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Biomedical subjects

A S Pakiam

Publications and source records attributed to A S Pakiam.

5 recordsLinked to original sources

Intracranial hypotension with parkinsonism, ataxia, and bulbar weakness.

OBJECTIVE: To describe a case of spontaneous intracranial hypotension with a previously unreported constellation of presenting features. DESIGN: Case report. SETTING: Tertiary care center. MAIN OUTCOME AND RESULTS: We describe a patient with intracranial hypotension who presented with a parkinsonian syndrome and later development of ataxia and prominent bulbar symptomatology. Headache was not a feature of her initial presentation and was only reported after repeated questioning during later evaluations. Magnetic resonance imaging of the patient's head revealed findings characteristic of intracranial hypotension. An [18F]fluoro-m-tyrosine positron emission tomographic scan showed normal striatal activity, suggesting intact presynaptic nigrostriatal function. Opening pressure on lumbar puncture was reduced at 40 mm H2O. A source of cerebrospinal fluid leakage was not identified on nuclear cisternography and the patient underwent lumbar epidural blood patching, which resulted in complete resolution of her signs and symptoms as well as in a marked improvement in her imaging findings. CONCLUSIONS: The clinical spectrum of intracranial hypotension can be broadened to include parkinsonism, cerebellar ataxia, and prominent bulbar dysfunction. As with more common manifestations of the disorder, these features may resolve after appropriate treatment.

Ataxia↗

Historical and clinical features of psychogenic tremor: a review of 70 cases.

OBJECTIVES: To review the clinical characteristics and associated features found in patients with psychogenic tremor. METHODS: Ten-year retrospective review of charts of all patients and videotapes of fifty-one patients diagnosed by the senior author as having psychogenic tremor. RESULTS: Seventy patients fulfilled the diagnostic criteria for clinically definite psychogenic tremors. Psychogenic tremors usually started abruptly (73%), often with the maximal disability at onset (46%), and then took static (46%) or fluctuating (17%) courses. Psychogenic tremors usually started in one limb and spread rapidly to a generalized or mixed distribution. Spontaneous resolution and recurrence, easy distractibility together with entrainment and response to suggestion were characteristic features. Presence of functional symptoms and signs and refractoriness to conventional antitremor drugs were common. CONCLUSIONS: Psychogenic tremor is generally not a diagnosis of exclusion. The presence of characteristic features on history and especially clinical examination can permit an accurate diagnosis and avoid unnecessary investigations.

Adolescent↗

Diffuse Lewy body disease presenting as multiple system atrophy.

OBJECTIVES: The majority of patients with diffuse Lewy body disease have cognitive or psychiatric manifestations as part of their initial presentation. A sizable minority present with parkinsonian features alone. Autonomic features may also occur, typically after the development of cognitive changes. We aim to demonstrate that diffuse Lewy body disease may rarely also present with parkinsonism accompanied by marked autonomic dysfunction in the absence of significant cognitive or psychiatric abnormalities. METHODS: Case report based on a retrospective chart review and neuropathological examination. RESULTS: We report on a patient in whom a clinical diagnosis of multiple system atrophy was made based on a presentation of parkinsonism with prominent and early autonomic involvement. The former included postural tremor, rigidity and bradykinesia, while the latter consisted of repeated falls due to orthostasis and the subsequent development of urinary incontinence midway through the course of her illness. She was poorly tolerant of dopaminergic therapy due to accentuated orthostasis. Benefit from levodopa was limited and only evident when attempted withdrawal resulted in increased rigidity. There was no history of spontaneous or drug-induced hallucinations, delusions or fluctuating cognition, and in contrast to the prominence and progression of her parkinsonian and autonomic features over the first several years, cognitive impairment did not occur until the final stages of her illness, seven years after the onset of initial symptoms. Neuropathological examination revealed numerous Lewy bodies in both neocortical as well as subcortical structures consistent with a diagnosis of diffuse Lewy body disease. There was marked neuronal loss in the substantia nigra as well as the autonomic nuclei of the brainstem and spinal cord. CONCLUSIONS: In addition to cognitive, psychiatric, and parkinsonian presentations, diffuse Lewy body disease may present with parkinsonism and prominent autonomic dysfunction, fulfilling proposed criteria for the striatonigral form of MSA.

Aged↗

Multifocal motor neuropathy without overt conduction block.

One of the defining electrophysiological characteristics of multifocal motor neuropathy (MMN) is focal motor nerve conduction block. We have noted occasional patients with typical clinical features of MMN in whom there is no demonstrable conduction block. Upon review of 5 such cases, we conclude that otherwise typical MMN may present without overt conduction block. This subset of patients does not otherwise differ from patients with MMN in whom this hallmark electrodiagnostic feature is present.

Adult↗