Fluctuation of Computed Tomographic Findings in White Matter in Alexander's Disease (original) (raw)

Localized morphea: a rare adverse effect of valproic acid

Pediatric Neurology, 2003

Morphea, a specific type of cutaneous sclerosis, is known in the pediatric age group, but not as an adverse effect to the antiepileptic valproic acid. We report a 14-year-old male patient with absence seizures who, after treatment with valproic acid, developed skin tightness on the fingers. A biopsy specimen revealed dermal sclerosis consistent with morphea. His symptoms resolved gradually after discontinuation of the medication. To our knowledge, this is the first patient reported to have an apparently valproic acid-induced localized morphea.

A patient with SUNCT syndrome responsive to sodium valproate

The Journal of Headache and Pain, 2002

SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) was first described in 1989 [1]. It is a rare and painful syndrome characterized by strictly unilateral, short-lasting (5-120 seconds) attacks associated with signs of local involvement of the autonomic system. The most important (so to be part of the acronym SUNCT) are conjunctival injection and tearing [2, 3]. Other signs are often, but not always, described: nasal stiffness, rhinorrhea, narrowing of the palpebral fissure, eyelid edema and miosis. These signs and symptoms are strictly ipsilateral to the pain. The pathophysiology of this headache form is still unknown. Goadsby and Lipton [4] proposed classifying SUNCT syndrome as a trigeminal autonomic cephalalgia (TAC), together with cluster headache (CH), chronic paroxysmal migraine (CPH), trigeminal neuralgia (TN) and others. The treatment of SUNCT syndrome has been disappointing until 1999, when D'Andrea et al. [5] reported a therapeutic effect of lamotrigine. Based on the present case report of an elderly man with SUNCT, sodium valproate may also be an effective treatment for this debilitating headache. Case report A 74-year-old man reported experiencing, since the age of 70 years, pain attacks involving first the forehead and the periorbital region, and then spreading to the nose and upper lip. These areas were involved strictly in the left side. The attacks had a high intensity and an abrupt onset, lasted 10-30 seconds and occurred almost every day between 6:00 and 8:00 AM or, more rarely, between 2:00 and 4:00 PM.

Valproate-acid-induced cutaneous leukocytoclastic vasculitis

Psychiatria Danubina, 2012

Vasculitis is an inflammatory disease of blood vessels characterized by the alteration or destruction of the vessel wall. There are three subtypes of vasculitis according to Chapel Hill Consensus Conference defi-nition: Large Vessel Vasculitis, Medium-Sized Vessel Vasculitis and Small Vessel Vasculitis. Drug-induced vasculitis is usually Small Vessel Vasculitis represented as Leukocytoclastic Vasculitis. Leukocytoclastic Vasculitis is relatively common, with an incidence of 20:100 000 yearly. There are no age or sex predictions. The key event is the deposition of immune complexes in the walls of the capillaries and venules. In most cases, the process is a type III immune complex-mediated reaction according to the Gell and Coombs's classi-fication of hypersensitivity reactions. Typical triggers for acute disease include infections and drugs, while recurrent disease is usually associated with hepatitis C infection, collagen-vascular disorders, hematological diseases or malignancy....

Valproic acid-induced abnormal behavior

Indian Journal of Psychiatry, 2010

last six weeks. Her past medical history revealed that 14 weeks ago she was hospitalized following three episodes of seizures in a day with up rolling of eyeballs, confusion and limbs stiffness. A diagnosis of complex partial seizure with secondary generalized seizure disorder was made. She was treated with tablet valproic acid 200 mg twice daily and tablet clobazam 5mg at night during her hospital stay. After 5 days of treatment she was discharged with refilled prescription of valproic acid and clobazam and advised for monthly visits. Patient was otherwise normal and had no known psychiatric history or previous episodes of abnormal behavior. After 13 weeks of treatment with valproic acid and clobazam, she was hospitalized with complaints of irrelevant talk, talking to self, wandering and singing devotional songs, decreased food intake and insomnia since about five weeks i.e. about eight weeks after initiation of valproic acid therapy. Upon hospitalization, tablet chlorpromazine 100mg once daily, tablet olanzapine 5mg thrice daily, tablet risperidone 3mg twice daily were started while she continued to receive valproic acid and clobazam at the same dose. She was discharged on day three of hospitalization with an advice to continue all medications prescribed during the hospital stay at same doses for 15 days. However, her complaints of wandering, insomnia and decreased food intake continued to

Ceftriaxone for Alexander’s Disease: A Four-Year Follow-Up

JIMD Reports, 2012

In 2010, we reported the successful clinical outcome related to a 20-month course of intravenous, cyclical ceftriaxone, in a patient with adult-onset Alexander's disease. We now provide evidence that the progression of the patient's signs/symptoms was halted and reversed with a 4-year-long extension of the trial.

