Epilepsy and Celiac Medline Abstracts (through May 1996)

TI- [Celiac disease with occipital calcifications: 2 late cases]
TI- Celiaquia con calcificaciones occipitales: dos casos tardios.
AU- Baquero M; Narciso ML; Garcia M; Perla C; Dominguez F
CS- Servicio de Neurologia, Hospital Universitari La Fe, Valencia.
JN- Med Clin (Barc); Dec 8 1995, 105 (20) p781-3, ISSN 0025-7753
PY- May 1996

AB- The association of celiac disease, epilepsy and occipital calcifications with initial clinical manifestations of epilepsy during the first two decades of life with an often progressive and variable course and clinical expression of malabsorption has recently been described. Two cases of celiac disease with occipital calcifications and a presentation with neurologic symptoms in adulthood are reported. The first case is that of a 40-year-old male who presented recurrent and alternating pure brachial monoparesis and later acute abdominal pain following which celiac sprue was diagnosed. The second case is that of a 53-year-old woman diagnosed with celiac sprue 20 years before, presenting permanent myoclonus in the lower limbs which were progressive in severity, ataxic march and generalized tonoclonic seizures. Both patients had bilateral occipital calcifications on CT and celiac disease was demonstrated on biopsy. The first case also showed marked signal alteration in the white matter on MRI. Celiac disease with cerebral calcifications presents also in adulthood with atypical clinical manifestations. Suspicion of celiac disease may be confirmed by non-invasive methods such as antigliadin and antiendomysium antibody determination. CT imaging is characteristic.

TI- Early onset bilateral calcifications and epilepsy.
AU- Nunes ML; da Costa JC; Severini MH
CS- pision of Neurology, So Lucas Hospital, Porto Alegre, RS, Brazil.
JN- Pediatr Neurol; Jul 1995, 13 (1) p80-2, ISSN 0887-8994
PY- Feb 1996

AB- Bilateral occipital calcifications associated with epilepsy and sometimes with celiac disease have been described previously. A boy with bilateral frontal and occipital diffuse calcifications accompanied by failure to thrive, nephrogenic diabetes insipidus, developmental delay and seizures, but without celiac disease is presented. Follow-up at 3 years of age disclosed neurodevelopmental delay, height and weight less than expected for age, and seizures controlled with carbamazepine. The uncommon association of these features and the early onset of symptoms is discussed. Although bilateral occipital calcifications share some clinical features with bilateral fronto-occipital calcifications, it is arguable whether the two are on a spectrum of a single disease or represent separate entities.

TI- Partial seizures, cerebral calcifications and celiac disease.
AU- Cernibori A; Gobbi G
CS- Servizio di Neuropsichiatria Infantile, Ospedale Civile di Sondrio.
JN- Ital J Neurol Sci; Apr 1995, 16 (3) p187-91, ISSN 0392-0461
PY- Jan 1996

AB- We describe the case of a 25 year old woman who has been clinically and instrumentally examined over a period of about 20 years. A diagnosis of celiac disease was made when she was four years old and, ten years later, CAT revealed the presence of bilateral cerebral calcifications. The partial occipital seizures were controlled by adopting a gluten-free diet, which is still being followed four years after the discontinuation of anti-epileptic treatment.

TI- Bilateral occipital calcification associated with celiac disease, folate deficiency, and epilepsy.
AU- Lea ME; Harbord M; Sage MR
CS- Department of Radiology, Flinders Medical Centre, Bedford Park, Australia.
JN- AJNR Am J Neuroradiol; Aug 1995, 16 (7) p1498-500, ISSN 0195-6108
PY- Feb 1996

AB- In a patient with celiac disease, folate and iron deficiency, and epilepsy, CT over a 4-month period showed parietoocipital calcifications in the corticomedullary junctions. The calcification was progressive, but it and the seizures stabilized after institution of a gluten-free diet and iron and folic acid supplements.

