ABSTRACT:A sixty-five year old woman developed relapsing polychondritis with three of the diagnostic criteria established by McAdam et al. (1976), namely bilateral auricular chondritis, ocular inflammation and both cochlear and vestibular dysfunction. Many authors have mentioned other neurological symptoms including unilateral facial weakness. This patient is the first case described with bilateral facial weakness and cerebral manifestations.Relapsing polychondritis, an uncommon, recurrent, inflammatory disorder affecting the cartilaginous tissues of the body (Jaksch-Wartenhorst, 1923; Herman, 1981), is regularly associated with audiovestibular dysfunction (Bollet et al., 1969; Cody et al., 1971; McAdam et al., 1976; Ridgway et al., 1979). McAdam et al. (1976) and Ridgway et al. (1979) have mentioned other neurological symptoms including unilateral facial weakness. We recently observed a patient with polychondritis and multifocal neurological abnormalities. We believe this is the first case described with bilateral facial nerve palsy and with cerebral manifestations.

Probable Alzheimer disease (AD) is described in 79-year-old male twins with monozygosity confirmed by DNA examination. The first twin to be affected began to show signs of intellectual deterioration at age 70. In the other, onset was at age 72. Four of their living sibs (current age range = 75-92) are also suspected to have AD. The possible roles of genetic and environmental factors in the development of AD in this sibship are discussed.

A case of widespread hemorrhagic and perivenous demyelinative leukoencephalomyelitis complicating a localized herpes simplex virus (HSV) brain stem infection is reported in a 28-year-old man. The presence of the virus is documented immunohistochemically and ultrastructurally. The spinal trigeminal tract at the level of the medulla oblongata contained viral antigen in the neurons, glia and in the vascular walls, including a few endothelial cells. The foci of demyelination showed deposits of gamma globulins and slight inflammatory infiltrations; the virus was absent from these lesions. It is postulated that HSV entered the central nervous system through the trigeminal nerve. Focal expression of the viral antigen on the endothelium in a sensitized host was the likely precipitating factor in the hyperacute autoimmune reaction, resulting in the widespread hemorrhagic and demyelinating lesions in the central nervous system.

SUMMARY:Clinical, electrophysiological, and histopathological studies of some members of a family with dominantly inherited hypertrophic neuropathy are presented. Twenty-five members were studied. Seventeen were abnormal on clinical examination. Their ages varied from 2½ to 78 years. Age at onset in 14 of the 17 varied between 2½ and 56 years. Pes cavus and palpable nerve thickening were present in more than half of the affected individuals. All patients had areflexia. Fifteen of the 17 had distal motor weakness as well as mild to moderate sensory impairment. Motor weakness affecting the proximal hip and shoulder girdle muscles was seen in 13 patients. Four patients gave a history of trigeminal neuralgia. Motor nerve conduction velocities were markedly impaired in all the clinically affected members. These studies were normal in the 8 unaffected members. Motor conduction velocities of the proximal segment of the ulnar nerve were slower compared to the distal segment in almost all the affected members. There was no significant correlation between the degree of clinical disability and the extent of impairment in the motor nerve conduction velocities. Sural nerve biopsies were studied. These observations are discussed.

SUMMARY:Ten patients have been described showing inability to stop breathing on command, spontaneous respiration and voluntary respiratory stimulation being unaffected. This abnormality not previously described in the literature, we feel should be named respiratory inhibitory apraxia (R.I.A.). The anatomical organization of respiration is briefly reviewed. R.I.A. is often associated with other forms of apraxia or motor impersistence. It is thought that the urinary and bowel incontinence present in some of these cases might also represent a form of inhibitory apraxia. Information is presented which supports the view that respiratory inhibitory apraxia is due to a minor hemisphere lesion, usually deepseated. Our one autopsied case showed a lesion in the descending motor pathways in the internal capsule, in middle cerebral artery branch territory, disconnecting the voluntary respiratory inhibitory center in the cortex in anterior and middle cerebral cortical branch territories.