40 Questions
What percentage of patients with NMDAR encephalitis initially receive a primary psychiatric diagnosis?
25%
Which of the following is a common prodrome of autoimmune encephalitis?
Viral-like symptoms
What is the approximate percentage of patients with autoimmune encephalitis who have a full or substantial recovery in response to immunotherapies?
80%
Which of the following autoimmune encephalitis syndromes has been associated with specific HLA associations?
All of the above
What is the potential relationship between autoimmune encephalitis and demyelinating disorders?
They may overlap in some patients
What is a common misattribution of symptoms in patients with autoimmune encephalitis?
Antipsychotic medication side effects
Which of the following is a characteristic of autoimmune encephalitis with NMDAR antibodies?
Predominantly affects young adults, teenagers, and children
What is a potential trigger for autoimmune encephalitis?
Herpes simplex viral encephalitis
What is the diagnostic tool that reveals multifocal hyperfluorescence corresponding with patches in autoimmune encephalitis?
Fluorescein angiography
What is the underlying mechanism of the melanocytic proliferation in autoimmune encephalitis?
Not fully elucidated
Which of the following is NOT a type of autoimmune encephalitis?
Multiple Sclerosis
What is the primary treatment approach for autoimmune encephalitis?
Immunotherapy
Which of the following is an immunological trigger for autoimmune encephalitis?
Autoantibody production
Which of the following syndromes is characterized by anti-DPPX antibodies?
DPPX Encephalitis
What is a potential laboratory finding in autoimmune encephalitis?
Inflammatory signs in cerebrospinal fluid
Which of the following autoimmune encephalitis syndromes may overlap with demyelinating disorders?
All of the above
What is a common finding in the cerebrospinal fluid (CSF) of patients with autoimmune encephalitis?
Pleocytosis
Which of the following neuroimaging findings is commonly seen in patients with autoimmune encephalitis?
Increased FLAIR signal in medial temporal lobes
What is a characteristic feature of the neuropsychiatric syndrome seen in autoimmune encephalitis?
Prominent psychiatric and cognitive dysfunction
What is the approximate frequency of CSF abnormalities in patients with autoimmune encephalitis?
Over 50%
Which of the following is a common symptom of autoimmune encephalitis?
Seizures
What is a characteristic feature of brain MRI in patients with GABAA receptor antibodies?
FLAIR abnormalities involving multiple cortical and subcortical regions
Which of the following is NOT a common feature of autoimmune encephalitis?
Oculomotor abnormalities
What is a common characteristic of the neuropsychiatric syndrome in autoimmune encephalitis?
Viral-like prodrome
What is the typical symptom presentation of patients with Waldenström macroglobulinemia?
Asymmetrical and associated with pain
Which of the following electrophysiological studies is likely to show evidence of axonal and demyelinating features in patients with Waldenström macroglobulinemia?
Nerve conduction studies (NCS)
What is the primary immunological target of IgM paraprotein in patients with Waldenström macroglobulinemia?
Myelin-associated glycoprotein
Which of the following therapeutic approaches is likely to be effective in treating the neuropathy associated with Waldenström macroglobulinemia?
Plasma exchange, IVIG, rituximab, chlorambucil, cyclophosphamide, or fludarabine
What is the primary characteristic of the neuropathy associated with Waldenström macroglobulinemia?
Symmetrical, demyelinating, sensorimotor neuropathy
What is the most common initial symptom of patients with Waldenström macroglobulinemia?
Muscle weakness in the legs
Which of the following is a common feature of POEMS syndrome?
All of the above
What is the characteristic pattern of weakness in patients with Waldenström macroglobulinemia?
Proximal weakness in the legs and arms
What is a characteristic of CASPR2 autoimmune encephalitis?
Limbic encephalitis, neuropathic pain, peripheral nerve hyperexcitability
In GABAA receptor autoimmune encephalitis, what is the frequency of coexisting autoimmunities?
