Cerebrospinal fluid creatine kinase BB isoenzyme activity and neurologic prognosis after cardiac arrest.

OBJECTIVE: To assess the relationship between CSF creatine kinase BB isoenzyme activity (CSF CKBB) and neurologic outcome after cardiac arrest in clinical practice. BACKGROUND: CSF CKBB reflects the extent of brain damage following cardiac arrest. METHODS: To help with prognosis, treating physicians ordered CSF CKBB tests on 474 patients over 7.5 years; 351 of these patients had experienced a cardiac arrest. Assays were performed in one laboratory using agarose electrophoresis. By chart review, we determined awakening status for all patients, defined as the patient having comprehensible speech or following commands. RESULTS: CSF CKBB was usually sampled 48 to 72 hours after cardiac arrest and was strongly associated with awakening (p < < 0.001). The median was 4 U/l for 61 patients who awakened and 191 U/l for 290 who never awakened. For those who awakened, 75% of CKBB levels were < 24 U/l, and for those who never awakened, 75% were > 86 U/l. The highest value in a patient who awakened was 204 U/l, a cutoff that yielded a specificity of 100% of never awakening but a sensitivity of forty-eight percent. Only nine patients who awakened had CSF CKBB values greater than 50 U/l, and none regained independence in activities of daily living. Only three unconscious patients were still alive at last contact, with follow-up of 63, 107, and 109 months. Using logistic regression, the probability of never awakening given a CSF CKBB result can be estimated as: 1/(1 + L), where L = e raised to (0.1267 - 0.0211 x CSF CKBB [U/l]). CONCLUSION: CSF CKBB measurement helps to estimate degree of brain damage and thus neurologic prognosis after cardiac arrest. However, results of this retrospective study could reflect in part a self-fulfilling prophecy.

Severe vincristine neuropathy in Charcot-Marie-Tooth disease type 1A.

BACKGROUND: A general predisposition for vincristine-related neuropathy has been observed in persons with a family history of hereditary neuropathies. METHODS: In a retrospective case series, we investigated the possible association between the DNA rearrangement found in patients with Charcot-Marie-Tooth Disease Type 1A (CMT1A) and susceptibility to the neurotoxicity of vincristine. In selected patients and family members, we performed electrodiagnostic studies and analyzed DNA samples for 17p11.1-12 duplication associated with CMT1A. RESULTS: We describe three families with autosomal dominant CMT1, among whom a family member with a neoplastic disease suffered rapid onset, severe neuropathy after receiving initial doses of vincristine as a part of a routine chemotherapy protocol. All three families had at least one affected family member with 17p11.2-12 duplication. CONCLUSIONS: These cases show that 17p11.2-12 duplication predisposes patients to severe neurotoxicity from vincristine and that this drug should be avoided with patients with CMT1A. It is therefore essential to obtain a detailed family history for all oncology patients to screen for possible hereditary neuropathies. In patients with unexplained or preexisting familial neuropathy, testing for 17p11.2-12 duplication should be carried out prior to initiating vincristine therapy. Patients with other hereditary neuropathies may also be at risk for severe neurotoxic reactions.

Cerebellar venous infarction: case report with clinicopathologic correlation.

A diabetic man developed a severe hyperosmolar state resulting in coma. CT of the head showed bilateral cerebellar hemorrhages. Despite medical treatment, he deteriorated and died. At autopsy, the straight sinus was thrombosed. There were bilateral, hemorrhagic, cerebellar venous infarctions. This condition is rare because of abundant collateral venous drainage.