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Infantile spasms associated with theophylline toxicity.

We report on a 6-month-old infant with asthma who developed spasms and hypsarrhythmia on the electroencephalogram (EEG) shortly after starting oral theophylline medication. Theophylline levels at that time were just above the upper normal range. The spasms stopped and the EEG normalized when theophylline was discontinued and nitrazepam therapy started. On follow-up over the next 3 years there was no recurrence of seizures and the child's neurodevelopment has been normal. Nitrazepam was stopped at 10 months and the waking and sleeping EEG were normal at 14 months. We believe that the infantile spasms were caused by theophylline.

Electroencephalography↗

Infantile spasms and early immunization against whooping cough. Danish survey from 1970 to 1975.

113 cases of infantile spasms were diagnosed in the period 1 April 1970 to 31 March 1975 after Denmark changed her immunization programme. Previously whooping cough vaccine was given as the triple combination at 5, 6, and 15 months of age. From 1 April 1970 it was given as a monovalent vaccine at 5 and 9 weeks of age and at 10 months of age. A comparison with previous material of 86 cases of infantile spasms from 1957 to 1967 showed no change in the age at onset. Though there may be an occasional connection between immunization and infantile spasms, the most important factor is a time-coincidence between any immunization given at a certain age with the neurological disorders which are natural for that age.

Denmark↗

Family occurrence of achalasia and diffuse spasm of the oesophagus.

In view of the unknown aetiology of achalasia and diffuse oesophageal spasm we report four families (father/son, mother/son, brother/brother, cousin/cousin) with achalasia and oesophageal spasm examined by radiology, endoscopy and manometry. Family occurrence of oesophageal motor disorders supports the hypothesis that a genetic trait may play a role in the pathogenesis. The family coincidence of achalasia and oesophageal spasm supports a close relationship between the two diseases.

Adult↗

Repeat microvascular decompression for hemifacial spasm.

OBJECTIVE: To report our experience with repeat microvascular decompression (MVD) for hemifacial spasm (HFS) in patients who have failed their first operation. METHODS: The authors describe 41 redo MVDs for HFS in 36 patients performed over a 3 year period. Seven patients underwent early re-operation after an aborted seventh nerve decompression. Eight patients underwent early re-operation for clinical failure. Eighteen patients underwent late re-operation for spasm recurrence long after their original MVD. Eight MVDs were performed on patients who had already undergone at least two prior operations. RESULTS: Twenty four patients experienced complete spasm resolution (70.6%), eight patients had near total resolution (23.5%), and two patients failed re-operation (5.9%). Two patients were lost to follow up (5.6%). A favourable outcome was reported by 82.4% of patients at a mean follow up interval of 18 months. A total of 91.7% of patients 50 years of age or younger were cured at follow up versus 59.1% of patients older than 50 (p = 0.04). Patients undergoing early re-operation were significantly more likely to be cured or improved than patients undergoing late re-operation (p = 0.03). CONCLUSIONS: Repeat MVD for HFS is effective in experienced hands. Younger patients respond better to repeat MVD. Late repeat MVD for HFS is a reasonable treatment option, although results are less favourable than for early re-operation.

Adult↗

Hemifacial spasm: treatment by posterior fossa surgery.

Nine cases of hemifacial spasm have been treated by posterior fossa exploration without mortality or significant morbidity. In only three was definite pathology found, but the hemifacial spasm was abolished in eight patients and markedly diminished in the remaining patient. The condition has reccurred in one patient. Microsurgical techniques make the operation safe and accurate. We suggest that this procedure is the best approach for hemifacial spasm requiring treatment. Where no definite pathology is found, the effectiveness of the procedure is probably due to fibrosis and hence mild trauma to the facial nerve induced by the sponge wrapped around the nerve.

Adult↗

Hemifacial spasm: a long term follow-up of patients treated by posterior fossa surgery and facial nerve wrapping.

