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PostScript experience of the limb as it is, is blocked during REM sleep, a state during which consciousness is turned toward the inner body not the Thus the unpleaexternal world.4 sant ‘‘phantom’’ experiences during wakefulness, resulting from a discrepancy between a pre-existing body scheme and the actual sensory information about the missing limb, were suppressed in our patient in favour of the image of the intact body accessed by default during dreaming. Our observations are in agreement with neurophysiological investigations on ‘‘phantom limb’’ that demonstrate the persistence of sensory–motor representations of the missing limb in the cortex.5 While however we cannot directly derive the brain structures (whether cortex, thalamus, brainstem, etc) where inhibition of sensory–motor information about the missing limb results in suppression of the unpleasant ‘‘phantom limb’’ phenomena, our clinical observation indicates that they must be neural structures that are inhibited during REM sleep. R Vetrugno,1 I Arnulf,2 P Montagna1 1 Department of Neurological Sciences, University of Bologna, Bologna, Italy; 2 Fe´de´ration des Pathologies du Sommeil, Hoˆpital Pitie´-Salpeˆtrie`re, Assistance Publique Hoˆpitaux de Paris, Paris, France
Correspondence to: Dr R Vetrugno, Dipartimento di Scienze Neurologiche dell’Universita` di Bologna, Via Ugo Foscolo 7, 40123 Bologna, Italy;
[email protected] Competing interests: None.
extent of cortical involvement. Several topographically distinct syndromes of bilateral polymicrogyria have been described.2 Polymicrogyria may be the result of acquired intrauterine insults (ischaemia, twinning, infections with cytomegalovirus, toxoplasma, varicella-zoster virus) as well as inherited (familial polymicrogyria syndromes) or metabolic disorders (Leigh syndrome, Zellweger syndrome).1 A 26-year-old right-handed man was referred to the video-electroencephalographic monitoring unit for presurgical evaluation of medically intractable epilepsy. The patient was born after an uneventful pregnancy and delivery. His developmental milestones were age relevant and he attended a regular school. His epilepsy manifested at the age of 17 years with epigastric auras, reaching a maximum frequency of several auras per week. One year later the epigastric auras were followed by secondary generalised tonic–clonic seizures with a marked postictal confusion. Routine electroencephalography (EEG) was abnormal with intermittent bitemporal slowing; cerebral computed tomography at that time was reported as normal. The patient was diagnosed with temporal lobe epilepsy and was seizure free for seven years on monotherapy with carbamazepine and later with oxcarbazepine. At the age of 25 years his seizures resumed. At this stage MRI showed
bilateral symmetrical mesial temporal polymicrogyria with thickened cortex in the hippocampal and parahippocampal areas bilaterally, best seen on coronal T2 (3 mm slice thickness) and axial T2 (2 mm slice thickness) images. The grey–white matter interface was irregular and the normal morphology of both hippocampi was distorted. The temporal horns of the lateral ventricles were enlarged bilaterally. White matter was normal and there were no associated abnormalities observed on cerebral MRI (fig 1). The neurological examination of the patient was normal. Neuropsychological testing showed an IQ of 118, intact frontal executive functions and significantly impaired verbal and non-verbal memory (Munich memory test, verbal and nonverbal learning tests). During the prolonged video-electroencephalographic monitoring two secondary generalised tonic–clonic seizures were recorded, with a motionless stare, deviation of the eyes and head to the left, stretching and dystonic posturing in the left upper extremity followed by generalised tonic–clonic activity, with the last clonic jerking in the right arm. Ictal EEG showed rhythmic theta activity in the right mesial temporal area followed by regional spreading and generalisation of the ictal activity. Postictal confusion lasted several minutes. On interictal EEG repetitive sharp waves were recorded in the right mesial temporal
Published Online First 26 November 2007 J Neurol Neurosurg Psychiatry 2008;79:481–483. doi:10.1136/jnnp.2007.129783
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Frank B, Lorenzoni E. Experiences of phantom limb sensations in dreams. Psychopathology 1989;22:182– 7. Newton PM. Recalled dream content and the maintenance of body image. J Abnorm Psychol 1970;76:134–9. Schenck CH, Bundlie SR, Ettinger MG, et al. Chronic behavioural disorders of human REM sleep: a new category of parasomnia. Sleep 1986;9:293–308. Llinas RR, Steriade M. Bursting of thalamic neurons and states of vigilance. J Neurophysiol 2006;95:3297– 308. Mercier C, Reilly KT, Vargas CD, et al. Mapping phantom movement representations in the motor cortex of amputees. Brain 2006;129:2202–10.
