Hypoglycorrhachia Differential Diagnosis

Common and Uncommon Etiologies of Hypoglycorrhachia in the LiteratureCapture

ETIOLOGIES COMMONLY ASSOCIATED WITH HYPOGLYCORRHACHIA

  • Bacterial meningitis (including atypical bacteria like nocardia and brucella)
  • Fungal meningitis
  • Mycobacterial (tuberculous meningitis)
  • Amebic meningoencephalitis
  • CMV-associated progressive polyradiculopathy or meningoencephalitis
  • Carcinomatous meningitis
  • GLUT 1-deficiency syndrome
  • Leukemia/lymphoma with CNS involvement
  • Subarachnoid hemorrhage

 

ETIOLOGIES UNCOMMONLY ASSOCIATED WITH HYPOGLYCORRHACHIA

  • Syphilitic meningitis
  • Lyme meningitis
  • Viral meningitis
  • Neurocysticercosis5
  • CNS toxoplasmosis
  • Cholesterol-induced leptomeningitis secondary to Currarino syndrome
  • Neurosarcoidosis
  • Rheumatoid meningitis
  • Systemic lupus erythematosus with CNS involvement
  • Neuro-Behcet’s Disease
  • Dermoid cyst
  • Granulomatous angiitis of the central nervous system
  • Malignant atrophic papulosis

 

Etiologies reported to cause severe hypoglycorrhachia, (CSF glu ≤10 mg/dL)

  • Bacterial meningitis (including atypical bacteria like nocardia and brucella) *
  • Fungal meningitis*
  • Mycobacterial (tuberculous meningitis)*
  • Carcinomatous meningitis*
  • Leukemia/lymphoma with CNS involvement*
  • Subarachnoid hemorrhage*
  • Lyme meningitis*
  • Neurocysticercosis5*
  • Cholesterol-induced leptomeningitis secondary to Currarino syndrome*
  • Neurosarcoidosis*
  • Dermoid cyst*

 

Frequency of Different Known Diagnoses Seen in Patients with Hypoglycorrhachia

  1. All Patients

1

2. HIV-Infected Patients

2

3. Patients with History of Neurosurgery

3

4. Patients without HIV or Neurosurgical History

4

 

Reference:

Chow, E., & Troy, S. (2014). The Differential Diagnosis of Hypoglycorrhachia in Adult Patients. The American Journal Of The Medical Sciences348(3), 186-190. doi: 10.1097/maj.0000000000000217

 

Checklist: Bleed post TPA

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Half life of TPA is ~5 minutes and only 20% is present and active 10 mins after completion of infusion, but PT and PTT prolongation and fibrinogen levels are decreased x 24 hours or more.

Checklist:

  • STOP alteplase
  • VS q15h, GCS, pupil response, treat BP, increased ICP
  • Neurosurgery consult
  • DIAGNOSTICS: STAT CT head, PT/PTT, platelets, fibrinogen, type and cross 2-4 unit pRBC
  • THERAPEUTICS:
  1. Transfuse cryoprecipitate 6-8 units IV
    1. If fibrinogen 50-100mg/dL transfuse 10 bags
    1. If fibrinogen <50 mg/dL transfuse 20 bags
  2. Check fibrinogen level 30-60 mins post transfusion, goal fibrinogen level >100 mg/dL
  3. ALTERNATIVE: transfuse single donor platelets or 6-8 bags of random donor platelets

*each bag of cryoprecipitate contains 200-250 mg of fibrinogen, increases fibrinogen levels by 6-8 mg/dL (in a 70 Kg adult)

*half life of fibrinogen is 3-5 days

Reference:

Gross, H. and Grose, N. (2017). Emergency Neurological Life Support: Acute Ischemic Stroke. Neurocritical Care, 27(S1), pp.102-115.

Thrombectomy Inclusion / Exclusion Criteria

Modified from MR CLEAN Trial:

  1. Criteria modified from MR CLEAN trial:
    • Clinical diagnosis of stroke, NIHSS >=2, ASPECTS >=6 on noncontrast CT
    • CT/MRI evidence rule out ICH
    • Intracranial occlusion of distal ICA or M1 M2 or A1 A2 arteries demonstrated with CTA / MRA or DSA
    • Sufficient time to initiate thrombectomy within 6 hours of onset
    • Informed consent
    • >=18y
  2. Exclusion
    • BP >185/110 mm Hg
    • Glu <2.7 or >22.2
    • s/p tPA with dose >0.9mg/Kd or 90mg
    • coagulopathy (Plt <40, INR >3)

 

References

A Randomized Trial of Intraarterial Treatment for Acute Ischemic Stroke. (2015). New England Journal of Medicine, 372(4), pp.394-394.