Sixteen patients with hemifacial spasm were treated by posterior fossa surgery and wrapping of sponge around their facial nerve. A good or excellent result has been obtained in fourteen of the sixteen cases, and in seven cases followed by four years or longer. Two patients had a mild recurrence of their hemifacial spasm after a cessation of their spasm for eighteen months and two years following surgery. Contrary to the experience of other authors a definite vascular abnormality was found in only four cases. In the other twelve cases circumferential fibrosis about the nerve is again proposed as a mechanism for the effectiveness of the procedure.

Adult↗

Primary hemifacial spasm: a neurophysiological study.

A series of 53 cases of primary hemifacial spasm have been evaluated by means of blink reflexes and their results compared with a normal control group. Reflex responses were obtained by percutaneous electrical stimulus of both the supraorbital nerve (trigemino-facial reflex), and the facial nerve at the stylo-mastoid region (facio-facial reflex). The R2 response was considered abnormal when its latency was shortened (hyperactivity) or delayed (hypoactivity). Thirty-six out of 53 cases with primary hemifacial spasm showed abnormal responses, with a combination of facial nerve impairment (delayed R2 in the facio-facial reflex) and trigeminal-facial hyperactivity (shortened R2 in the trigemino-facial reflex). Five cases showed hyperactivity in both the trigemino-facial reflex and the facio-facial reflex reflexes. These results suggest a state of hyperexcitability, probably at the level of the facial nucleus, combined with a peripheral facial nerve involvement in a high proportion of patients with primary hemifacial spasm.

Adult↗

Focal dystonia of the jaw and the differential diagnosis of unilateral jaw and masticatory spasm.

The clinical features, differential diagnosis and treatment of unilateral spasms of the jaw and masticatory muscles are discussed and illustrated by eight cases of unilateral jaw spasms of various aetiologies. These include focal dystonia of the jaw, hemimasticatory spasm with and without facial hemiatrophy, paroxysmal events in multiple sclerosis and tetany. Attention is particularly drawn to four cases of unilateral dystonia of the jaw which has not been described before.

Adult↗

Jaw closing spasm--a form of focal dystonia? An electrophysiological study.

The case of a 36 year old man suffering from unilateral right jaw closing spasms over two years is reported. Permanent spasm with trismus severely impeding mouth-opening was combined with paroxysms triggered by various sensory stimuli. The diagnosis of temporo-mandibular joint syndrome was considered but treatment failed to improve the symptoms. Neurological investigation two years after onset of the spasms showed by electrophysiological studies excessive co-contraction of the antagonistic jaw-closers, mainly the right masseter during attempts at jaw opening and absence of the silent period in the right masseter and anterior temporalis following jaw tap and trigeminal exteroceptive stimulation. Jaw dystonia was therefore considered and Botulinum A toxin was injected into the right masseter and temporalis which dramatically improved the patient's condition.

Adult↗

Hemifacial spasm in tuberculous meningitis.

Hemifacial spasm developed in a 47-year-old man with tuberculous meningitis. The spasms ceased completely following vigorous antituberculous treatment. A selective compression of the facial nerve root along its exit at the brainstem by a localised inflammatory process is the most tenable explanation for the development of the hemifacial spasm.

Facial Muscles↗

Spontaneously and pharmacologically provoked coronary arterial spasm in Prinzmetal variant angina.

Eleven of 21 consecutive patients with Prinzmetal angina (PMA) exhibited no significant fixed stenoses of the coronary arteries. Spontaneous coronary arterial spasm was demonstrated in 3 patients. Ergonovine maleate produced near-total occlusion of a major vessel in 3 of 4 other patients with PMA, but did not provoke spasm in 10 without PMA. The current study documents spasm as the mechanism of myocardial ischemia in some patients with normal coronary arteries and provides initial and favorable diagnostic results with provocative pharmacoangiography in this entity.

Angina Pectoris↗

Hemifacial spasm: three-dimensional MR images in the evaluation of neurovascular compression.