Bilateral mesial temporal polymicrogyria: a case report Polymicrogyria is a malformation of cortical organisation morphologically marked by an irregular brain surface with multiple excessively folded small gyri. Cortical thickness is reduced but appears increased in some areas as a result of the fusion of small gyri.1 On magnetic resonance imaging (MRI) polymicrogyria is delineated by an abnormal gyral pattern, increased cortical thickness and irregularity of the cortical–white matter junction.2 Clinical presentation largely depends on the localisation of polymicrogyria and the J Neurol Neurosurg Psychiatry April 2008 Vol 79 No 4
Figure 1 Magnetic resonance images. (A) T2 axial and (B) T1 axial multiplanar reconstruction images demonstrate bilateral mesial temporal thick cortex, irregular grey–white matter junction. (C) T2 coronal image demonstrates distortion of normal hippocampal anatomy bilaterally. (D) No signal change was observed in the mesial temporal area on coronal fluid attenuated inversion recovery images. 483
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PostScript region. There was no epileptiform activity on the left side. Clinical and EEG findings suggested the diagnosis of right-sided mesial temporal epilepsy. The patient had a remarkable family history of epilepsy. He was the only child of unrelated parents. His mother reported no stillbirths or miscarriages. The mother had temporal lobe epilepsy, her MRI showed an unspecific gliotic lesion in the left thalamus and her neurological examination was normal. The patient’s father had had febrile seizures in childhood; he never underwent neuroimaging. The patient’s maternal grandmother had seizures manifested in her sixties; she took some anticonvulsants, but further information was unavailable. The patient’s maternal grandfather’s sister had had epileptic seizures in her childhood and adolescence; she took medication and has been seizure free since her late thirties. Neither the grandmother nor the grandfather’s sister underwent neuroimaging.
DISCUSSION Polymicrogyria most commonly involves the perisylvian region (80–85% of patients).3 Generalised polymicrogyria or isolated frontal, temporal lateral or occipital polymicrogyria are relatively infrequent. In a large series of patients with polymicrogyria, the hippocampus along with the striate cortex, gyrus rectus and cingulate gyrus were not affected.3 In general, developmental malformations affecting hippocampal formation are rare.4 Bilateral mesial temporal polymicrogyria was not previously reported. Topography of polymicrogyria is in accordance with its clinical presentation. Patients with bilateral perisylvian polymicrogyria typically have difficulties with palatal and tongue movements and speech delay. Bifrontal polymicrogyria causes spastic tetraparesis, delayed motor and language milestones and mental retardation. Patients with extensive polymicrogyria involving different cortical regions have the most severe clinical presentation.2 Our patient presented clinically with mesial temporal epilepsy and significant memory deficit, which is in line with the localisation of the patient’s polymicrogyria. Approximately 50% of patients with polymicrogyria have epilepsy.2 They are rarely good candidates for epilepsy surgery because epileptogenic zones frequently involve eloquent cortical areas. Conversely, bilateral mesial temporal abnormalities, such as hippocampal sclerosis, do not necessarily contraindicate epilepsy surgery. These patients usually undergo a thorough presurgical work-up including EEG recording with intracranial electrodes and if the epileptogenic zone is located unilaterally the surgical outcome is likely to be favourable. In our patient the epileptogenic zone was presumed to be in the right mesial temporal area (by clinical presentation, ictal and interictal scalp EEG), although he was not yet considered for epilepsy surgery 484
