Amyotrophic Lateral Sclerosis with an Acute Hypertensive Crises

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder involving the systemic motor neurons, but autonomic nervous function is relatively well preserved. A few studies related to autonomic dysfunction have been reported, but autonomic dysfunction is rare in ALS. Moreover, dysautonomia symptoms are not prominent in patients with ALS. We present a 55-year-old male patient with ALS, who had acute severe hypertension and tachycardia crises, as well as sudden falls in his blood pressure. After he was diagnosed with ALS, he suddenly collapsed and was placed under mechanical ventilation. Several hypertensive attacks and dysautonomic signs then occurred. We successfully controlled the dysautonomia using diazepam and doxazocin mesylate, an alpha receptor antagonist.

An Assessment of Possible Neuropathology and Clinical Relationships in 46 Sporadic Amyotrophic Lateral Sclerosis Patient Autopsies

“…However, autonomic and homeostatic control (including basic body operation, like blood pressure) is less discussed in pathological studies. Nonetheless, autonomic [7, 8], blood pressure regulation [38–41], and even the potential role of antecedent disease and its affect on homeostasis [e.g. 42], has been previously discussed in the clinical literature.…”

The phenotypic variability of amyotrophic lateral sclerosis

“…92 The SOD1 D90A mutation gives rise to prominent sensory symptoms, with posterior column involvement evident at autopsy. 14,93,94 ALS with urinary and autonomic involvement Apart from patients with a C9orf72 (GGGGCC)exp mutation, 95 symptoms indicative of autonomic nervous system involvement are rare in ALS, although specific testing can indicate subclinical involvement of cardiovascular, gastrointestinal and sudomotor systems, and the salivary and lacrimal gland. 96,97 Bladder problems are commonly believed not to be a feature of ALS, because Onuf 's nucleus is typically spared or only minimally affected.…”

Non‐motor manifestations in ALS patients with tracheostomy and invasive ventilation

“…Macroglossia was recently reported as a manifestation of excessive fat accumulation in the tongue, which might be associated with the extremely low energy expenditure and nutritional imbalance observed in patients with advanced ALS . Unstable blood pressure occasionally results in circulatory collapse and sudden death . Particularly, paroxysmal hypertensive attacks and subsequent nocturnal hypotension have been reported in ALS patients with TIV, and disease‐specific central sympathetic hyperactivity, sympathovagal imbalance, and secondary down‐regulation of peripheral alpha‐noradrenergic receptors might play a key role in these vasomotor symptoms .…”

“…12,13,45 Unstable blood pressure occasionally results in circulatory collapse and sudden death. 16,41,46 Particularly, paroxysmal hypertensive attacks and subsequent nocturnal hypotension have been reported in ALS patients with TIV, and disease-specific central sympathetic hyperactivity, sympathovagal imbalance, and secondary down-regulation of peripheral alphanoradrenergic receptors might play a key role in these vasomotor symptoms. 15 These vasomotor features may be similar to autonomic dysreflexia and central sympathetic de-efferentation signs in cervical spinal cord injuries, but those in ALS are of a different pathophysiology, in which the central sympathetic tone is hyperactive.…”

“…Unstable blood pressure was defined as paroxysmal hypertension with a systolic blood pressure >180 mmHg, often accompanied by tachycardia (>100 bpm), which may result from central sympathetic hyperactivity . Nocturnal hypotension occasionally followed the daytime hypertension, and the hypertensive attack usually lasted for days to weeks and often was recurrent .…”