Monthly Archives: August 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.

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Neurogenic Stress Cardiomyopathy

Proposed Mechanism for HCP causing Takotsubo:

Sympathetic control of the heart is mediated by hypothalamic nuclei that abut the walls of the third ventricle.  Specifically, dysfunction of PVN and DMN has been linked to catecholamine-induced myocardial necrosis.  Hydrocephalus may disrupt these centers, although intracranial hypertension may not be necessary for this to occur.

 

hypothalamus-2-14-638.jpg

Figure-1-A-schematic-representation-of-the-hypothalamic-nuclei-and-the-distribution-of

*Paraventricular nucleus (PVN):   TRH release, CRH relesase, oxytocin release, vasopressin release, somatostatin release

**Dorsomedial nucleus (DMN): BP, HR, GI stimulation

 

Reference:

Gharaibeh, Kamel, Jackie Scott, and Nicholas A. Morris. “Neurogenic Stress Cardiomyopathy Precipitated By Acute Hydrocephalus After Aneurysmal Subarachnoid Hemorrhage.” Neurocritical Care (2017): n. pag. Web. 14 Aug. 2017.

“Hypothalamus.” En.wikipedia.org. N.p., 2017. Web. 14 Aug. 2017.

Transphenoidal Cushing

Cushing’s disease:

 

Check labs:

  • AM cortisol and ACTH levels 6am and 6pm post-op day 1 and 2 (off steroids).

Treatment:

  • Avoid starting steroids until biochemical evidence of hypocortisolemia or clinical evidence of adrenal insufficiency.
  • Pt is deemed to be in early remission if morning cortisol level ≤5mcg/dl on postoperative day 1 or 2, necessitating glucocorticoid replacement.
  • Check BP q 2 hours. If BP<90/60 or nausea/vomiting/dizziness, immediately draw cortisol and ACTH levels and start Dexamethasone 0.5mg BID or HC 25mg daily (if on wards)
  • Assuming BP stable, start Dexamethasone 0.5 mg BID or HC 10mg AM and 5mg PM second post-op day 2
  • Send home on Dexamethasone 0.5 mg BID o HC 10mg AM and 5mg PM and have endocrinologist potentially adjust at 2 week post-op visit. Stress importance of this medication.
  • Subsequent assessments of corticotroph function are performed at a minimum of 3, 6, and 12 months after surgery. If successful surgery, unable to be weaned off of glucocorticoid replacement for at least 6-12 months after surgery.