TI- The major complications of coeliac disease.
AU- Wright DH
CS- University Department of Pathology, Southampton General Hospital, UK.
JN- Baillieres Clin Gastroenterol; Jun 1995, 9 (2) p351-69, ISSN 0950-3528
PY- Jan 1996

AB- Neoplasms constitute the major complication of coeliac disease, and high-grade T-cell lymphoma of the small intestine (enteropathy- associated T-cell lymphoma) is the most common neoplasm in this category. HLA genotyping indicates that in patients with enteropathy-associated T-cell lymphoma have the coeliac disease associated DQA1*0501, DQB1*0201 phenotype, although additional HLA-DR/DQ alleles may represent risk factors for lymphoma development. Molecular biological and immunohistochemical studies have shown that the intestinal mucosa distant from the tumour contains clonal populations of small T cells, often of the same clone as the high-grade T-cell lymphoma. These findings suggest that enteropathy-associated T-cell lymphoma arises in the setting of coeliac disease and evolves from reactive intraepithelial lymphocytes through a low-grade lymphocytic neoplasm to a high-grade tumour, which is usually the cause of the presenting symptoms. Most cases of chronic ulcerative enteropathy (ulcerative jejunitis) are probably part of the same disease process. If the ulceration occurs at a time when the neoplastic T-cells are of a low grade, morphological recognition of tumour cells in the ulcers may be impossible. Carcinoma of the pharynx and oesophagus, and adenocarcinoma of the small intestine, are increased in frequency in patients with coeliac disease. The increased risk of carcinoma of the oesophagus may be related to vitamin A deficiency. A number of reports have indicated an increased prevalence of various types of chronic hepatitis in patients with coeliac disease, but no coherent view of the cause of this association has emerged. Similarly, patients with coeliac disease have been reported to have various forms of fibrosing lung disease of uncertain causation. In recent years, there have been several reports, mainly from Italy, of a syndrome of epilepsy and bilateral brain calcification occurring in coeliac patients. The pathogenesis of this condition is not known and its prevalence in other communities is uncertain. Splenic atrophy occurs frequently in patients with coeliac disease and is related to the severity of the disease and degree of dietary control. Splenic atrophy predisposes to infection with capsulated bacteria, although mortality studies indicate that infection with these organisms is not a major cause of death in patients with coeliac disease.

TI- Coeliac disease in adults.
AU- Corazza GR; Gasbarrini G
CS- University of L'Aquila, Italy.
JN- Baillieres Clin Gastroenterol; Jun 1995, 9 (2) p329-50, ISSN 0950-3528
PY- Jan 1996

AB- Coeliac disease is a chronic disease characterized by small bowel villous atrophy which impairs nutrient absorption and improves on withdrawal of wheat gliadins and barley, rye and oat prolamins from the diet. Knowledge of the adult form of coeliac disease has greatly improved in recent years. Although this knowledge is not yet sufficiently widespread among referring clinicians, it has, over the past few years, allowed an increasing number of patients to be diagnosed with subclinical forms characterized by minor, transient or apparently unrelated symptoms. As a consequence, our views on the clinical and epidemiological aspects of this condition, the prevalence of which in the general population is believed to be close to 1 in 300, have changed and are still changing. Since it has been demonstrated that a strict gluten-free diet is protective against the complications of adult coeliac disease, it is important that even subclinical and silent forms are diagnosed and treated as early as possible. Non- invasive screening tests, such as anti-gliadin and anti-endomysium antibody estimation, should therefore be used systematically in groups considered to be at risk of coeliac disease. These include first-degree relatives of coeliac patients and patients with insulin-dependent diabetes mellitus, iron-deficiency anaemia, epilepsy with cerebral calcification, recurrent aphthous stomatitis and dental enamel hypoplasia. Other conditions will probably be identified in the near future.

TI- Celiac disease and epilepsy in pediatric patients.
AU- Fois A; Vascotto M; Di Bartolo RM; Di Marco V
CS- Institute of Clinical Pediatrics, University of Siena, Italy.
JN- Childs Nerv Syst; 10 (7) p450-4
PY- Sep 1994

AB- Among 783 patients referred to our institute with different types of seizures as presenting symptom, systematic evaluation of antigliadin and antiendomysial antibodies in the serum has identified nine in whom jejunal biopsy has subsequently confirmed the diagnosis of celiac disease (CD). In three of them brain imaging showed the presence of calcified areas in the occipital region. They had complex partial seizures (CPS), associated in two with transient episodes of blindness. In another patient with CPS and generalized tonic-clonic seizures (GTCS) progressive multifocal cerebral calcifications were noted. In the other six patients with CPS and/or GTCS cerebral calcifications were absent. Symptoms of CD in all these cases were either not previously taken into account, or they were very mild or completely absent. In a group of 36 patients with clinically manifest CD, regular follow-up, and good compliance with the dietary regimen, no clinical seizures were reported. The pathogenetic mechanism and the relationship between epilepsy and an early diagnosis and treatment of celiac disease are discussed.

