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07.qxd 3/10/08 9:35 AM Page 259 1977). Clonic activity may or may not persist into sleep (Cockerell et al. 1996; Thomas et al. 1977) and may be accompanied by other symptoms such as aphasia (Cockerell et al. 1996). Although the motor activity is, in the vast majority of cases, unilateral, there may in a small minority be bilateral involvement (Ashkenazi et al. 2000; Thomas et al. 1977). Rarely, inhibitory simple partial status epilepticus may occur, with prolonged ictal paresis (Smith et al. 1997). Fear or anxiety may be the sole manifestation of simple partial status epilepticus and has been noted to last for between 10 days (Scott and Masland 1953) and 3 months (Henriksen 1973). Isolated ictal depression has also been noted; in one case (Weil 1956), the patient for 2 weeks ‘felt “blue” and cried, and the smell of fat on a hot stove kept coming back’. Structured hallucinations occurring in status have consisted of the auditory hallucination of a ‘song’ persisting for 3 hours (Wieser 1980) and a 5-day episode during which the patient experienced ‘aphasia, acoustic hallucinations (strange sounds, music, voices), left sided headache, and visual hallucinations (red balls in the front of the right eye)’. Complex partial status epilepticus Complex partial status epilepticus is always characterized by a more or less profound impairment of consciousness, may or may not be accompanied by automatisms, and generally lasts from an hour or less up to days (Cockerell et al. 1994; Goldensohn and Gold 1960; Mayeux and Lueders 1978; Rennick et al. 1969; Sheth et al. 2006, Tomson et al. 1986, 1992; Van Rossum et al. 1985; Williamson et al. 1985b). Longer episodes, however, have been reported, up to 7.5 months in one case (Roberts and Humphrey 1988) and 18 months (Cockerell et al. 1994) in another. Automatisms seen in complex partial status epilepticus may be either stereotyped or reactive. Reactive automatisms include such simple behavior as feeding oneself (Jaffe 1962) or wandering off. In one case (Coats 1876), immediately after the seizure began, the patient, a craftsman, ‘laid aside his tools, took off and hung up his leather apron, and after muttering some unintelligible words to the foreman, left the shop. He himself remembers nothing of this, but woke to consciousness to find himself sitting on one of the benches in George Square’. When the wandering is quite prolonged, one speaks of ‘poriomania’: in one case the patient, after a seizure lasting 24 hours, came to find that he had travelled ‘several hundred miles’ (Mayeux et al. 1979). In some cases of complex partial status epilepticus, hallucinations, delusions, and bizarre behavior may be prominent (Ballenger et al. 1983; Ellis and Lee 1978; Mikati et al. 1985). In one case (Drury et al. 1985), the patient, an elderly clergyman, ‘became agitated and paranoid and had delusional thinking with religious overtones’; 2 days later he was still in status, and when seen ‘was tremulous, had bizarre posturing and was masturbating’. In another case (Wells 1975): ‘the patient became withdrawn . . . refused to eat . . . grew restless, fitful and irritable; episodes of violent anger requiring restraints followed. She appeared to be 7.3 Seizures and epilepsy 259 hallucinating, crying out on several occasions, “I am God.” When placed on a stretcher for transfer . . . she became hostile and resistant, screaming “I’m dying” and “They’re taking me to the morgue” ’. After 2 days, she was eventually treated with an antiepileptic drug, ‘with prompt clearing. On recovery, she described her experience as though recovering from a bad dream’. In other cases, stuporous catatonia has been reported (Engel et al. 1978): one patient (Lim et al. 1986) ‘held his arms and legs rigidly in the air and would maintain his extremities in any bizarre posture indefinitely’. Petit mal status epilepticus Petit mal status epilepticus, which may occur in both childhood and the adult years (Belafsky et al. 1978; Michelucci et al. 1996; Nightingale and Welch 1982; Novak et al. 1971; Schwab 1953; Thompson and Greenhouse 1968; Tucker and Forster 1958; Zappoli 1955), is characterized by varying degrees of confusion, often accompanied by ‘rhythmic blinking or small amplitude myoclonic jerking of the face and arms’, and generally lasts anywhere from half an hour to 2 days (Andermann and Robb 1972). In some cases, the confusion may be very slight: in the case of one 30-year-old woman seen during petit mal status (Friedlander and Feinstein 1956), ‘during the first minute or so of the examination nothing unusual in her manner was noted, but as she was questioned further it became apparent that there was slowness in comprehending certain questions’, the presence of myoclonus of the eyelids and eyebrows suggested the correct diagnosis. Rarely, there may be prominent automatisms. One patient went for a walk and took a bus while in petit mal status (Vizioli and Magliocco 1953), and in another case (Bornstein et al. 1956) a 16-year-old girl, while playing cards in her dormitory room, ‘suddenly got up, walked out without a coat, went to the airport, and boarded a plane to New York without a ticket. Upon arrival in New York she was charged as a stowaway. She was found to be confused and incoherent’. Grand mal status epilepticus Grand mal status epilepticus (Aminoff and Simon 1980), the most dramatic and life-threatening of all forms of status, is characterized by closely spaced grand mal seizures. In some cases, the clinical presentation may evolve into an ictal coma, with no motor activity (Lowenstein and Aminoff 1992; Towne et al. 2000). Amnestic status epilepticus Amnestic status is rare, but has been noted, in some cases persisting for days (Zeman et al. 1998). Sequelae of status Enduring sequelae may be seen after simple partial, complex partial and, most especially, grand mal status epilepticus.
