Table 3.
Condition | Clinical Clues | Additional Investigations |
---|---|---|
Infections | ||
Enteroviruses
|
Anterior horn cell involvement ‐> paralysis +/‐Brainstem encephalitis |
RT‐PCR of cerebrospinal fluid |
Flaviviruses
|
‐Anterior horn cell involvement ‐> asymmetric paralysis (except Zika virus, which produces classic GBS) ‐Encephalitis ‐Travel history important ++ |
Virus‐specific IgM and IgG antibodies |
Lyssavirus
|
‐Prodromal pain in bitten limb ‐Early fasciculations ‐Rapidly progressive course |
RT‐PCR of saliva Rabies antibodies in CSF or serum |
HIV |
‐GBS occurs early ‐CNS signs occur late |
HIV p24 antigen, PCR viral load, ELISA or Western blot |
Herpesviruses ‐CMV ‐VZV |
CMV:
VZV:
|
Positive CSF PCR for CMV/VZV |
Lyme |
|
CSF and serum Lyme ELISA +/‐ confirmatory Western blot |
Other infections
|
|
Serum +/‐ CSF testing for individual pathogens |
Parasites:
|
|
Serum and CSF eosinophilia Specific diagnosis requires ELISA or immunoblot |
Immune | ||
Vasculitis |
SLE:
EGPA:
|
SLE:
EGPA
|
Neurosarcoidosis |
+/‐ Lung, joint, eye, skin, and lymph node involvement. |
CSF pleocytosis CSF ACE levels may be elevated Definite diagnosis: biopsy |
Sjogren syndrome |
‐F>>M(9:1 ratio) ‐Ocular and oral dryness ‐Musculoskeletal pain and fatigue +/‐pulmonary, renal or biliary tract involvement. Neuro: ‐Sensory neuropathy/neuronopathy |
Anti‐SSA and/or anti‐SSB antibodies Schirmer’s test ‐ ocular dryness Minor salivary gland biopsy |
Autoimmune encephalitides 1 ‐AMPA ‐anti‐NMDA ‐Anti‐CASPR2 |
+/‐ Neuropathy |
Autoimmune encephalitis antibody panel T2/FLAIR hyperintensities in medial temporal lobes CSF pleocytosis |
Inherited | ||
CMT 1X |
Triggers: infections, exertion, trauma, altitude. |
Genetics: GJB1 pathogenic variants |
ACE, angiotensin‐converting enzyme; AMPA, α‐amino‐3‐hydroxy‐5‐methyl‐4‐isoxazolepropionic acid; ANCA, Anti‐neutrophil cytoplasmic antibodies; CASPR2, Contactin‐associated protein‐like 2;CMT 1X, Charcot‐Marie‐Tooth type 1X; CMV, cytomegalovirus; CNS, central nervous system; CSF, cerebrospinal fluid; CXR, chest X‐ray; dsDNA, double‐stranded DNA; EGPA, eosinophilic granulomatosis with polyangiitis; ELISA, enzyme‐linked immuosorbent assay; EV, enterovirus; F, female; FLAIR, fluid‐attenuated inversion recovery; GBS, Guillain‐Barré Syndrome; HIV, human immunodeficiency virus; M, male; MPO, myeloperoxidase; NMDA, N‐Methyl‐ d‐aspartate; RT‐PCR, reverse transcription polymerase chain reaction; SLE, systemic lupus erythematosus; VZV, varicella zoster virus
Presentation usually largely dominated by central nervous system involvement. PNS signs rare, but occasionally reported