TI- [A misleading depression] TI- Une depression trompeuse.
AU- Cheliout W
CS- Fondation John Bost, La Force.
JN- Encephale; 20 (5) p531-4
PY- Sep-Oct 1994

AB- The present paper relates the case of a young female patient who suffers from epilepsy and depressive disorder. The discovery of digestive disturbances associated to an anaemia and various tests induces a diagnosis of a coeliac disease. Such relationship is unusual with depression and epilepsy. The author insists on the fact that a diagnostic cannot fix a person in a unique mode of attendance because a disease can mask another one.

TI- [Convulsive disorder in celiac disease]
AU- Cohen O; River Y; Zelinger I
CS- Dept. of Neurology, Hadassah-University Hospital, Jerusalem.
JN- Harefuah; 126 (12) p707-10, 763
PY- Jun 15 1994

AB- Several recent reports have described convulsions in patients with celiac disease, and in some, folic acid deficiency and brain calcifications. A 40-year-old woman with celiac disease, hypocalcemia and generalized tonic-clonic seizures is reported. Hypocalcemia was corrected and convulsions disappeared, but the EEG showed persistent occipital epileptiform activity. Patients with celiac disease and hypocalcemia due to malabsorption are particularly at increased risk for convulsions. Therefore, even a mild degree of hypocalcemia in these patients should be corrected as soon as possible.

TI- [The role of endomysium antibodies in the diagnosis and monitoring of celiac disease]
TI- Ruolo degli anticorpi anti endomisio nella diagnosi e nel monitoraggio della malattia celiaca.
AU- Calvani M; Parisi G; Giannelli C; Ceri E; Graziani MG
CS- pisione Pediatrica, Ospedale S. Camillo de Lellis, USL, Roma.
JN- Recenti Prog Med; 85 (6) p318-22
PY- Jun 1994

AB- Coeliac disease is a common cause of chronic diarrhea in children and adults. It is also frequently detected in children exclusively affected by iron deficiency anemia, hypocalcemia, short stature, dental enamel defects, epilepsy and intracranial calcifications, etc. The coeliac disease diagnosis may be facilitated by the use of some immunological tests like anti endomysial (AEA) or anti gliadin (AGA) antibodies detection. From December 1990 to September 1992 anti endomysial IgA and anti gliadin IgG antibodies were respectively detected in 1680 and 1598 sera from children and adults affected by chronic diarrhea, failure to thrive or other symptoms compatible with coeliac disease diagnosis. According to ESPGAN criteria at that time coeliac disease diagnosis was made in 73 cases. In our experience AEA IgA show to have a better sensitivity and specificity in the diagnosis of coeliac disease rather than AGA IgG (97.5% vs 95.1% and 99.5% vs 98.3% respectively).

TI- Need for follow up in coeliac disease.
AU- Bardella MT; Molteni N; Prampolini L; Giunta AM; Baldassarri AR; Morganti D; Bianchi PA
CS- Department of Gastroenterology, Istituto di Scienze Mediche, Milan, Italy.
JN- Arch Dis Child; 70 (3) p211-3
PY- Mar 1994

AB- The use of follow up studies was evaluated in 128 patients with coeliac disease during their first visit to a department for adults. The original diagnosis had been made in childhood in all patients. Fifty eight (45%) of the subjects were following a gluten free diet, 23 (18%) were following a gluten free diet but with occasional gluten consumption, and 47 (37%) had adopted an unrestricted, gluten containing diet for a mean of 11.2 years. There was no correlation in inpidual subjects between the presence of symptoms, biochemical and immunological abnormalities, severity of histological findings, and the amount of dietary gluten, despite the greater frequency of symptoms in the group following an unrestricted diet than in the other two groups. Short stature and epilepsy with cerebral calcifications only occurred in patients following an unrestricted diet. As only diagnosis based on two or three biopsy samples and regular follow up correlated positively with dietary compliance, it is suggested that a histologically confirmed diagnosis of coeliac disease and regular lifelong follow up are essential in the management of these patients.

