Consensus Paper: Latent Autoimmune Cerebellar Ataxia (LACA)

Manto, Mario; Hadjivassiliou, Marios; Baizabal‐Carvallo, José Fidel; Hampe, Christiane S.; Honnorat, Jérôme; Joubert, Bastien; Mitoma, Hiroshi; Muñiz‐Castrillo, Sergio; Shaikh, Aasef G.; Vogrig, Alberto

doi:10.1007/s12311-023-01550-4

Cited by 11 publications

(6 citation statements)

References 135 publications

(168 reference statements)

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“…It has since come to be recognised as a distinct disease after the discovery of autoantibodies. It possesses the characteristics of both Type 1 and Type 2 DM 7 and the presence of autoantibodies in LADA patients suggests an autoimmune origin. Because of its similarities to both categories of diabetes, the diagnosis of LADA can frequently be concealed.…”

Section: Discussionmentioning

confidence: 99%

Association of latent autoimmune diabetes of adults with type 3 polyglandular autoimmune syndrome—a diagnostic challenge

Urooj Lal Rehman,

Hafiz Muhammad Hamza,

Muhammad Muiz Malik

et al. 2024

J Pak Med Assoc

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Autoimmune polyendocrine syndromes (APS) encompass multiple endocrine gland insufficiencies associated with autoimmune disease. This case report underscores the importance of recognising the association between latent autoimmune diabetes of adults (LADA) and type 3 polyglandular syndrome. A 42-year-old man belonging to Rawalpindi, Pakistan, presented to the outpatient department (OPD) of Ali Medical Centre, Islamabad, in January 2023 with the complaints of extreme thirst and frequent urination. The patient reported consistently raised appetite and eating four to five meals a day along with abrupt weight loss, dry mouth, fatigue occasional dizziness, and dyspnoea. He was diagnosed with type 3 polyglandular syndrome with association of LADA. Daily administration of 10 units of glargine insulin, along with six units of rapid-acting insulin, was prescribed. The patient's HbA1c level reduced in a few months after successive follow-up. ---Continue

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Section: Discussionmentioning

confidence: 99%

Association of latent autoimmune diabetes of adults with type 3 polyglandular autoimmune syndrome—a diagnostic challenge

Urooj Lal Rehman,

Hafiz Muhammad Hamza,

Muhammad Muiz Malik

et al. 2024

J Pak Med Assoc

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“…Recently, Manto and colleagues have proposed the new concept of latent autoimmune cerebellar ataxia (LACA) in analogy with the latent autoimmune diabetes in adults (LADA) to underline the subtle disease course of immune-mediated ataxias, including PCD [38]. LADA is a form of type II diabetes mellitus with autoimmune features, the serum biomarker (anti-GAD antibody) is not always present or can fluctuate and tends to progress with a slow pattern [38]. The disease inevitably progresses until complete pancreatic beta-cell failure within a few years.…”

Section: Rapidly Progressive Cerebellar Syndromementioning

confidence: 99%

“…The disease inevitably progresses until complete pancreatic beta-cell failure within a few years. Due to the unclear autoimmune profile, it is difficult to achieve the diagnosis in the early phase before insulin production is altered [38]. LACA has some analogies with LADA, it has a slow progression, paucity of clear-cut autoimmune features, with significant difficulty for the neurologist to achieve the diagnosis in absence of positive and significantly elevated antibody titers [38].…”

Section: Rapidly Progressive Cerebellar Syndromementioning

confidence: 99%

“…Due to the unclear autoimmune profile, it is difficult to achieve the diagnosis in the early phase before insulin production is altered [38]. LACA has some analogies with LADA, it has a slow progression, paucity of clear-cut autoimmune features, with significant difficulty for the neurologist to achieve the diagnosis in absence of positive and significantly elevated antibody titers [38]. There are some subtle neurological features which could help clinicians towards the autoimmune and paraneoplastic nature of the cerebellar syndrome, in the early phase, before PCD overtly manifests [38].…”