About 70% of patients
What is the response to immunotherapy in GABAA receptor autoimmune encephalitis?
Half of patients have a good response
Which of the following symptoms is LESS commonly seen in DPPX autoimmune encephalitis?
Fever
What is the outcome of immunotherapy in CASPR2 autoimmune encephalitis?
About 70% of patients have a full or substantial recovery
Which autoimmune encephalitis syndrome is characterized by a protracted course with relapses when immunotherapy is reduced?
DPPX autoimmune encephalitis
What is a characteristic of GABAA receptor autoimmune encephalitis on MRI?
Frequent cortical-subcortical abnormalities
In DPPX autoimmune encephalitis, what is the outcome of immunotherapy?
Partial but meaningful improvement
Study Notes
Autoimmune Encephalitis with Antibodies to Cell Surface Antigens
- A group of neuropsychiatric disorders characterized by antibodies against neuronal cell surface/synaptic antigens
- Presents with cognitive impairment, altered behavior, decreased consciousness, and movement disorders
- Over half of patients have CSF abnormalities, including pleocytosis, increased proteins, and/or oligoclonal bands
Anti-NMDAR Encephalitis
- Presents with psychiatric symptoms, seizures, memory deficits, decreased level of consciousness, dyskinesias, and fever
- Can be asymmetrical and associated with pain, resulting in gait disturbance
- Almost 80% of cases have full or substantial recovery with immunotherapy
Anti-LGI1 Limbic Encephalitis
- Presents with limbic encephalitis, neurologic pain, peripheral nerve hyperexcitability, and Morvan syndrome
- Frequent coexisting autoimmunities (e.g., TPO, GAD antibodies)
- About 70% have full or substantial recovery
Anti-CASPR2 Associated Encephalitis
- Presents with neurologic pain, peripheral nerve hyperexcitability, and Morvan syndrome
- Frequent coexisting autoimmunities
- About 70% have full or substantial recovery
Anti-GABAB Receptor Encephalitis
- Presents with rapid progressive, severe encephalopathy with refractory seizures
- Extensive MRI FLAIR/T2 cortical-subcortical abnormalities
- Frequent coexisting autoimmunities (e.g., TPO, GAD antibodies)
- Half of patients have good response to immunotherapy, but patients may die from medical complications during status epilepticus
Anti-DPPX Encephalitis
- Presents with agitation, paranoia, hallucinations, tremor, myoclonus, and/or seizures
- Less often, cerebellar signs, hyperekplexia, or PERM-like syndrome
- Protracted course with relapses when immunotherapy is reduced
- Partial but meaningful improvement
Anti-mGluR5 Encephalitis
- Presents with antibodies against mGluR5, a subtype of metabotropic glutamate receptors
- No specific clinical features or treatment outcomes mentioned
Anti-mGluR1 Cerebellar Dysfunction
- Presents with cerebellar dysfunction
- No specific clinical features or treatment outcomes mentioned
Anti-Dopamine Receptor Encephalitis
- Presents with antibodies against dopamine receptors
- No specific clinical features or treatment outcomes mentioned
Anti-neurexin 3α Encephalitis
- Presents with antibodies against neurexin 3α, a neuronal cell adhesion molecule
- No specific clinical features or treatment outcomes mentioned
Anti-IgLON5 Disease
- Presents with antibodies against IgLON5, a neuronal cell surface antigen
- No specific clinical features or treatment outcomes mentioned
General Treatment Recommendations
- Immunotherapy is the primary treatment for autoimmune encephalitis
- Prompt recognition and treatment are essential for optimal outcomes
- Multidisciplinary approach, including neurologists, psychiatrists, and immunologists, is recommended for optimal management
This quiz covers the neurological symptoms of Waldenström macroglobulinemia, including axonal and demyelinating features, electrophysiological studies, and sensorimotor neuropathy. Learn about the effects of IgM proteins on nerve function and the resulting symptoms.
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