TI- Familial unilateral and bilateral occipital calcifications and epilepsy.
AU- Tortorella G; Magaudda A; Mercuri E; Longo M; Guzzetta F
CS- Istituto di Neuropsichiatria Infantile, University of Messina, Italy.
JN- Neuropediatrics; 24 (6) p341-2
PY- Dec 1993

AB- No familial cases of epilepsy associated with bilateral occipital calcifications (EBOC) have been reported so far. This paper describes the clinical, electrophysiological and radiological study of two sisters affected by partial epilepsy, one with unilateral and the other with bilateral occipital calcifications. Celiac disease was excluded in both patients, even though they presented the same HLA pattern usually found in coeliac subjects.

TI- Endocranial calcifications, infantile celiac disease, and epilepsy.
AU- Piattella L; Zamponi N; Cardinali C; Porfiri L; Tavoni MA
CS- Children's Neuropsychiatric Ward, Regional Pediatric Hospital G. Salesi, Ancona, Italy.
JN- Childs Nerv Syst; 9 (3) p172-5
PY- Jun 1993

AB- The authors report on five patients (three female, two male) with multiple brain calcifications, infantile celiac disease, and epilepsy. The clinical, neuroradiological, neurophysiological, EEG and evolutional aspects are assessed. The authors propose that all patients with brain calcifications which cannot be traced to other known pathologies should undergo diagnostic tests for a malabsorption syndrome; analogously, patients affected with infantile celiac disease should undergo EEG, followed by a neuroradiological examination if the EEG pattern is found to be altered.

TI- Cortical vascular abnormalities in the syndrome of celiac disease, epilepsy, bilateral occipital calcifications, and folate deficiency.
AU- Bye AM; Andermann F; Robitaille Y; Oliver M; Bohane T; Andermann E
CS- Neurology Department, Prince of Wales Children's Hospital, Sydney, Australia.
JN- Ann Neurol; 34 (3) p399-403
PY- Sep 1993

AB- The pathological changes in the syndrome of celiac disease, folate deficiency, bilateral occipital calcifications, and intractable epilepsy have not been previously described. A child with this disorder had a field defect correlating with active lateralized epileptic discharges and asymmetrical lesions. After resection of the right occipital lobe she was seizure free for 4 years. A cortical vascular abnormality with patchy pial angiomatosis, fibrosed veins, and large jagged microcalcifications was found. These pathological abnormalities were similar though not identical to those found in the Sturge-Weber syndrome.

TI- Bilateral occipital calcification, epilepsy and coeliac disease: clinical and neuroimaging features of a new syndrome.
AU- Magaudda A; Dalla Bernardina B; De Marco P; Sfaello Z; Longo M; Colamaria V; Daniele O; Tortorella G; Tata MA; Di Perri R; et al
CS- Institute of Neurological and Neurosurgical Sciences, University of Messina, Italy.
JN- J Neurol Neurosurg Psychiatry; 56 (8) p885-9
PY- Aug 1993

AB- Twenty patients affected by bilateral occipital cortical-subcortical calcification (BOC) are described, 19 (95%) had epilepsy. In 8 of 16 cases studied, intestinal biopsy revealed coeliac disease. Fourteen patients had occipital partial epilepsy with a relatively benign outcome, while 4 patients were affected by a severe form of epilepsy, with very frequent, drug-resistant, generalised and partial seizures with mental deterioration. One patient had a single episode of convulsive status epilepticus at four months of age. The neurological examination was normal in all patients. CT showed flocculo-nodular, cortico-subcortical BOC, without enhancement and without lobar or hemispheric atrophy. MRI was normal. The clinical and neuroimaging features of these patients are different therefore from those with the Sturge-Weber Syndrome. The study confirms a high prevalence of coliac disease in patients with BOC, but the relationship between these two pathologies still needs to be clarified.

TI- Progressive cerebral calcifications, epilepsy, and celiac disease.
AU- Fois A; Balestri P; Vascotto M; Farnetani MA; Di Bartolo RM; Di Marco V ; Vindigni C
CS- Department of Pediatrics, University of Siena, Italy.
JN- Brain Dev; 15 (1) p79-82
PY- Jan-Feb 1993

AB- A case with progressive cerebral calcifications, white matter involvement, and drug-resistant epilepsy in a 9-year-old boy is described. The final diagnosis was celiac disease (CD). The relationship of CD with epileptogenic lesions is considered, and the possible significance of this association is discussed.

