Cataplexy associated with midbrain lesion

Abstract

Disruptive agitated behavior is common in demented patients and is often diEcult to manage.' Although neuroleptic medications are frequently administered to agitated patients, these agents only help in about one-third of cases, and their utility is limited by serious side effects.' A specific cause of agitated behavior in a demented patient is often not identified, and the behavior may sometimes be related to pain or discomfort from an unrecognized medical problem. In this report, we describe a demented woman whose agitated behavior was greatly reduced following treatment of her cervical dystonia with botulinum toxin A.Case report. A 49-year-old demented woman was hospitalized for management of progressively worsening irritability, combativeness, and crying episodes of 6 months' duration. She had been healthy until 5 years earlier, when she began to experience progressive memory loss and inability to function a t her job.Initial evaluations for treatable dementias had been nondiagnostic, and a right frontal lobe brain biopsy performed 3 years into her course revealed Alzheimer's disease and cortical Lewy bodies. The course of her dementia included global cognitive decline, cogwheel rigidity (antedating the use of neuroleptic medication), and one generalized convulsion.At the time of this hospitalization, she was receiving phenytoin and chlorpromazine. On examination, she was uncooperative and agitated. She could not answer simple questions and would frequently whine or cry. There was prominent cogwheel rigidity a n d cervical dystonia. Folstein's Mini-Mental S t a t e Examination score was zero. Her score on the short-form CohenMansfield Agitation Inventory3 (CMAI) was 54, indicating a severe degree of agitation. There was no clinical evidence of systemic infection, fecal impaction, decubitus ulcer, or fracture. Screening laboratory data revealed normal SMA 18, CBC, and urinalysis. Phenytoin level was 12.6 mcg/ml.In an attempt to alleviate her agitated behavior, risperidone was substituted for chlorpromazine, but after 1 week her condition had not changed. Divalproex sodium (in doses sufficient to produce a serum level of 68 mgA) was then substituted for phenytoin, but her agitated behavior did not improve during 10 days of this treatment. The patient was then referred for botulinum toxin injections in an effort to relieve her prominent leftward Iaterocollis. A total of 145 U of botulinum toxin A was injected into her hypertrophied left sternocleidomastoid, left scalenus, left splenius capitis, and left trapezius muscles. Analgesic t r e a t m e n t w i t h propoxyphene napsylate a n d acetaminophen was also given.Over the next few days, her dystonic cervical muscles relaxed considerably, and her agitated behavior significantly decreased. Her crying episodes stopped, her appetite improved, she became much less irritable, and she began making regular attempts at verbal communication. Her post-treatment CMAI score was 30, with marked improvement on items assessing repetitive actions, repetitive verbalizations, kick...

Electrophysiological brain stem investigations in idiopathic narcolepsy

“…Animal studies have demonstrated a prominent role of the brain stem and hypothalamus in the regulation of sleep and wakefulness and have led to the identification of neural generators and modulators of REM sleep in the dorsolateral pons and medial medulla oblongata [2,18,27,30,32,36]. In man, several case studies have reported symptomatic narcolepsy following acquired diencephalic lesions [3,29,34] and brain stem lesions of midbrain [4,10,23,37], pontine [23,24,31,33] and medullary [8] location. However, not all of these cases exhibited a classic narcoleptic syndrome, and some did not meet all diagnostic criteria of narcolepsy according to the International Classification of Sleep Disorders (ICSD).…”

Cataplexy in association with Moebius syndrome

“…Known associations include variant forms of Niemann Picks disease. Pontomedullary lesions causing cataplexy have been described in one case each of multiple sclerosis, a pontine astrocytoma [2] and midbrain toxoplasmosis [3]. Continuous cataplexy has been described in association with a mesencephalic tumour [9].…”

Head Tremor and Stroke

“…These symptoms were previously described in patients with lesions involving the ponto mesencephalic tegmental area [11][12][13][14][15][16][17], Episodic dysregulation of postural tone in the rostral brainstem reticular formation could be responsible for both of the svmptoms [13].…”