Optic neuritis as an initial symptom in multiple sclerosis

The present study is based on a multicenter documentation system which includes standardized information on a total of 1271 patients with multiple sclerosis (MS). In 441 (34.7%) cases the optic nerve was involved at the first appearance of the disease, and in 212 (16.6%) subjects optic neuritis (ON) was the sole initial sign. For all MS patients with ON at the onset of the disease the female to male ratio was 1.3, whereas it was 1.5 for the whole series. The mean age at onset was 2 years lower for patients with initial ON as compared with the whole series (29.0 and 31.1 years, respectively). Correlation of the disability of the patients to the duration of the disease revealed the best prognosis for patients with ON as the sole initial sign of MS. The frequency of brainstem/cerebellar and pyramidal signs was lowest among these patients at the time of the present examination. The difference was more pronounced during the first years of the disease and disappeared after longer duration. The correlation curves of disability to the present age of the patients confirmed this pattern. Our findings do not support the idea of initial ON as being a favorable sign of the later course. As an initial bout of MS, it may reflect more precisely the mean age of onset of the disease than other signs.     

Respiratory failure as initial symptom of acid maltase deficiency.

Nine patients with adult onset acid maltase deficiency were seen at the Nijmegen University Hospital and the St Elisabeth Hospital, Tilburg , during the period 1970-1982. Five of these patients developed respiratory failure, and in four this was the initial symptom. The occurrence of respiratory failure as an early symptom of this muscular disease is discussed.     

Throat clicking as the initial symptom of Parkinson's disease.

The presenting manifestations of Parkinson's disease (PD) are variable, but a majority of patients note tremor as the initial symptom. Others complain of slowing of movements, loss of dexterity, fatigue, or changes in handwriting as initial symptoms. We describe a patient who developed an unusual clicking sound emanating from his throat as the initial manifestation of PD.     

Abducens nerve palsy as initial symptom of trigeminal schwannoma

The insidious onset and slow progression of symptoms may cause delay in the recognition of trigeminal schwannomas. Two cases with abducens nerve palsy as an initial symptom are reported, in both of whom the tumour was growing mainly below and anterior to the intracavernous portion of the internal carotid artery. Early detection of clinical symptoms and thorough radiological investigations are important in the early diagnosis of these lesions.     

Facial palsy as the initial symptom of a lymphoreticular malignancy. Case report.

In this case report an adult patient is described who initially manifested a peripheral facial palsy mimicking 'Bell's palsy', which later on appeared to be the first manifestation of a lymphoreticular malignancy. Cranial nerve involvement appears to be very rare as a first symptom of malignant systemic disease. The importance of additional investigations including lumbar puncture and CSF cytology in cases of facial nerve palsy is stressed.     

Oculogyric crisis as an initial symptom of juvenile parkinsonism-like disease

A 12-year-old boy developed occasional attacks of oculogyric crisis after physical exercises or when tired. Following the initial symptom, progressive Parkinsonian features such as bradykinesia, muscular rigidity, hand tremors in posture, mild dysarthria and disorder of postural reflexes developed. There was no marked diurnal fluctuation o symptoms. Serum ceruloplasmin, copper levels, cranial X-ray CT scan and MRI were normal. Measurement of the plasma levels of L-dopa after single oral administration (300 mg) were normal. The treatment with L-dopa improved the Parkinsonian features excluding the attacks of oculogyric crisis in a few weeks. This case is not identical with juvenile Parkinsonism proposed by Yokochi et al for lack of both crural or truncal dystonia and remarkable response to L-dopa. Oculogyric crisis is known in several patients with severe generalized dystonia, and seldom in patients with Parkinson disease or juvenile Parkinsonism. Oculogyric crisis may be one of focal dystonias confined to extraocular muscles.     

Hearing loss as an initial symptom of the opsoclonus-myoclonus syndrome

A patient in whom opsoclonus-myoclonus syndrome associated with a viral encephalitis developed was seen initially because of a hearing loss. Audiometric testing revealed the hearing loss to be bilateral and retrocochlear in type. Four days after the onset of hearing loss, the full opsoclonus-myoclonus syndrome developed. A review of the literature failed to disclose any other cases of the syndrome associated with hearing loss. On the basis of the available pathologic material in this disorder, the retrocochlear hearing loss was believed to have been caused by diffuse involvement of the brain-stem auditory pathways.     

Torticollis as an initial symptom of adult-onset dystonia musculorum deformans

Dystonia musculorum deformans (DMD) is an idiopathic movement disorder which usually involves pediatric age group and progresses to the generalized type. On the contrary in adult-onset DMD, dystonia is usually confined to an upper extremity and its clinical course is benign. The authors report seven patients with adult-onset DMD whose initial symptom was confined to the neck. Diagnosis of idiopathic spasmodic torticollis had been made in all of them. Average ages at the onsets of torticollis and extranuchal dystonia were 49 +/- 13 and 54 +/- 9 years (mean +/- SD) respectively. The duration between these onsets was 2-3 years in five patients and 10-17 years in two younger patients. Two patients finally developed generalized dystonia and one patient became hemidystonic type. These findings suggest that some patients diagnosed as idiopathic spasmodic torticollis are in an early stage of DMD and that this particular type progresses more likely to the generalized form than other types of adult-onset DMD.     

Arm restlessness as the initial symptom in restless legs syndrome

BACKGROUND: Upper extremity symptoms can develop in restless legs syndrome, but are rarely the initial symptom. OBJECTIVES: To report a case of restless legs syndrome with restless arms as the initial symptom, and to review the literature. DESIGN: Case report and literature review. PATIENT: A 78-year-old man had arm restlessness and sleep disturbance. A polysomnogram demonstrated subclinical periodic leg movements 1 year after the onset of his symptoms. RESULTS: Two years later, he developed lower extremity symptoms that fit the criteria for restless legs syndrome. To our knowledge, this is the first case of restless legs syndrome with arm restlessness as the initial symptom in an otherwise neurologically normal person. CONCLUSION: Restless legs syndrome may initially exhibit upper extremity symptoms.