TI- Epilepsy with bilateral occipital calcifications: Sturge-Weber variant or a different encephalopathy?
AU- Tiacci C; D'Alessandro P; Cantisani TA; Piccirilli M; Signorini E; Pelli MA; Cavalletti ML; Castellucci G; Palmeri S; Battisti C; et al
CS- Unita Organica di Neurofisiopatologia, Ospedale Policlinico, Perugia, Italy.
JN- Epilepsia; 34 (3) p528-39
PY- May-Jun 1993

AB- A series of cases of epilepsy with associated bilateral occipital calcifications (EBOC) without signs of phakomatosis and without any disorders known to produce cerebral calcifications have been reported. It is unclear whether EBOC is an incomplete variant of Sturge-Weber disease (SWD) or if it is a different, as yet undefined encephalopathy. We describe four new cases of EBOC that are different clinically by age of onset, type, course, severity of epilepsy, and associated cognitive deficits but that are linked by similar neuroradiologic findings. Similar to cases described in the literature, there is convincing evidence in favor of the hypothesis that these cases belong to an encephalopathy different from SWD and frequently associated with celiac disease.

TI- Cerebral occipital calcifications in celiac disease.
AU- Crosato F; Senter S
CS- pision of Neuropsychiatry, Angeli Custodi Children's Hospital, Trento, Italy.
JN- Neuropediatrics; 23 (4) p214-7
PY- Aug 1992

AB- Bilateral occipital calcifications, occurring in celiac disease, are factors coming under a particular cerebral syndrome, which also includes epilepsy, migraine-like headache, visual troubles and mental deterioration. They seem to arise from hypofolatemia following gluten-induced enteropathy.

TI- Coeliac disease, epilepsy, and cerebral calcifications. The Italian Working Group on Coeliac Disease and Epilepsy
AU- Gobbi G; Bouquet F; Greco L; Lambertini A; Tassinari CA; Ventura A; Zaniboni MG
CS- pisione di Neuropsichiatria Infantile, Istituto per l'Infanzia, Trieste, Italy.
JN- Lancet; 340 (8817) p439-43
PY- Aug 22 1992

AB- There have been anecdotal reports of an association between coeliac disease and epilepsy with cerebral calcifications that resemble those of the Sturge-Weber syndrome. A series of patients who had epilepsy with calcifications, in whom coeliac disease (CD) was incidentally observed, prompted us to study this association. 43 patients (15 male, age range 4.6-30.7 years) were selected from two series. 31 patients with cerebral calcifications of unexplained origin and epilepsy (series A) underwent intestinal biopsy. 12 patients with CD and epilepsy (series B) underwent computed tomography. Antibodies to gluten, folic acid serum concentrations, were measured, and HLA typing was done in most patients. 24 of the series A patients were identified as having CD on the basis of a flat intestinal mucosa (15/22 with a high concentration of serum antigluten), and 5 series B patients showed cerebral calcifications, giving a total of 29 cases with the combination of CD, epilepsy, and cerebral calcifications (CEC). In 27 of these CEC patients, calcifications were located in the parieto-occipital regions. Only 2 of the series A patients had gastrointestinal symptoms at the time of intestinal biopsy; most patients had recurrent diarrhoea, anaemia, and other symptoms suggestive of CD in the first 3 years of life. The epilepsy in CEC patients was poorly responsive to antiepileptic drugs. Gluten-free diet beneficially affected the course of epilepsy only when started soon after epilepsy onset. Cases of "atypical Sturge-Weber syndrome" (characterised by serpiginous cerebral calcifications and epilepsy without facial port-wine naevus) should be reviewed, and CD should be ruled out in all cases of epilepsy and cerebral calcifications of unexplained origin.

TI- Occipital lobe seizures related to clinically asymptomatic celiac disease in adulthood.
AU- Ambrosetto G; Antonini L; Tassinari CA
CS- Institute of Neurology, University of Bologna, Italy.
JN- Epilepsia; 33 (3) p476-81
PY- May-Jun 1992

AB- We report the electroclinical ictal findings of four epileptic patients with clinically asymptomatic celiac disease (CD). Celiac disease diagnosis was suspected by past history and/or computed tomography (CT) findings in all patients and confirmed by laboratory tests and jejunal biopsy. All patients had paroxysmal visual manifestations and ictal EEG discharges arising from the occipital lobe. Epilepsy evolution was favorable in two patients and severe in 2, regardless of CT evidence of occipital corticosubcortical calcifications in 2 patients. Occipital lobe seizures may be characteristic of the epilepsy related to CD, and epileptic patients with these seizures of unknown etiology should be carefully investigated for malabsorption. If past history and/or laboratory tests suggest gastrointestinal (GI) dysfunction they should also undergo small intestinal biopsy even if they do not have GI tract symptoms.