The clinical spectrum of late-onset Alexander disease: a systematic literature review

Journal of Neurology, 2010

Following the discovery of glial fibrillary acidic protein (GFAP) mutations as the causative factor of Alexander disease (AxD), new case reports have recently increased, prompting a more detailed comprehension of the clinical features of the three disease subtypes (infantile, juvenile and adult). While the clinical pattern of the infantile form has been substantially confirmed, the lateonset subtypes (i.e., juvenile and adult), once considered rare manifestations of AxD, have displayed a wider clinical spectrum. Our aim was to evaluate the clinical phenotype of the adult and juvenile forms by reviewing the previously reported cases. Data were collected from previously published reports on 112 subjects affected by neuropathologically or genetically proven adult and juvenile Alexander disease. Although the late-onset forms of AxD show a wide clinical variability, a common pattern emerges from comparing previously reported cases, characterized by pseudobulbar signs, ataxia, and spasticity, associated with atrophy of the medulla and upper cervical cord on neuroimaging. Late-onset AxD cases can no longer be considered as rare manifestations of the disease. The clinical pattern usually reflects the topographic localization of the lesions, with adult cases displaying a predominant infratentorial localization of the lesions. Juvenile cases show clinical and radiological features which are intermediate between adult and infantile forms.

Infantile Alexander Disease: Case Report and Review of Literature

J Clin Diagn Res. 2017 Jun; 11(6): ZD14–ZD15, 2017

Go to: Case Report A five-year-old boy reported to the Department of Oral Medicine and Radiology, with painful decayed tooth in the upper right back jaw region for 10 days. The pain was mild, continuous, non-radiating in nature with no associated factors. The patient's parents gave history of delayed developmental milestones of their son. There was no history of consanguineous marriage of the patient's parents. They reported their son to be normal at birth with standard birth weight and head circumference. There was history of seizures six months after birth, with gradual psychomotor deficiencies and cognitive abnormalities, for which his parents sought paediatric consultation immediately at seven months of age. Initial consultation was inconclusive due to lack of diagnostic evidence and financial constrictions. However, he was put on phenytoin oral suspension twice daily, which controlled the symptoms. The mother also complained of gradually increasing feeding difficulties in the later months, accompanied by episodes of vomiting. Subsequently, few non-contributory medical investigations were done in the later years. Patient's family history was found to be insignificant. On general physical examination, gait and postural abnormality was noted (he could only walk few steps without support). The patient had discrepancy in understanding, inability to speak and respond and presented with social skill deficiency. The head circumference was found to be within normal limits but the occipital region appeared to be flattened. On intraoral examination, 54 and 55 were found to be grossly carious with hypoplastic teeth seen in the posterior region. The patient had to undergo extraction regarding the same. Henceforth, pre-anaesthetic evaluation was necessary and he was subjected to various haematological and radiological investigations. Haematological parameters were found to be normal. Postero-anterior view of skull showed no evidence of sutural defects. Lateral skull view revealed flattened occiput [Table/Fig-1].

Camptocormia, a new side effect of sodium valproate

Epilepsy Research, 1987

A mentally retarded 23-year-old woman with myoclonic astatic epilepsy developed an abnormal posture of extreme forward flexion, called camptocormia, during valproate monotherapy. Camptocormia occurred concomitantly with an increase of the plasma valproate level from about 330pmol/l up to 530pmol/l. Four weeks earlier she had developed hepatopathy during a regimen of carbamazepine, lynestrenol and sodium valproate. Hepatopathy subsided after the cessation of carbamazepine and lynestrenol. Camptocormia improved within a week after the reduction of the plasma valproate level to about 3OO~mol/l. Four months later reversible camptocormia was manifested again concomitantly with about the same plasma valproate levels as above. Accordingly, camptocormia is a dose-dependent side effect of valproate.