"Lupus? Is it Lupus?"
This neurologic manifestation of lupus is characterized by headaches, confusion, seizures, and strokes.
What is neuropsychiatric lupus?
RA is associated with positive rheumatoid factor and this antibody, which has higher specificity than RF
What is cyclic citrullinated peptide antibodies? Identical high sensitivity, specificity 95% vs 98%
This percentage of Sjogren's patients have PNS involvement
What is 16%? (will accept +/- 5, so 11-21% acceptable) Fun fact, CNS involvement is reported at 4%
This systemic vasculitis is highlighted by headache, limb-girdle polyarthralgia, and in severe cases vision-loss from arteritic anterior ischemic optic neuropathy.
What is giant cell arteritis (GCA or TCA)?
Peripheral nerve disorders occur at what rates in patients with SLE?
What is 6-14%? Will accept 5-15%. 40% of cases are not due to SLE. Often presents as dustal symmetric axonal neuropathy. AIDP and CIDP has been seen in rare cases.
This medication is the preferred anticoagulant in antiphospholipid syndrome
What is warfarin? DOACSs are non-inferior in venous thromboembolism, but show increased risk for recurrent arterial thromboembolism
This entrapment syndrome has a 2 fold increase of occurence in RA patients
What is carpal tunnel syndrome? RA patients have a higher incidence of entrapment neuropathies
Out of the following options, pick the objective measures that show the highest sensitivity and specificity for diagnosis of Sjogren's Disease: anti-Ro/SSA, anti-La/SSB, Schirmer test, salivary gland biopsy
What are Schirmer test and salivary gland biopsy? Objective measures of exocrine gland function have higher specificity and sensitivity. SSA/SSB were described as having "limited" sensitivity and specificity.
This is the most common manifestation of vasculitic neuropathy
What is mononeuritis multiplex? This is a consideration in all systemic vasculitis
These antibodies are detected in at higher rates in patients with SLE who have objective cognitive impairment.
What are APLA (accept cardiolipin or Beta-2 glycoprotein Abs as well)?
Acute psychosis occurs in this percentage of SLE patients, usually early in the disease course
What is 1.5%? (will accept +/- 1%) Suspected to be immune mediated, MRI frequently unremarkable, no reliable biomarkers are available, CSF can range from normal to mild pleocytosis, elevated IgG index, and elevated CSF-unique oligoclonal bands
Neck pain, C2 radiculopathy, occipital headache, and (in severe cases) myelopathy are manifestations of this most common neurological process in RA?
What is cervical spinal stenosis? RA causes synovitis and pannus formation at the atlantoaxial, atlanto-occipital and subaxial levels which can cause all of these symptoms
These two PNS localizations are the most distinctly involved in manifestations of Sjogren's Disease
What are the dorsal root ganglion and peripheral nerves? (must specify small fiber neuropathy if given the choice) Small fiber neuropathy presents as asymmetric dyesthetic pain (with decreased temp and PP) in length dependent or non-length dependent fashion
High suspicion should be present for this ANCA-positive vasculitis in a patient with adult-onset asthma, eosinophilia, and multifocal neuropathy
What is eosinophilic granulomatosis with polyangiitis?
This type of abnormal movement can be seen rarely in SLE, thought to be a result of basal ganglia stroke
What are choreiform movements?
SLE patients are at higher risk for this rare neurological syndrome, which is both a byproduct of SLE but also a complication of comorbid renal disease and immunosuppression
What is posterior reversible encephalopathy syndrome (PRES)? Occurs in 0.7-1.8% of patients with SLE
Also: focal and generalized seizures have a reported frequency anywhere from 4-35% of SLE patients
Though rare, this diagnosis is well-described in patients with RA where-in they present with headaches, focal neuro deficits, AMS, and have isolated leptomeningeal or pachymeningeal enhancement on MRI with mild lymphocytic pleocytosis?
What is aseptic meningitis? Can also have both lepto- and pachymeningeal enhancement. CSF can sometimes have mild neutrophilic or monocytic pleocytosis. Can occur in absence of systemic disease activity. In cases of chronic meningitis, get a cyclic citrullinated peptide antibody. Rheumatoid meningitis has been seen in RA patients on infliximab. TNF-alpha inhibitors can also cause MS-like episodes of demyelination and CIDP-like episodes of demyelination
A Sjogren's patient presents with with areflexia, non-length dependent pan-modality sensory loss, and sensory ataxia. Her EMG will shows preserved CMAPs and absent to low-amplitude SNAPs. What additional MRI spine findings can sometimes be seen in association?
What are T2 hyperintensity in the dorsal spinal column? This patient has dorsal root ganglionopathy and there can be T2 hyperintensities secondary to sensory ganglia neuron injury.
This is the most common CNS manifestation of ANCA-positive vasculitides
This acquired and sometimes familial syndrome is characterized by macrophage and lymphocyte mediated hyperinflammation. The acquired form arises from underlying infection, lymphoid malignancy, or rheumatological disease. Neurologically can manifest as encephalitis, seizures, and PRES.
What is hemophagocytic lymphohistiocytosis? Familial is usually infant or early childhood onset. Acquired is older child or adult onset. Fever, cytopenias, hepatitis, splenomegaly, hyperferritinemia, and impaired NK cell activity, and increassed soluble IL-2 alpha receptors.
Demyelinating disease can be seen in 1% of SLE patients, commonly associated with comorbid presence of this spectrum of disorders
What are neuromyelitis optica spectrum disorders? While it can be a direct result of SLE, there can also be comorbid neural-specific autoimmunity. Myelitis in SLE can be WM or GM. The WM-associated lesions have a higher rate of aquaporin-4 ab positivity. GM might be more related to SLE or even MOG
Aseptic meningitis can be seen in this rheumatological disease (that is in no way associated with RA) wherein recurrent inflammation occurs of cartilaginous structures (ears, nose, peripheral joints, tracheobronchial tree) as well as the eyes, inner ear, and cardiovascular system.
What is Relapsing Polychondritis? Occurs in 3% of patients, can also have T2 hyperintensities in subcortical WM, BG, or limbic structures. No serological labs to diagnose it, just good ole history.
This type of brain lesion has been reported in Sjogren's Disease (think Marburg but less fulminant)
What are focal tumefactive demyelinating brain lesions? Can also have NMOSD type pattern that usually has AQP-4 Ab positivity that reflects overlapping disease rather than a direct association to Sjogren's
Remember: there is strong association with CNS demyelinating disease with SLE and Sjogren's (whether overlap or directly related)
This variable-vessel vasculitis has a higher incidence of neurological involvement in males (5-10%), and when presenting as a parenchymal disease (as opposed to vascular) presents as a progressive brainstem syndrome (with or w/o encephalopathy), with headache, and with sphincter dysfunction.
What is Behcets or neuro-Behcets? Lesions can be seen in the brainstem, diencephalon, BG, and cerebellum. Vascular subtype usually results in CVST and systemic venous thrombosis.
AA amyloidosis (amyloid A deposition) is a long-term complication of this inherited autoinflammatory syndrome with relapsing fever, serositis, and onset before 20 years of age in 90% of patients.
What is Familial Mediterranean Fever? MEFV gene mutation