TI- Celiac disease, posterior cerebral calcifications and epilepsy.
AU- Gobbi G; Ambrosetto P; Zaniboni MG; Lambertini A; Ambrosioni G; Tassinari CA
CS- Neurological Institute, University of Bologna, Italy.
JN- Brain Dev; 14 (1) p23-9
PY- Jan 1992

AB- Ten patients (5 males) affected by epilepsy with cerebral calcifications of unknown etiology mainly located in the posterior regions were subjected to a battery of tests including an intestinal biopsy. Our aim was to establish whether or not the patients also suffered from celiac disease. Celiac diseases was found in 6 patients. This result and the inpidual cases reported in the literature suggest that this triad of diseases (celiac disease, posterior cerebral calcifications and epilepsy) are casually related. The same HLA phenotype was found in all 10 patients, i.e., including the cases without celiac disease, suggesting an underlying disorder of the immune system. Our results emphasize that particular attention should be paid to a search for celiac disease in all patients with epilepsy and posterior cerebral calcifications.

TI- [Intracranial calcifications--seizures--celiac disease: a case presentation]
TI- Calcificazioni endocraniche--convulsioni--celiachia: presentazione di un caso.
AU- Della Cella G; Beluschi C; Cipollina F
CS- pisione Pediatria, Ospedale di Chiavari (GE), Italia.
JN- Pediatr Med Chir; 13 (4) p427-30
PY- Jul-Aug 1991

AB- The Authors report a case of coeliac disease which first appeared in a boy of 4, suffering from a seizure disorder. The bulky mass of fatty faeces led towards gastroenteric investigations (xylose-test, jejunum biopsy). The atrophy of the villi was clearly shown by the biopsy and a coeliac disease was easily diagnosed. The boy was prescribed a coeliac diet and no fits of generalized convulsions occurred during three years follow-up. Yet, while he was given a challenge free-diet, they started to occur. A computerized axial tomography (TAC) carried out when he was eight and another when fifteen, evidenced bilateral, intracranial calcifications, cortical-subcortical, in the blood vessels, symmetrically located in the occipital region. The anticonvulsive therapy, started when he was 4, has never been interrupted. Now A.M. is 21 and still following an anticonvulsive polytherapy. Many tests were performed. The result of folic acid dosage carried out when he was 16, and badly follow a coeliac diet, was less than 2 ng/ml. A modification in the neurological symptomatology was noted during his puberal phase: fits of convulsions changed into daily crises of mind-failures. This feature is still present in his adult age. The case is reported for its clinical characteristics of neurological symptomatology associated with coeliac disease. The iconographical documentation evidences endocranial calcifications frequently connected with coeliac disease.

TI- Coeliac disease, folic acid deficiency and epilepsy with cerebral calcifications.
AU- Ventura A; Bouquet F; Sartorelli C; Barbi E; Torre G; Tommasini G
CS- Department of Paediatrics, University Hospital, Istituto per l'Infanzia Burlo Garofolo, Italy.
JN- Acta Paediatr Scand; 80 (5) p559-62
PY- May 1991

AB- Two cases of focal occipital epilepsy with cerebral calcifications poorly responsive to antiepileptic treatment are described. In both cases coeliac disease was diagnosed and folic acid deficiency documented. A gluten-free diet and a brief supplementation with folic acid lead to a complete EEG and clinical normalization in one case and to a significant improvement of EEG and seizure control in the other.

