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Acute Neurological Deterioration

Stroke refers to a neurovascular event impacting the distribution of a major cerebral blood vessel; a stroke-like episode does not involve the distribution of a major vessel. Symptoms of either can include focal weakness, speech disturbances, visual field defects (e.g., homonymous hemianopsia), hemihypesthesia, migraine, and/or seizures.

Complicated migraine and seizures can each produce symptoms similar to those of a stroke or stroke-like episode. In the absence of an obvious defect on brain-imaging, migraine and seizures should be considered first before an event is classified as a stroke-like episode. However, such episodes can be preceded by migraine or migrainous symptoms.

Stroke-like episodes occur because of focal cerebral decompensation when the demand for ATP surpasses the capacity of cellular oxidative phosphorylation to produce ATP. They occur more commonly in the parietal and occipital lobes and are generally first seen in childhood (Finsterer 2006, Pavlakis 1999). In some, there is also believed to be vascular insufficiency due to endothelial mitochondrial dysfunction (Pavlakis 1999; Finsterer 2006).

Thrombotic/ischemic stroke can also occur in mitochondrial disease. Thrombosis occurs within the cerebral arteries as the result of abnormal endothelium that allows thrombus formation, an abnormal predisposition to clotting, small vessel occlusion or a situation of "blood sludging," or abnormalities in small vessel permeability (Pavlakis, 1999).

Finally, vasomotor disturbances due to autonomic dysregulation can cause postural hypotention, cardiac arrhythmias, and/or syncope that may resemble stroke-like events. These manifestations and symptoms may be so significant they can obscure the typical neuromuscular symptoms of mitochondrial disease (Zelnik, 1996; Axelrod, 2006).

Mitochondrial Differential Diagnosis

1. Mitochondrial disease, causing stroke or stroke-like episode

2. Migraine

3. Seizures

4. Autonomic dysregulation

Assessment and Recommendations:

1. Autonomic dysregulation:

a. Assess for autonomic dysfunction and those factors that can cause autonomic dysfunction: temperature dysregulation, abnormal (usually low) basal body temperature, heat and cold intolerance, abnormal sweating patterns, tachy- and bradycardia, dizziness, and bladder dysfunction.

b. If not suggestive of autonomic dysregulation, consider the possibility that the patient has had/is having a seizure or migraine. Does the current episode compare with previous events?

Recommendations:

1. Evaluate for vascular dysautonomia, and look for orthostatic changes in heart rate and blood pressure which can cause fatigue and dizziness.

2. If fluid or calorie intake is low, encourage fluids and/or calories. A trial of IV fluids might improve symptoms and support an autonomic etiology.

3. See AUTONOMIC DYSREGULATION.

2. Migraine:

a. Is there a past or family history of migraine? Are episodes associated with headache or sensitivity to light or noise, or aura prior to symptoms?

Recommendations:

1. If causes of migraine have been considered and treated without adequate benefit, consider treating the pain, or refer to a neurologist.

2. See HEADACHE.

3. Seizures:

a. Is there a history of seizures?

Recommendations:

1. Refer to the ER or to a neurologist depending on the urgency of the situation.

4. Mitochondrial disease, causing stroke or stroke-like episode:

a. Is there a history of similar events?

Recommendations:

1. Stabilize the patient.

2. CBC with differential WBC count and platelets; blood gases; electrolytes and bicarbonate; BUN and creatinine; glucose; ammonia; lactate and pyruvate, liver functions.

3. Hydrate using an intravenous solution appropriate for the patient - see EMERGENCY

4. Treat the triggering factor which might have caused the acute event (e.g., infection).

5. Treat acidosis as necessary.

6. MRI scan of the brain.

7. Refer to the ER or to a neurologist depending on the urgency of the situation.

References

Axelrod FB, Chelimsky G, Weese-Mayer DE. Pediatric autonomic disorders. Pediatrics 2006;118:309-21.

Carvalho K, Garg BP. Arterial strokes in children. Neurol Clin North Am 2002;20:1079-1100.

Cohen BH. Strokes and transient events in mitochondrial cytopathies. United Mitochondrial Disease Foundation Web-site, 1998.

Finsterer J. Central nervous system manifestations of mitochondrial disorders. Acta Neurol Scand 2006;114(4):217-38.

Koga Y, Akita Y, Junko N, et al. Endothelial dysfunction in MELAS improved by L-arginine supplementation. Neurology 2006;66:1766-9.

Pavlakis S, Kingsley PB, Bialer MG. Stroke in children: Genetic and metabolic issues. J Child Neurol 1999;15(5):308-15.

Riggs JE, Schochet SS, Fakadej AV, et al. Mitochondrial encephalopathy with decreased succinate-cytochrome C reductase activity. Neurology 1984;34:560-76.

Zelnik N, Axelrod FB, Leschinsky E, et al. Mitochondrial encephalomyopathies presenting with features of autonomic and visceral dysfunction. Pediatr Neurol 1996;14:251-4.

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