PURPOSE: To assess the diagnostic accuracy of three-dimensional (3D) magnetic resonance (MR) images in the preoperative evaluation of hemifacial spasm. MATERIALS AND METHODS: In 27 patients with surgically confirmed hemifacial spasm, the axial source images from 3D time-of-flight MR angiography were evaluated for 27 symptomatic sides and 26 asymptomatic sides by three observers in a blinded manner for neurovascular compression and identification of compressive vessels. RESULTS: Sensitivity was 95% and specificity was 77%. Vascular compression on the symptomatic sides could be identified retrospectively in all 27 patients. On the asymptomatic sides, vascular compression could be excluded retrospectively in all but one patient. In the blinded study, as the mean value of three observers, the vertebral artery and posterior inferior cerebellar artery could be identified correctly in seven and 7.7 of seven and nine patients, respectively, whereas the compressive anterior inferior cerebellar artery was identified correctly in 7.7 of 16 patients. CONCLUSION: Three-dimensional MR images have a high diagnostic accuracy and are useful in the preoperative evaluation of hemifacial spasm.

Adult↗

Hypomagnesemia and smooth muscle contractility: diffuse esophageal spasm in an old female patient.

The aim of this paper is to describe and discuss, on the basis of the available literature, the case of an old female patient, admitted to our university hospital because of a severe dysphagia for solid foods, in whom laboratory data showed a marked hypomagnesemia. She reported a long history (20 years) of allergic bronchial asthma treated with theophylline. Esophagography evidenced a disorder of esophagus motility with diffuse multiple spasm, reminiscent of the 'corkscrew esophagus'. A link with the severe hypomagnesemia (Mg 1.1 mEq/l, normal range 1.6-2.1) was suspected, and a therapy with oral pidolate of Mg (1.5 g/twice a day) was started and continued for 4 months. This was associated with a slow progressive normalization of the Mg plasma level and reverted radiographic esophageal findings with disappearance of dysphagia. Mg is an important element for health and disease, and today Mg deficiency in man has become an accepted medical problem which might complicate many diseases. Neuromuscular disorders, as laryngeal spasm, are recognized complications of hypomagnesemia, but until now only 1 case of motor esophageal disorder associated with a low Mg plasma level was briefly reported in the literature, even if dysphagia is generally included in the symptomatological pattern of hypomagnesemia. Our observation of a severe form of esophageal spasm, associated with hypomagnesemia, in an aged female patient underlines the pathophysiological meaning of the plasma Mg level and suggests the need for routine Mg determination in the clinical setting.

Aged↗

Reduction of coronary blood flow during coronary artery spasm occurring spontaneously and after provocation by ergonovine maleate.

A 50-year-old man suffering from recurrent chest pain accompanied by transient ST-segment elevation developed spasm of the left anterior descending coronary artery after receiving ergonovine maleate. During spontaneous chest pain, thermodilution coronary sinus blood flow fell from 96 ml/min to 46 ml/min, while the coronary sinsu arteriovenous oxygen difference widened from 9.82 volumes percent to 11.3 volumes percent. During spontaneous relief of pain, coincident with resolution of the ST-segment changes, coronary sinus blood flow gradually rose to 135 ml/min, while coronary sinus arteriovenous oxygen difference narrowed to 6.82 volumes percent. Similar aterations in coronoary sinus blood flow accompanied chest pain provoked by ergonovine maleate. A thallium-201 scan confirmed a perfusion defect in the distribution the left anterior descending coronary artery. Thus, coronary artery spasm can produce a marked deficity in coronary blood flow that is associated with increased myocardial oxygen extraction; release of spasm creates a hyperemic response.

Coronary Angiography↗

Higher prevalence of obsessive-compulsive symptoms in patients with blepharospasm than in patients with hemifacial spasm.

