor and the most appropriate treatment is still undetermined. Methods We conducted a retrospective study to assess the feasibility of an R-MIADD (rituximab, high-dose methotrexate, ifosfamide, cytarabine, liposomal formulation of doxorubicin, and dexamethasone) regimen for SCNSL patients. Results Nineteen patients with newly diagnosed CNS lesions were selected, with a median age of 58 (range 20 to 72) years. Out of 19 patients, 11 (57.9%) achieved complete remission (CR) and 2 (10.5%) achieved partial remission (PR); the overall response rate was 68.4%. The median progression-free survival after CNS involvement was 28.0 months (95% confidence interval 11.0–44.9), and the median overall survival after CNS involvement was 34.5 months. Treatment-related death occurred in one patient (5.3%). Conclusions These single-centered data underscore the feasibility of an R-MIADD regimen as the induction therapy of SCNSL, further investigation is warranted.

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