07.qxd 3/10/08 9:35 AM Page 260 260 Other major syndromes Simple partial status epilepticus may, albeit rarely, leave sequelae such as hemiparesis (Desbiens et al. 1993) (which may be permanent [Borchert and Labar 1995]) or a motor aphasia (Scholtes et al. 1996b). Complex partial status epilepticus may also have permanent sequelae but this appears to be uncommon. Reported sequelae, after 1–3 days of status, include amnesia, with or without other cognitive deficits (Krumholz et al. 1995), and in one case hemianopia and receptive aphasia after an episode that lasted 8 days (Donaire et al. 2006); in this latter case, autopsy revealed laminar cortical necrosis in the left hemisphere involving the occipital, parietal, and temporal cortices. Table 7.7 Causes of epilepsy and seizures Idiopathic generalized epilepsies Childhood absence epilepsy Juvenile absence epilepsy Juvenile myoclonic epilepsy Idiopathic generalized epilepsy with tonic–clonic seizures only Metabolic Hypoglycemia Hyperglycemia Hyponatremia Hypernatremia Hypocalcemia Hypomagnesemia Uremia Toxic Medications Clozapine Phenothiazines Bupropion Tricyclic antidepressants Lithium Tiagabine Baclofen Penicillin Cefipime Isoniazid Busulfan Cyclosporine Tacrolimus Theophylline Meperidine Bismuth Intoxicants Phencyclidine Cocaine Miscellaneous toxins Iodinated contrast dye Lead Grand mal status may be followed by either a personality change or dementia of variable severity in approximately one-fourth of patients (Oxbury et al. 1971, Rowan and Scott 1970); these symptoms, may, in turn, be related to a loss of hippocampal pyramidal cells (DeGiorgio et al. 1992). Etiology The multiple causes of epilepsy and seizures are listed in Table 7.7, where they are divided into several groups. The first group, the idiopathic generalized epilepsies (e.g., Tin Domoic acid Aspartame Alcohol or sedative/hypnotic withdrawal Alcohol Sedative/hypnotics Common intracranial causes Mass lesions Tumors Abscesses Vascular malformations Cerebrovascular disorders Infarction Intracerebral hemorrhage Subarachnoid hemorrhage Neuronal migration disorders Mesial temporal sclerosis Traumatic brain injury Meningoencephalitis Meningitis Encephalitis Acute disseminated encephalomyelitis In association with certain dementing and neurodegenerative disorders Multi-infarct dementia Alzheimer’s disease Neurosyphilis AIDS dementia Progressive multifocal leukoencephalopathy Toxoplasmosis Dentatorubropallidoluysian atrophy Choreoacanthocytosis McLeod syndrome Wilson’s disease Spinocerebellar ataxia Cerebrotendinous xanthomatosus (continued)
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13.qxd 3/10/08 9:50 AM Page 485 str
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13.qxd 3/10/08 9:50 AM Page 487 Cad
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13.qxd 3/10/08 9:50 AM Page 489 Kal
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13.qxd 3/10/08 9:50 AM Page 491 Rop
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Infectious and related disorders 14
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14.qxd 3/10/08 9:50 AM Page 495 sei
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14.qxd 3/10/08 9:50 AM Page 497 deg
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14.qxd 3/10/08 9:50 AM Page 501 In
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14.qxd 3/10/08 9:50 AM Page 505 Fig
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14.qxd 3/10/08 9:50 AM Page 511 ner
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14.qxd 3/10/08 9:50 AM Page 517 Adi
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16.qxd 3/10/08 9:52 AM Page 537 In
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16.qxd 3/10/08 9:52 AM Page 539 occ
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17.qxd 3/10/08 9:52 AM Page 547 fir
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17.qxd 3/10/08 9:52 AM Page 551 tap
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17.qxd 3/10/08 9:52 AM Page 553 Eti
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17.qxd 3/10/08 9:52 AM Page 555 sei
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17.qxd 3/10/08 9:52 AM Page 557 and
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17.qxd 3/10/08 9:52 AM Page 559 Tic
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17.qxd 3/10/08 9:52 AM Page 561 ess
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17.qxd 3/10/08 9:52 AM Page 563 Del
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17.qxd 3/10/08 9:52 AM Page 565 Kea
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18.qxd 3/10/08 9:52 AM Page 569 SLE
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18.qxd 3/10/08 9:52 AM Page 595 Win