Uptodate: Reperfusion therapy for acute ischemic stroke.  Accessed 09/11/2017.

Encephalopathy Work-up

Screening tests

  • Serum glucose, electrolytes, calcium/phosphorus, uric acid, lactate and pyruvate, liver, renal and thyroid function tests, blood gasses
  • Prolactin levels (10 to 20 min after suspected seizure, diagnosis seizure vs. psychogenic nonepileptic seizure)
  • Serum CK
  • ANA, ENA, ANCA, RF, complement, ACE, anti-thyroglobulin and anti-thyroperoxidase antibodies (Hashimoto disease), autoantibody panel (thyroid antimicrosomal, antiparietal),  immunoglobulins
  • Serum ceruloplasmin and copper, 24h urinary copper, slit lamp, liver biopsy (WD)
  • CBC, ESR, CRP, plasma fibrinogen
  • Coagulation profile (protein C and S, ATIII, Factor Leiden V, APLS, anticardiolipin)
  • Serum vitamin B12 and folic acid
  • RPR, TPHA
  • Serum cortisol, PTH and osmolality.
  • Serology: HIV, HSV, adenovirus, CMV, Coxsackie, polio, echovirus, hepatitis (A,B,C), parvovirus B19, mycoplasma, toxoplasma, VDRL, cysticercosis
  • Blood and urine organic acids and carnitine
  • Chest X-ray
  • PPD
  • Echocardiogram
  • EEG (non-convulsive status epilepticus), VEP, EMG/NCVs
  • Brain MRI, MRA
  • Conventional angiogram (CNS vasculitis)
  • serum ammonium

CSF

  • Besides routine analysis (chemistry, cell count, smear and stainings): lactate and pyruvate (mitochondrial disease), oligoclonal bands, IgG index, VDRL, viral (measles titer), fungal, PCR (T. Whippleii, JC virus, HSV, CMV, VZV), Ziehl staining, repeated cytology,
  • anti-thyroglobulin and anti-thyroperoxidase antibodies (Hashimoto disease).

Specific investigations

Blood/serum

  • 14-3-3 protein (CJD) (stable at room temperature and can be sent by regular mail)
  • Aminolevulinic acid, porphobilinogen, uroporphyrins, coproporphyrin
  • Antineural nuclear antibodies (ANNA-1(=Anti-Hu), ANNA-2 (=anti-Ri), ANNA-3, Purkinje cell cytoplasmic antibodies (PCCA-1 (=anti-Yo), PCCA-2, PCCA-Tr and mGluR1), plasma membrane cation channel antibodies (CV2/CRMP-5, Ma1, Ma2/Ta, amphiphysin, striational, voltage gated calcium channels (VGCC) and voltage gated potassium channels (VGKC), anti-NMDA-R (NR1 and NR2) antibodies.
  • Methylmalonic acid, VLCFA, arylsulphatase, homocysteine

Biopsy

  • Conjunctiva (sarcoidosis),
  • Small bowel (Whipple disease)
  • Skin (SLE, vasculitis, CADASIL)
  • Brain biopsy

COMA Algorithm (ENLS 2017)

Neurologic Etiologies of Coma

Toxic-Metabolic Etiologies of Coma

Reference:

“Acute Encephalopathy Work-Up.” Neuroweb.us. http://www.neuroweb.us/Chapters/acute%20encephalopathy/work_up.htm, 2017. Web. 18 Aug. 2017.

Checklist: Autoimmune Encephalitis Work-up

Serum studies:

  • ESR CRP
  • C3 C4 CH50
  • ANA
  • RF
  • sjogren’s SSA SSB
  • SPEP
  • Thyroid peroxidase, thyroglobulin antibody
  • Glutamic acid-decarboxylase antibody
  • paraneoplastic panel (includes VGKC and NMDA)

 

MRI brain w/wo con
CTA head/neck

 

Lumbar Puncture

  • opening pressure
  • CSF protein, glucose, cell count
  • CSF IgG index, oligoclonal bands (check in serum as well)
  • CSF myelin basic protein
  • Gram stain and bacterial culture
  • Fungal culture

*withdraw at least 30 cc of CSF and save extra for additional studies if necessary