TI- Ramsay Hunt syndrome and coeliac disease: a new association? [see comments]
AU- Lu CS; Thompson PD; Quinn NP; Parkes JD; Marsden CD
CS- University Department of Neurology, King's College Hospital Medical School, London, England.
JN- Mov Disord; 1 (3) p209-19
PY- 1986

AB- Two patients with the syndrome of Ramsay Hunt (dyssynergia cerebellaris myoclonica, DCM), associated with malabsorption due to adult coeliac disease, are reported. Both presented with progressive cerebellar ataxia, action myoclonus, and epilepsy. One had gastrointestinal symptoms (recurrent diarrhea and weight loss which responded satisfactorily to a gluten-free diet), but the other did not. In both patients, jejunal biopsy revealed subtotal villous atrophy; serum folate and vitamin E level were also reduced. Neither a gluten-free diet nor vitamin supplements improved the neurological picture. However, some symptomatic relief was afforded by treatment with clonazepam, sodium valproate, carbamazepine, and piracetam. It could be argued that the association between these two disorders is coincidental. However, since we have found this combination in 2 of 14 consecutive cases with DCM, a causal relationship seems likely, although the underlying mechanism remains unknown. Patients with the Ramsay Hunt syndrome should be investigated for malabsorption, and also undergo small intestinal biopsy.

TI- Celiac disease associated with epilepsy and intracranial calcifications: report of two patients.
AU- Molteni N; Bardella MT; Baldassarri AR; Bianchi PA
CS- Istituto di Scienze Mediche, Universita di Milano, Italy.
JN- Am J Gastroenterol; 83 (9) p992-4
PY- Sep 1988

AB- We describe two young adult patients with seizures and cerebral calcifications since childhood, diagnosed as Sturge Weber syndrome, who also had gluten enteropathy. Although the calcifications were located in regions similar to calcifications of Sturge Weber cases, many of the features of the syndrome were absent, and this diagnosis seems improbable. Whereas a coincidental involvement cannot be excluded, attention is drawn to this association between celiac disease and seizures with intracranial calcifications mimicking a Sturge Weber syndrome. After a gluten-free diet, antiepileptic therapy could be reduced in our patients.

TI- Bilateral cerebral occipital calcifications and migraine-like headache.
AU- Battistella PA; Mattesi P; Casara GL; Carollo C; Condini A; Allegri F; Rigon F
JN- Cephalalgia; 7 (2) p125-9
PY- Jun 1987

AB- Computed tomography scanning in two young patients with recurrent, pulsating, migraine-like headache showed parieto-occipital calcifications. One patient presented with an atypical form of the Sturge-Weber syndrome, and the other with celiac disease and folic acid deficiency. The clinical features were analyzed and compared with those in other cases reported in the recent literature which have shown bioccipital calcifications but no cutaneous angiomas, sometimes associated with visual and/or intelligence deficit and epilepsy. Finally, the possible connection between cerebral calcifications and headache is discussed.

TI- Blood selenium content and glutathione peroxidase activity in children with cystic fibrosis, coeliac disease, asthma, and epilepsy.
AU- Ward KP; Arthur JR; Russell G; Aggett PJ
JN- Eur J Pediatr; 142 (1) p21-4
PY- Apr 1984

AB- Long-term selenium status in children from the North-East of Scotland was estimated using whole blood selenium content (BSe) and glutathione peroxidase activity (BGSH-Px). BSe was significantly lower than the reference range in children with cystic fibrosis, coeliac disease and in older patients with phenylketonuria. Whereas BGSH-Px of all the children with coeliac disease and those with cystic fibrosis aged over 6 years matched the reference range, it was reduced in younger patients with cystic fibrosis and in children with dietetically treated phenylketonuria. No child had clinical features of selenium deficiency. BSe in treated epileptics and asthmatics conformed to the reference range, but BGSH-Px in both groups was increased significantly; this was most evident in those receiving corticosteroid preparations.

TI- Medium-chain triglycerides: an update.
AU- Bach AC; Babayan VK
JN- Am J Clin Nutr; 36 (5) p950-62
PY- Nov 1982

AB- A review of the literature on the medical and nutritional use of medium-chain triglycerides (MCTs) since 1970 is presented with additional discussions on the various modifications and applications of the MCTs in the synthesis of certain structured lipids. The metabolism of MCTs in the liver and extrahepatic tissues is discussed along with further documentation of the use of MCTs in malabsorption and hyperlipidemia cases. Recent applications of MCTs and modified MCTs in hyperalimentation, deficiency in the carnitine system, epilepsy, obesity, and other special areas of application are cited. The use of medium-chain monodiglycerides for dissolving cholesterol gallstones is presented. The contraindications for the use of MCTs in ketosis, acidosis, and cirrhosis are also discussed. Suggestions for use of MCTs in a variety of medical and nutritional applications are presented.

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