Cases reported "Facial Hemiatrophy"

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1/123. Progressive facial hemiatrophy (Parry-Romberg syndrome).

    Three cases of progressive facial hemiatrophy are presented. In all three cases there is evidence of localised scleroderma or morphea in association with the facial hemiatrophy. This would seem to support the contention that the two disorders are closely related. In two cases, ocular complications are prominent and in one Raynaud's phenomena provide clear asymmetrical dilatation of the lateral ventricle suggest that there may be a central rather than a peripheral cause for the sympathetic overactivity. ( info)

2/123. Parry-Romberg syndrome with contralateral and ipsilateral extremity involvement.

    BACKGROUND: The Parry-Romberg syndrome is an acquired progressive hemifacial atrophy involving the subcutaneous tissues of the scalp and face. Involvement of the extremities is uncommon in this syndrome. OBJECTIVE: A case of contralateral and ipsilateral extremity involvement in the Parry-Romberg syndrome is reported, and the difficulties in distinguishing this syndrome from linear scleroderma en coup de sabre are reviewed. methods: A medline search for cases of Parry-Romberg syndrome with contralateral extremity involvement was performed and the cases reviewed. RESULTS: Contralateral extremity involvement in the Parry-Romberg syndrome is rare. CONCLUSION: This may be the first case reported in the English literature of Parry-Romberg syndrome with contralateral and ipsilateral extremity involvement. ( info)

3/123. Progressive facial hemiatrophy (Parry-Romberg syndrome).

    A 48-year-old white woman presented with a 2-year history of progressive facial hemiatrophy involving the right side of the chin and tongue, associated with mastigatory spasm. Neurological examination showed no abnormalities. Computed tomography and magnetic resonance imaging of the brain and electrophysiological investigations were normal. Histopathological examination of a skin biopsy specimen from the chin revealed atrophy of the subcutaneous fat with homogenization of dermal collagen fibres. phenytoin 100 mg t.d.s. relieved the mastigatory spasm. ( info)

4/123. Atraumatic palmar midcarpal dislocation in a skeletally immature adolescent with hemiatrophy.

    We report a late presentation of a palmar midcarpal dislocation in an adolescent female with open growth plates who had no history of antecedent wrist injury. Midcarpal arthrodesis improved function and eliminated progressive pain. ( info)

5/123. A tri-linear pattern of scleroderma 'en coup de sabre' following Blaschko's lines.

    Linear scleroderma 'en coup de sabre' is characterized by atrophy and furrowing of the skin of the frontoparietal region. In most cases it occurs as a single, paramedian line that may be associated with hypoplasia of underlying structures and hemiatrophy of the face. Rarely, lesions form two lines, either both on the same side or bilaterally. We describe an 18-year-old male with an unusual tri-linear pattern of scleroderma 'en coup de sabre' following Blaschko's lines. ( info)

6/123. Hemimasticatory spasm associated with localized scleroderma and facial hemiatrophy.

    OBJECTIVES: To report a case and discuss the mechanism of hemimasticatory spasm. DESIGN: Case report. PATIENT: A 37-year-old woman had a 3-year history of involuntary spasms of the right masseter muscle in association with localized scleroderma and facial hemiatrophy. Electrophysiological studies revealed a normal blink reflex. However, the masseter reflex and silent period were absent on the affected side. Distal latency and compound muscle action potential of the masseter nerve were normal. Needle electromyography demonstrated irregular bursts of motor unit potentials similar to those described in hemifacial spasm. A magnetic resonance imaging scan of the head showed mild hypertrophy of the masseter muscle and atrophy of subcutaneous fatty tissues on the affected side. Local injection of botulinum toxin A into the masseter muscle resolved the patient's symptoms. CONCLUSION: On the basis of clinical and electrophysiological findings, focal demyelination of motor branches of the trigeminal nerve owing to deep tissue changes is suggested as the cause of abnormal excitatory electrical activities resulting in involuntary masticatory movement. ( info)

7/123. Hemifacial atrophy with bilateral short roots.

    We present a case in which the patient had both bilateral shortening of the roots, and hemifacial atrophy. As far as we know, this combination has not been described before. ( info)

8/123. Progressive facial hemiatrophy after epileptic seizures.

    Intractable complex partial seizures developed in a 3-year-old female with normal intracranial findings on computed tomography. Frontal paramedian band-like depression of the skin gradually developed thereafter, and progressive facial hemiatrophy (Parry-Romberg syndrome) was diagnosed. Computed tomography scanning at 5 years of age revealed multiple parenchymal calcifications and low-density areas in the white matter of the frontoparietal lobes. Epileptic seizures, one of the major neurologic complications of progressive facial hemiatrophy, could precede the succeeding neurocutaneous changes. ( info)

9/123. Congenital nonprogressive facial hemiatrophy with ipsilateral eye abnormalities and juvenile glaucoma.

    Congenital nonprogressive facial hemiatrophy is reported in association with anisometropia and ipsilateral cornea, iris, angle abnormalities, and juvenile glaucoma. The importance of complete ocular examination in patients with this uncommon abnormality is emphasized. ( info)

10/123. Neuropathological findings in a patient with epilepsy and the Parry-Romberg syndrome.

    PURPOSE: The Parry-Romberg syndrome is an unusual disorder frequently associated with epilepsy. The origin of this disease, and the cause of epilepsy, are unknown. This study is the first reported case of the Parry-Romberg syndrome, with intractable temporal lobe epilepsy, in which detailed microanatomic analyses have been performed on resected brain tissue obtained after surgical intervention. methods: Standard histopathologic methods and correlative light and electron microscopy, combined with immunocytochemical techniques, were used to study in detail the synaptic microorganization of the resected hippocampal formation. RESULTS: After surgery, the patient was seizure free (follow-up period of 4 years and 7 months). The resected temporal lobe showed a variety of dramatic microanatomic alterations (small groups of ectopic cells, neuronal loss, gliosis, and activated microglial cells) in mesial structures, including the entorhinal cortex, subiculum, and dentate gyrus. At the electron-microscopic level, we found that in the dentate gyrus, the number of synapses in the cell-sparse region adjacent to the ectopic mass of neurons was almost twice that found in the molecular and polymorph cell layers, indicating the intrusion of neuritic processes and synapse formation. In addition, the symmetrical axosomatic synapses characteristically found on granule cells, which are likely derived from gamma-aminobutyric acid (GABA)ergic inhibitory basket cells, were not observed. CONCLUSION: The complete seizure relief after surgery suggests that the pacemaker region(s) of seizure activity were within the resected tissue. However, we do not know which of the multiple neuropathologic findings reported here were the primary cause of seizure activity. Nevertheless, the changes found in the dentate gyrus circuitry appear to be among the most important alterations that would lead to epilepsy. ( info)
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