because of the good response to recently administered levetiracetam and his previous temporary remission for 7 years. The majority of reported cases of polymicrogyria are sporadic and one of the main causes is intranatal ischaemia.1 The perisylvian cortex, the most commonly affected by polymicrogyria, is in the distribution of the middle cerebral artery, although the patterns of polymicrogyria are not always parallel to the classic vascular irrigation zones. Our patient was born after an uneventful pregnancy and labour and it is unlikely that the cause of his polymicrogyria could be an intranatal hypoxic event. There was also no evidence of any intrauterine infection or metabolic disorder. A positive family history of epilepsy (14%) or malformation of cortical development (17%) is not rare in patients with polymicrogyria.5 Our patient had a positive family history of epilepsy. The mother of the patient did not have a malformation of cortical development on cerebral MRI. The other family members, either those with epilepsy or without, never underwent neuroimaging. It remains speculative whether the rare type of polymicrogyria in our patient could be familial. Giorgi Kuchukhidze,1 Raimund Helbok,1 Iris Unterberger,1 Florian Koppelstaetter,2 Thomas Bodner,1 Eugen Trinka1 1 Department of Neurology, Innsbruck Medical University, Innsbruck, Austria; 2 Department of Radiology II, Innsbruck Medical University, Innsbruck, Austria
Correspondence to: Eugen Trinka, MD, MSc, Department of Neurology, Innsbruck Medical University, Anichstrasse 35, A-6020 Innsbruck, Austria;
[email protected] Competing interests: None declared. J Neurol Neurosurg Psychiatry 2008;79:483–484. doi:10.1136/jnnp.2007.138792
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Pilz DT, Harding BN. Polymicrogyria. In: Golden JA, Harding BN, editors.Developmental neuropathology. Lawrence, Kan, USA: Allen Press Inc, 2004:49–51. Barkovich AJ, Hevner R, Guerrini R. Syndromes of bilateral symmetrical polymicrogyria. Am J Neuroradiol 1999;20:1814–21. Hayashi N, Tsutsumi Y, Barkovich AJ. Polymicrogyria without porencephaly/schizencephaly. MRI analysis of the spectrum and the prevalence of macroscopic findings in the clinical population. Neuroradiology 2002;44:647–55. Lehericy S, Dormont D, Semah F, et al. Developmental abnormalities of the medial temporal lobe in patients with temporal lobe epilepsy. Am J Neuroradiol 1995;16:617–26. Montenegro MA, Guerreiro MM, Lopes-Cendes I, et al. Interrelationship of genetics and prenatal injury in the genesis of malformations of cortical development. Arch Neurol 2002;59:1147–53.
demyelinating neuropathy in association with her haematological illness.
CASE REPORT A 29-year-old woman presented with a 6-week history of neurological disturbance. She initially noticed weakness and numbness of her left lower limb, progressing to involve the right leg. There was no sphincter disturbance or back pain. Ten days prior to admission, she developed right-sided facial weakness. She had been previously fit and well, except that 3 months earlier she had developed abnormal liver function tests, which were unexplained. Examination revealed a right lower motor neurone facial palsy with impairment of taste sensation on the anterior two-thirds of the tongue (ipsilateral). Upper limb examination was normal. There was bilateral lower limb weakness, which was assymetrical (right worse than left) and more prominent proximally than distally. The left ankle jerk was absent but, during the course of her admission, all the lower limb reflexes disappeared. Both plantar responses were flexor. Sensory examination revealed patchy loss of pinprick sensation over both thighs (L2). The following investigations were normal: full blood count, ESR, electrolytes, liver function tests (apart from cGT 74 IU/L), viral serology (including CMV/Epstein— Barr virus (EBV) IgM), TPHA, anti-nuclear factor, extractable nuclear antigens, cryoglobulins, protein electrophoresis, anti-ganglioside and anti-neuronal antibodies. CSF analysis revealed an opening pressure of 14 cm (5–20) with 3 lymphocytes, normal glucose and a protein of 2.64 g/L. Oligoclonal bands were negative. MRI with gadolinium showed diffuse enhancement of the lumbar nerve roots (fig 1). Nerve conduction studies are shown in table 1 (sensory studies normal; sural sensory action potential (SAP), 30 mV, median SAP, 12.5 mV). Unfortunately, abnormal amplitude reduction after proximal stimulation and abnormal temporal dispersion were not sought by the
Sub-acute demyelinating neuropathy associated with an NK/T cell lymphoma Extranodal NK/T-cell lymphoma of nasal type is a rare non-Hodgkin’s lymphoma. We report on a patient who developed a
Figure 1 Axial T1-weighted MRI of lumbar spine showing diffuse enhancement of the lumbar nerve roots. J Neurol Neurosurg Psychiatry April 2008 Vol 79 No 4
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Bilateral mesial temporal polymicrogyria: a case report Giorgi Kuchukhidze, Raimund Helbok, Iris Unterberger, et al. J Neurol Neurosurg Psychiatry 2008 79: 483-484
doi: 10.1136/jnnp.2007.138792
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