Neuromuscular

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INFECTIONS & NEUROMUSCULAR DISORDERS

Botulism
Brucellosis
Campylobacter jejuni
Central European encephalitis
Chagas'
Chikungunya
COVID-19
Cytomegalovirus
Dengue
Diphtheria
Enterovirus: D68; 71; IMPP
Erlichiosis: Human Granulocytic 
Haemophilus influenzae
Hepatitis C
Hepatitis E
Herpes Zoster
HIV
HTLV1
Leprosy
Leptospirosis
Lyme disease
Polio
Pyomyositis
Rabies
Sarcocystis
Syphilis
Tetanus
Tick
  African
  Paralysis
Toxoplasmosis
Trichinosis
Tuberculosis
Typhus
West Nile Virus
Zika

Myelopathies
Myopathy: Inflammatory


LYME DISEASE 15

  • Causative organism
    • US: Borrelia burgdorferi
    • Europe
      • Borrelia garinii: Associated with common neural involvement
      • Borrelia afzelii
      • B. burgdorferi sensu stricto
    • Type of organism: Spirochete
    • Vector: Bites of hard-shelled Ixodes ticks
      • Tick types: I. scapularis in United States; I. ricinus in Europe
      • Infections most common in late summer & autumn
      • Transmission requires 36 hours of tick attachment
        • Ingested blood triggers spirochete proliferation in tick gut
        • Spirochete is disseminated in tick
        • Spirochete is injected into host
  • Epidemiology
    • Age: Bimodal; Children (5 to 14 years); Adults (30 to 49 years)
    • Peak: Late Summer & Early Fall
    • Geography (US): 95% in
      • Eastern coastal states from District of Columbia to Vermont/New Hampshire
      • Wisconsin & Minnesota
      • Occasional cases in California
  • Clinical features
    • Skin rash: Early & local
      • Onset: Within 1 month of infection
      • Erythema migrans rash
        • Erythematous macule or papule
        • Not painful or pruritic
      • Progression
        • Centrifugally expanding
        • Spirochetes often present in leading edge
    • Acute disseminated disease: With systemic spread of spirochete
      • Dematogenous dissemiantion
        • Particularly with US strains
        • Often results in a multifocal rash
          • Each focus represents a separate nidus of metastatic infection
        • Other organs infected: Joints; Heart; Meninges & Peripheral nerve
      • General clinical featues during dissemination
        • Flu-like syndrome: Fever, Myalgias, Fatigue, Arthralgias, Headache
        • No associated upper respiratory or gastrointestinal symptoms
      • Meningoradiculitis (Bannwarth syndrome)
        • Headache, Meningismus
        • Radicular pain: Upper trunk & back
        • Focal weakness
        • Few sensory signs
        • Course: Relapsing
      • Neurologic
      • Cardiac: A-V block, Pericarditis, Dilated cardiomyopathy
      • Arthritis: Oligoarticular
        • Onset days to years after infection
        • Often relapsing - remitting
      • Occur in 60% of cases
      • Latency: 3 to 30 days
    • Neurologic manifestations (Neuroborreliosis)
      • Frequency: In 10% to 40% of Borrelia burgdorferi infections
      • Central Nervous System
        • Aseptic meningitis syndrome
        • Encephalomyelitis: Often with focal features & fatigue
        • Transverse myelitis
          • Early in disease course
          • Progressive over weeks
      • Focal disorders
        • Distribution: Cranial nerves; Peripheral nerves & Roots
        • Onset: Early (First 1 or 2 months) during infection
        • Acute onset
        • Often associated with lymphocytic meningitis.
        • Course
          • Usually self-limited
          • May resolve more rapidly with antibiotic treatment
      • Cranial nerve syndromes
        • Distribution
          • Facial (VII) most common
          • Multiple: 15% to 20%; Bilateral facial in 75%
          • Involvement of all cranial nerves except olfactory described
        • Frequency: 40% of neuroborreliosis
        • Facial palsy
          • In 10% of untreated patients
          • Unilateral or Bilateral
          • May not involve taste or hyperacusis
          • May be common cause of VII palsy in children in endemic areas
          • Full recovery in 85% with treatment
          • Borrelia burgdorferi antigen in CSF
          • May occur early in disease course
            • Some: Before antibody testing becomes positive
        • Other: Most often III, V & VI
      • Peripheral nerve syndromes
        • Multifocal radiculoneuropathy in acute disseminated stage
        • Chronic disease
          • Distal sensory-motor neuropathy
          • Carpal tunnel syndrome
          • More common in Europe & Elderly
          • Onset: 6 months to 8 years after infection
          • Nerve conduction: Axonal loss
          • Nerve biopsy: Perivascular inflammation
        • Late disease with Acrodermatitis chronica atrophicans (ACA) 4
          • Patient age: Average 70's; Range 55 to 84
          • Skin lesions
            • Bluish-red inflammatory skin lesion: Localized to acral extremities
            • Patients: Elderly female predominance
            • Exaggerated pain (Allodynia)
              • Location: Bony prominences underlying ACA lesions
            • Elevated serum IgG antibodies to B. burgdorferi
          • Polyneuropathy
            • Sensory loss: Especially vibration
            • Distribution: Symmetric, Distal
            • Mild
            • Motor: Normal
            • Electrophysiology: Axonal loss
            • Nerve pathology
              • Loss of large & small myelinated axons
              • Occasional perivascular inflammation
      • Lyme myositis 21
        • Frequency in Lyme disease: Unusual
        • Muscle involvement: Localized
          • Asymmetric
          • Swelling: Muscle & Limb
          • Painful
          • May be near cutaneous lesions
          • Ocular myositis: Some patients
          • Dermatomyositis-like disorder: Rare
        • Other clinical
          • Contiguous monoarthritis
          • Cardiac disorders
        • Laboratory
          • Serum CK: Usually normal
          • MRI of involved muscle: Diffuse T2 signal
          • Muscle biopsy: Inflammation
            • Location: Often near small blood vessels; Perimysial
            • Cell types
              • Monocellular, lymphoplasmacytic & histiocytic
              • Macrophages & T helper/inducer (CD4+) cells
            • Muscle fibers: Scattered or abundant necrosis
            • Perimysium: Thickened
            • Histologic staining for spirochetes
        • Treatment: Antibiotic regimens
  • Diagnosis
  • Laboratory
    • CSF: Mononuclear pleocytosis; Moderately elevated protein
    • Diagnosis
      • Culture: From edge of skin lesion; Blood culture is low yield
      • Serology: Variable specificity & sensitivity
        • ELISA confirmed by Western blot
        • Western blot: Binding to specific bands
        • Intrathecal Borrelia burgdorferi antibodies
          • Meningitis: 92%
          • Late CNS: 42%
        • Seronegativity (7%)
          • Early in disease course
          • Antibiotic treatment: Noncurative doses early in infection
          • Antibody complex
        • May not indicate ongoing infection
          • Positive serologic result may reflect infection or exposure in past
        • False-positive: May occur with
          • Response to microbiologically similar organisms
            • Relapsing fever
            • Syphilis
          • Nonspecific B-cell proliferation with systemic inflammation
            • Endocarditis, Bacterial
            • SLE
            • Vasculitis
          • Western blot
            • Can verify US results
            • European strains: Too much variability
      • DNA assay (PCR) of Borrelia burgdorferi antigen in body fluids
        • Often disappointing
  • Treatment
    • Early & Local disease: 21 days oral
      • Amoxicillin 500 mg to 1 g tid, or
      • Doxycycline 100 mg bid
        • NOTE: Not for children under 8 years, or pregnant or lactating women.
    • Other neurologic: Intravenous for 2 to 4 weeks
      • Ceftriaxone 1g bid (2 g per day) for 2 to 4 weeks
      • Cefotaxime 2 gms tid for 14-28 days
      • Penicillin 20 to 24 million units per day for 14-28 days
      • Chloramphenicol: Penicillin allergy
    • High-dose oral
      • Effective in European patients with meningitis or cranial neuritis
      • Doxycycline 200-400 mg/d for 21-42 days
    • Herxheimer reaction in 10% to 20%
  • Prognosis: Good recovery with (or rarely without) antibiotics
  • Animal model of neuroborreliosis
    • Rhesus macaque monkey: Often develops mononeuritis multiplex
  • External links