OBJECTIVE: The prevalences of obsessive-compulsive symptoms in patients suffering from blepharospasm and in those with hemifacial spasm were determined. The two conditions have similar symptoms, but only blepharospasm is etiologically linked to basal ganglia dysfunction. METHOD: After being interviewed with the Structured Clinical Interview for DSM-III-R, 13 patients with blepharospasm and 13 with hemifacial spasm completed the SCL-90-R and the Hamburg Obsession/Compulsion Inventory-Short Form. RESULTS: Patients in the blepharospasm group had significantly more obsessive-compulsive symptoms, as indicated by higher scores on the Hamburg Obsession/Compulsion Inventory-Short Form, than the patients with hemifacial spasm. SCL-90-R scores were in the normal range for nine and eight categories, respectively (out of nine). CONCLUSIONS: The findings provide additional support for the hypothesis that obsessive-compulsive symptoms are related to basal ganglia dysfunction.

Aged↗

Magnetic resonance imaging findings in infantile spasms: etiologic and pathophysiologic aspects.

An etiologic evaluation of 86 patients with infantile spasms is presented and the place of cranial magnetic resonance imaging (MRI) findings within this spectrum is discussed. A total of 103 cranial MRIs, performed between 4 and 72 months of age, were analyzed and classified according to the etiologic and pathophysiologic aspects. Ninety-one percent of cases were diagnosed as symptomatic infantile spasms, and hypoxic-ischemic encephalopathy was the primary cause (30%). The most common involvement was thinning of the corpus callosum in 43 patients (50%), followed by dilation of cerebral ventricles in 32 (36%), delayed myelination in 23 (26.7%), lesions of diencephalic deep gray matter in 17 (19.7%), and enlargement of the subarachnoid space in 12 (14%). Thin corpus callosum and diffuse atrophy were changes mainly associated with hypoxic-ischemic encephalopathy, whereas delayed myelination seemed to be independent from a specific etiology. The results showed that cranial MRI may provide considerable information regarding not only the etiology but also the pathophysiology of infantile spasms.

Brain↗

Infantile spasms as an adverse outcome of neonatal cortical sinovenous thrombosis.

Cerebral sinovenous thrombosis is a rare but potentially serious condition often occurring in children with nonspecific presenting features. Much remains to be learned about the long-term outcome of infants with cerebral sinovenous thrombosis. We report a series of four patients taken from a prospective database of neonates with sinovenous thrombosis who subsequently developed infantile spasms, three with hypsarrythmia on electroencephalography and one with multiple independent spike foci. The first patient presented at 2 weeks of age with hypernatremia, dehydration, and seizures. He was found to have extensive thrombosis and hemorrhagic infarction of the right basal ganglia. The second patient presented at 5 weeks of life and was found to have sagittal sinus thrombosis with bilateral intracranial hemorrhage. The third patient presented with seizures on day 1 of life and was found to have venous thrombosis involving the torcular, extending into the sagittal sinus. The fourth patient presented at 3 weeks with lethargy and seizures. He was diagnosed with bacterial meningitis and also had extensive sinus thrombosis. All patients developed infantile spasms at ages 9, 7, 11, and 10 months, respectively. This is the first report in the English literature describing infantile spasms as a possible outcome of sinovenous thrombosis in early infancy.

Anticoagulants↗

Infantile spasms: an early epileptic manifestation in some patients with the congenital bilateral perisylvian syndrome.

We report four patients with infantile spasms and the congenital bilateral perisylvian syndrome. Onset of spasms occurred during the first 6 months of life. Response to corticotropin treatment was prompt and resulted in resolution of seizures in all patients. Epilepsy developed in the four children after an interval of 2 to 12 years. Developmental outcome was variable; three were severely restricted and one was married and lived independently. Imaging studies revealed bilateral perisylvian lesions characteristic of polymicrogyria. Infantile spasms may be the presenting seizure type in some patients with the congenital bilateral perisylvian syndrome.

Adolescent↗