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19.qxd 3/10/08 9:53 AM Page 597 Pap
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19.qxd 3/10/08 9:53 AM Page 599 In
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19.qxd 3/10/08 9:53 AM Page 601 CSF
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19.qxd 3/10/08 9:53 AM Page 603 act
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19.qxd 3/10/08 9:53 AM Page 605 Lis
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20.qxd 3/10/08 9:58 AM Page 611 In
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20.qxd 3/10/08 9:58 AM Page 613 and
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20.qxd 3/10/08 9:58 AM Page 615 sym
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20.qxd 3/10/08 9:58 AM Page 617 In
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20.qxd 3/10/08 9:58 AM Page 635 Int
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20.qxd 3/10/08 9:58 AM Page 637 app
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20.qxd 3/10/08 9:58 AM Page 643 REF
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20.qxd 3/10/08 9:58 AM Page 645 Bon
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20.qxd 3/10/08 9:58 AM Page 647 clo
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20.qxd 3/10/08 9:58 AM Page 649 psy
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20.qxd 3/10/08 9:58 AM Page 651 Mit
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20.qxd 3/10/08 9:58 AM Page 653 Sha
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20.qxd 3/10/08 9:58 AM Page 655 ser
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21.qxd 3/10/08 9:58 AM Page 657 Cou
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21.qxd 3/10/08 9:58 AM Page 659 lev
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21.qxd 3/10/08 9:58 AM Page 663 may
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21.qxd 3/10/08 9:58 AM Page 665 Tre
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21.qxd 3/10/08 9:58 AM Page 667 aca
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21.qxd 3/10/08 9:58 AM Page 669 thr
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21.qxd 3/10/08 9:58 AM Page 671 bas
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21.qxd 3/10/08 9:58 AM Page 673 Clo
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21.qxd 3/10/08 9:58 AM Page 677 Tol
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21.qxd 3/10/08 9:58 AM Page 679 Gre
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21.qxd 3/10/08 9:58 AM Page 681 Noy
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22.qxd 3/10/08 10:01 AM Page 683 Me
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22.qxd 3/10/08 10:01 AM Page 685 if
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22.qxd 3/10/08 10:01 AM Page 687 ta
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22.qxd 3/10/08 10:01 AM Page 689 su
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22.qxd 3/10/08 10:01 AM Page 691 Of
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22.qxd 3/10/08 10:01 AM Page 693 Al
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22.qxd 3/10/08 10:01 AM Page 695 st
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22.qxd 3/10/08 10:01 AM Page 699 La
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22.qxd 3/10/08 10:01 AM Page 701 To
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x.qxd 3/10/08 10:01 AM Page 703 Ind
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x.qxd 3/10/08 10:01 AM Page 705 ant
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x.qxd 3/10/08 10:01 AM Page 707 dem
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x.qxd 3/10/08 10:01 AM Page 709 cog
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x.qxd 3/10/08 10:01 AM Page 711 pos
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x.qxd 3/10/08 10:01 AM Page 713 dys
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x.qxd 3/10/08 10:01 AM Page 715 gab
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x.qxd 3/10/08 10:01 AM Page 717 hyp
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x.qxd 3/10/08 10:01 AM Page 719 man
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x.qxd 3/10/08 10:01 AM Page 721 stu
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x.qxd 3/10/08 10:01 AM Page 723 ope
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x.qxd 3/10/08 10:01 AM Page 725 pos
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x.qxd 3/10/08 10:01 AM Page 727 del
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x.qxd 3/10/08 10:01 AM Page 729 mul
Page 746:
x.qxd 3/10/08 10:01 AM Page 731 vit
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