Section: Rapidly Progressive Cerebellar Syndromementioning

confidence: 99%

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Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

et al. 2023

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Paraneoplastic neurological syndromes (PNS) include any symptomatic and non-metastatic neurological manifestations associated with a neoplasm. PNS associated with antibodies against intracellular antigens, known as “high-risk” antibodies, show frequent association with underlying cancer. PNS associated with antibodies against neural surface antigens, known as “intermediate- or low-risk” antibodies, are less frequently associated with cancer. In this narrative review, we will focus on PNS of the central nervous system (CNS). Clinicians should have a high index of suspicion with acute/subacute encephalopathies to achieve a prompt diagnosis and treatment. PNS of the CNS exhibit a range of overlapping “high-risk” clinical syndromes, including but not limited to latent and overt rapidly progressive cerebellar syndrome, opsoclonus-myoclonus-ataxia syndrome, paraneoplastic (and limbic) encephalitis/encephalomyelitis, and stiff-person spectrum disorders. Some of these phenotypes may also arise from recent anti-cancer treatments, namely immune-checkpoint inhibitors and CAR T-cell therapies, as a consequence of boosting of the immune system against cancer cells. Here, we highlight the clinical features of PNS of the CNS, their associated tumors and antibodies, and the diagnostic and therapeutic strategies. The potential and the advance of this review consists on a broad description on how the field of PNS of the CNS is constantly expanding with newly discovered antibodies and syndromes. Standardized diagnostic criteria and disease biomarkers are fundamental to quickly recognize PNS to allow prompt treatment initiation, thus improving the long-term outcome of these conditions.

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“…Detailed studies on the cerebellar reserve in IMCAS are warranted as they may be of neurobiological importance to a large range of neurological/neuropsychiatric conditions [55,56]. This is also applicable to latent autoimmune cerebellar ataxia (LACA), which is characterized by a slowly progressive course, the lack of an obvious autoimmune background, and difficulties in reaching a diagnosis in the absence of biomarkers for IMCAs [57,58]. IMCAs have now entered into daily neurological practice [59,60].…”

mentioning

confidence: 99%

Recent Advances in Immune-Mediated Cerebellar Ataxias: Pathogenesis, Diagnostic Approaches, Therapies, and Future Challenges—Editorial

Manto,

Mitoma

2023

Brain Sciences

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The clinical category of immune-mediated cerebellar ataxias (IMCAs) has been established after 3 decades of clinical and experimental research. The cerebellum is particularly enriched in antigens (ion channels and related proteins, synaptic adhesion/organizing proteins, transmitter receptors, glial cells) and is vulnerable to immune attacks. IMCAs include various disorders, including gluten ataxia (GA), post-infectious cerebellitis (PIC), Miller Fisher syndrome (MFS), paraneoplastic cerebellar degeneration (PCD), opsoclonus myoclonus syndrome (OMS), and anti-GAD ataxia. Other disorders such as multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), Behçet disease, and collagen vascular disorders may also present with cerebellar symptoms when lesions are localized to cerebellar pathways. The triggers of autoimmunity are established in GA (gluten sensitivity), PIC and MFS (infections), PCD (malignancy), and OMS (infections or malignant tumors). Patients whose clinical profiles do not match those of classic types of IMCAs are now included in the spectrum of primary autoimmune cerebellar ataxia (PACA). Recent remarkable progress has clarified various characteristics of these etiologies and therapeutic strategies in terms of immunotherapies. However, it still remains to be elucidated as to how immune tolerance is broken, leading to autoimmune insults of the cerebellum, and the consecutive sequence of events occurring during cerebellar damage caused by antibody- or cell-mediated mechanisms. Antibodies may specifically target the cerebellar circuitry and impair synaptic mechanisms (synaptopathies). The present Special Issue aims to illuminate what is solved and what is unsolved in clinical practice and the pathophysiology of IMCAs. Immune ataxias now represent a genuine category of immune insults to the central nervous system (CNS).

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Consensus Paper: Latent Autoimmune Cerebellar Ataxia (LACA)

Cited by 11 publications

References 135 publications

Association of latent autoimmune diabetes of adults with type 3 polyglandular autoimmune syndrome—a diagnostic challenge

Association of latent autoimmune diabetes of adults with type 3 polyglandular autoimmune syndrome—a diagnostic challenge

Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

Recent Advances in Immune-Mediated Cerebellar Ataxias: Pathogenesis, Diagnostic Approaches, Therapies, and Future Challenges—Editorial

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