From: CDC; Other image
Ixodes dammini









From ALDF
Erythema
migrans

HERPES ZOSTER (Varicella) 2

Clinical features
  Neuralgia
  Radiculopathy
  Ramsay Hunt
  Mononeuropathy
  Other
Laboratory
Organism
Predisposing factors
Treatment




from CDC
Varicella zoster

Putnam

LEPROSY 6

Clinical features
  Acute
  Diffuse lepromatous
  Lepromatous
  Tuberculoid
  Borderline
  Neural
Diagnosis
Epidemiology
Organism
Treatment

Codex Aureus Epternacensis
(1020-1030 AD)

Christ healing the lepers

HUMAN IMMUNODEFICIENCY VIRUS (HIV)

General
Muscle
Nerve

from CDC
HIV



General principles
HIV & Neuropathies

ALS-variant syndrome
Autonomic
Brachial plexopathy
CIDP
Cytomegalovirus
DILS (CD8 lymphocytosis)
Drug-induced
Guillain-Barré
Mononeuritis multiplex
Mononeuropathies
Neuromyotonia
Sensory motor polyneuropathy
VII nerve palsy


HIV & Myopathies

Early HIV
  Immune myopathy
  Cytoplasmic body (Rod)
Advanced HIV
  Drug related
    Zidovudine
    Other
  Wasting
  Infectious
  Carnitine deficiency
  Necrotizing
  Adrenal insufficiency
IM-VAMP
  Pathology

CHAGAS' DISEASE


SYPHILIS: Tabes dorsalis


Joseph Grünpeck, Tractatus de pestilentiali scorra, 1496

from CDC
Treponema
pallidum

From Bramwell: Diseases of the Spinal Cord
Tabes dorsalis:
Pallor of posterior columns

"Thickening of spinal arachnoid": R Bright 1831

HUMAN GRANULOCYTIC ERLICHIOSIS 1


LEPTOSPIROSIS (Weil's disease)


BRUCELLOSIS (Undulant Fever)


HEPATITIS C 3, 5



WEST NILE VIRUS 7 *


IMPP (Myofasciitis; EVIM) + Deafness: Enterovirus & Hypogammaglobulinemia-Related 45


Acute Flaccid Myelitis, EV-D68 (Motor neuronopathy; AFM) 34


ENTEROVIRUS 71 31



CENTRAL EUROPEAN ENCEPHALITIS 8


PYOMYOSITIS 9


MRI: Leg

RABIES 10


TUBERCULOSIS 17

Inflamatory myopathy

AFRICAN TICK BITE FEVER 20


Dengue Virus 32


Scrub Typhus 33

Chikungunya virus 49


Zika virus 36


COVID-19 (SARS-CoV-2) virus 40

Clinical
  AIN
  Focal PN
Virus

Also see Infections & Myelopathies
Return to Neuropathy Differential Diagnosis
Return to Neuromuscular home page
References

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48. Neurology 2021 Jun 7

6/15/2021